Survival Trends and Causes of Death in Dermatomyositis and Polymyositis: A 19-year Longitudinal Study in Taiwan

Survival Trends and Causes of Death in Dermatomyositis and Polymyositis: A 19-year Longitudinal Study in Taiwan | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Article Survival Trends and Causes of Death in Dermatomyositis and Polymyositis: A 19-year Longitudinal Study in Taiwan Gin Hoong Lee, Ming-Feng Liao, Chun-Che Chu, Hung-Chou Kuo, Mei-Yun Cheng, and 4 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-7485056/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Dermatomyositis (DM) and polymyositis (PM) are rare idiopathic inflammatory myopathies associated with substantial morbidity and mortality. However, data on mortality patterns and prognostic factors in Asian populations, particularly in Taiwan, remain limited. We conducted a retrospective cohort study of 2,264 adult patients diagnosed with DM or PM between 2001 and 2019 using electronic medical records from the largest tertiary referral center in Taiwan. Mortality data were linked with the National Death Registry, and standardized mortality ratios (SMRs) were calculated relative to the general population. During 21,991 person-years of follow-up, 635 deaths were recorded (319 DM, 316 PM), with 5-year mortality rate of 18.0%. Malignancy and pneumonia were the leading causes of death, with significantly elevated SMRs. Patients diagnosed before age 45 had the highest relative risk of death, with SMRs of 6.36 for males and 17.94 for females. Survival analysis revealed significantly reduced survival in patients with malignancies or major infections (log-rank p < 0.0001). Cox regression identified older age at onset, male sex, malignancy, and major infection as independent risk factors for mortality. These findings highlight the persistently high mortality burden in DM and PM patients in Taiwan and underscore the need for early malignancy screening, infection prevention, and tailored management strategies to improve long-term outcomes. Biological sciences/Cancer Health sciences/Diseases Health sciences/Medical research Health sciences/Oncology Health sciences/Risk factors Figures Figure 1 Introduction Dermatomyositis (DM) and polymyositis (PM) are idiopathic inflammatory muscle disorders characterized by skin rashes, progressive proximal muscle weakness, and involvement of other organs such as the lungs and heart [ 1 ]. Serious comorbidities, including interstitial lung disease, malignancies and cardiopulmonary complications, are frequently associated with DM and PM [ 2 , 3 ]. Studies in Isreal and China reported with DM/PM had a five to nine times the risk of death compared to general population [ 4 – 6 ]. Mortality rates in patients with DM/PM have been reported to range from 12.3% to 60.5% at 5 years [ 4 , 6 , 7 ]. While recent studies in Canada and the UK revealed that mortality rates of patients with DM/PM were improving in recent cohorts compared to previous cohorts. However, mortality remained significantly elevated in patients with DM/PM compared to the general population [ 8 , 9 ]. Prior studies showed that malignancies, interstitial lung disease, cariovascular disease and infections to be major causes of deaths in patients with DM/PM [ 8 , 10 – 12 ]. Current mainstay treatments for DM and PM include glucocorticoid and cytostatic drugs, which suppresses the immune system and further increase the risks of major infections [ 13 ]. A cohort study in China reported infection as a leading cause of mortality in patients with idiopathic inflammatory myositis, where pulmonary infection was the most common infection, followed by bacteremia without focus [ 14 ]. DM/PM patient with major infection was found to have poorer survival rate compared to patients without infections [ 14 ]. However, there are limited recent reports on mortality rates and causes of death in DM/PM patients in Taiwan. We conducted this study to evaluate and update the recent mortality situation in the Taiwanese population, examining mortality rates, causes of death, and associated risk factors of DM/PM. Results Baseline characteristic: From 2001–2019, a total of 1100 patients with DM and a total of 1164 patients with PM were identified. The mean of the age of diagnosis of patients with DM and PM were 49.11 ± 15.47 years and 48.53 ± 15.03 years, respectively. Among DM patients, 395 (35.91%) were male and 705 (64.09%) were female; while among PM patients, 463 (39.77%) were male and 701 (60.23%) were female. The mean follow-up duration in DM and PM patients were 8.77 ± 5.39 years and 9.35 ± 5.26 years respectively. Mortality During the 21991.32 person-years of observation, 635 deaths were recorded, with 319 in DM patients and 316 in PM patients. Table 3 shows the observed and expected deaths and calculated SMR for patients with dermatomyositis and polymyositis, stratified by sex and age group at diagnosis. The highest number of observed deaths (125 males, 160 females) occurred in patients aged 45–64 years. The highest crude mortality rate (113.34 and 86.23/1000 person-years for male and female, respectively) was observed in patients aged 65 and older. However, compared to the general population, patient aged below 45 had the highest SMR, 6.36 (95% CI: 4.52–8.20) for males and 17.94 (95% CI: 13.71–22.18) for females. Dermatomyositis In patients with DM, 319 deaths were observed (180 females, 139 males). The estimated survival rate at 1, 5, 10 and 15 years were 90.2% (95% CI: 88.3–91.8), 79.4 (95% CI: 76.8–81.7), 73.4 (95% CI: 70.5–76.1) and 67.1 (95% CI: 63.7–70.2), respectively. The leading causes of death were malignant neoplasms (80 deaths), diseases of the musculoskeletal system and connective tissues (48 deaths), and pneumonia (21 deaths). Pneumonia and malignant neoplasm had significantly higher SMR of 2.46 (95% CI: 1.41–3.51) and 1.80 (95% CI: 1.40–2.19), respectively. In the male population, pneumonia and malignant neoplasm also had an even higher SMR than expected of 2.76 (95% CI: 1.13–4.40) and 2.46 (95% CI: 1.77–3.16), respectively. However, in females a significantly lower SMR was observed for chronic lower respiratory diseases 0.28 (95% CI: 0.00-0.84). Polymyositis In PM patients, a total of 316 deaths were observed (162 females, 154 males). The estimated survival rate at 1, 5, 10 and 15 years were 93.4 (95% CI: 91.8–94.7), 84.4 (95% CI: 82.2–86.4), 77.0 (95% CI: 74.3–79.5), and 68.8 (95% CI: 65.5–71.8), respectively. The top three causes of death were malignant neoplasms (53 deaths), diseases of the musculoskeletal system and connective tissues (31 deaths), and pneumonia (24 deaths). Pneumonia and diseases of heart had the significantly higher SMRs: 2.74 (95% CI: 1.64–3.83) and 2.48 (95% CI: 1.73–3.23) respectively. In the male population, significantly higher SMRs were observed in pneumonia, (4.11; 95% CI: 2.09–6.12), diseases of heart (2.95; 95% CI: 1.69–4.21), and malignant neoplasm (1.82; 95% CI: 1.21–2.42). However, in females, a significantly lower SMR was observed for cerebrovascular disease 0.15 (95% CI: 0.00-0.44). Survival analysis in DM/PM regarding malignancy and major infection status. The Kaplan-Meier survival analysis was done to estimate the survival probabilities of patient with dermatomyositis or polymyositis (Fig. 1 ). The overall 15-year survival rate in DM patients is lower than PM patients, though not statistically significant, P = 0.0753. In DM patients, those with malignancy had significantly lower 5-, 10- and 15-year survival rate than those without malignancy (55.7%, 40.3%, and 33.7% vs 80.8%, 75.6% and 69.4%, respectively, log rank test P < 0.0001). Patients with major infections also had significantly lower 5-, 10- and 15-year survival rate than those without major infections as well (74.4%, 66.9% and 58.9% vs 84.5%, 80.1% and 75.7%, respectively, log rank test P < 0.0001). In PM patients, those with malignancy had significantly lower 5-, 10- and 15-year survival rate than those without malignancy (68.4%, 49.3% and 30.6% vs 85.0%, 78.1% and 70.3%, respectively, log rank test P < 0.0001). Patients with major infections also had significantly lower 5-, 10- and 15-year survival rate than those without major infections as well (81.1%, 71.4% and 60.2% vs 86.5%, 80.7% and 74.4%, respectively, log rank test P < 0.0001). Risk factors for mortality in DM/PM patients Cox proportional hazard regression analysis revealed that age at onset, male gender, malignancy, hypertension, and major infection were significant prognostic factors for mortality in both DM and PM patients (Table 5 ). The presence of malignancy had the highest hazard ratio (HR) for mortality: 2.81 (95% CI: 2.01–3.93) in DM patients and 3.03 (95% CI: 2.00–4.60) in PM patients. Stratified multivariate analysis revealed that age at onset (HR 1.05, 95% CI 1.05–1.06, P < 0.0001), male gender (HR 1.40, 95% CI 1.11–1.76, P = 0.0041) and presence of major infection (HR 1.54, 95% CI 1.21–1.98, P = 0.0005) were independent predictors of mortality in DM patients. While in PM patients, age at onset (HR 1.06, 95% CI 1.05–1.07, P < 0.0001), presence of malignancy (HR 1.67, 95% CI 1.08–2.58, P = 0.022) and presence of major infection (HR 1.41, 95% CI 1.10–1.80, P = 0.006) were independent predictors of mortality. Discussion This study provides comprehensive data and analysis on the causes of death and survival outcomes of DM/PM patients in Taiwan. The 5-year mortality rate of 18.0% in this cohort is within the range observed in previous studies [ 4 , 6 , 7 ]. Advances in disease understanding and treatments may have contributed to this improved survival outcome, compared to older cohorts. Malignancy was identified as the leading cause of death in both DM and PM patients, with significantly elevated SMRs of 1.80 in DM patients. Poor survival outcome is noted in DM/PM patients with malignancies, where most mortality occurs within the first 5 years. Previous studies showed an increased cancer risk in patients with DM/PM and most cancers were diagnosed within 1 year of diagnosis of DM/PM [ 2 , 6 ]. In contrast to several previous DM/PM cohorts such as in Finland, Hungary and US which reported higher mortality due to cardiovascular complications [ 15 – 17 ]. This could be due to ethinicity and regional difference, where western population more commonly had cardiovascular risk factors [ 18 ]. We also found that malignancy and major infections were independent prognostic factor of mortality in patients with DM/PM. Pneumonia was found to have two-and-a-half-fold increased mortality risk in patients with DM/PM compared to the general populations. Lung complications such as interstitial lung diseases are common in dermatomyositis and polymyositis patients, while cancer treatments and immunosuppressant uses expose patients to higher risk of infection [ 3 , 11 ]. These factors combined, major infections such as pneumonia in patients with DM/PM brings higher mortality risks compared to the general populations. Compared to general population, disease of heart had significantly higher SMR in patients with polymyositis, which is consistent with prior research showing cardiac complications such as arrhythmias, myocarditis and heart failure in PM [ 10 , 19 , 20 ]. Cardiac involvement in patients with PM are not uncommon, including complications such as ischemic heart disease, myocardial infarction, heart failure, cardiac arrhythmia, pericardial effusions and even pulmonary hypertensions [ 10 , 20 ]. Chronic inflammation from DM/PM may also damage the myocardium causing further cardiac complications. Additionally, corticosteroid use and reduced physical activity from muscle weakness may exacerbate cardiovascular risk factors like hypertension and dyslipidemia, further contributing to mortality. Proactive cardiac surveillance in PM management is needed to improve the mortality condition. While the overall prevalence and demographic characteristics of dermatomyositis (DM) and polymyositis (PM), such as age and sex distribution, appear similar across Western and Asian populations, significant disparities exist in disease subtypes, associated malignancies, and autoantibody profiles [ 21 ]. Notably, DM is more prevalent in Asia compared to PM, and while the overall cancer risk is comparable, Asian patients exhibit a higher incidence of nasopharyngeal and lung cancers, in contrast to the ovarian, lung, gastric, and colorectal cancers more commonly observed in Western cohorts [ 21 , 22 ]. These regional differences also extend to the prevalence of specific autoantibodies (e.g., anti-p155/140 in Asia versus anti-Jo-1 in the West) and a higher incidence of interstitial lung disease in Asian DM/PM patients [ 23 ]. These differences underscored the importance of ethnicity-specific considerations in clinical screening and management strategies. This study highlights that patient under 45 years of age have markedly higher SMRs compared to the general population, with SMRs of 6.36 for males and 17.94 for females, which was similarly reported in the study by Kridin et al [ 5 ]. This finding is striking because it suggests that despite the relatively low crude mortality rates in younger patients, the relative risk of death compared to the general population is disproportionately high. Potential causes for this significant disparities include delayed diagnosis, more aggressive disease phenotypes or complications like malignancy or infections that may disproportionately affect younger patients with DM/PM, compared to the general populations. This finding underscores the need for heightened clinical vigilance and tailored management strategies for reduction of this significant mortality risk posed upon the younger DM/PM patients. Interestingly, we found that older age of onset and male gender were both independent prognostic factor of mortality in DM patients in the Cox regression analysis. Male sex was also previously reported as a risk factor of mortality in China and Italy [ 24 , 25 ]. Previous study showed that male patients had higher prevelance of severe interstitial lung disease as well as rapidly progressive interstitial lung disease, as well as higher inflammatory biomarkers than the female patients [ 26 ]. A group in Japan showed that androgen deficiency promotes development of pulmonary emphysema, and androgen replacement therapy reverses this condition in mice [ 27 ]. This suggests sex hormones may play a role in the disease process of interstitial lung disease and inflammatory myositis. Limitations This study was conducted in the largest tertiary referral medical center in Taiwan and included a relatively large sample size of DM/PM patients with long observational period (19 years). We used the National Death Registry data to have a comprehensive overview of mortality causes and patterns in DM/PM patients across Taiwan, which allowed us to examine SMR and survival outcomes in detail. However, several limitations in this study needed to be addressed. Firstly, relying on National Death Registry data for analysis, potential coding errors in cause-of-death reporting may be present which could affect the accuracy of analysis. Interstitial lung disease is not recorded as a cause-of-death in the National Death Registry and such data could not be retrieved for analysis. Secondly, a cohort of myositis patients in a single center tertiary referral center may not be fully representative of all DM/PM patients in Taiwan and may likely to include a higher proportion of more severe or complicated cases. Thirdly, we did not include patients with amyopathic dermatomyositis and inclusion body myositis due to ICD diagnostic coding constraints. Lastly, due to the retrospective nature of our study, we were unable to collect complete clinical data such as use of immunosuppressive agents and rate of smoking in the enrolled patients, which are important factors affecting the mortality and morbidity outcomes of these patients. Conclusion Our study provides valuable insights into the detailed mortality situation of DM/PM patients in Taiwan. High mortality rate is seen in DM/PM patients, and infection and malignancy are the leading cause of death. Older age of onset, male, presence of malignancy and major infections were all poor prognosis factors of mortality. Vigilant surveillance for malignancy and steps to mitigate aspiration risk are important to reduce mortality in this population. Further study in characteristics of different autoantibody disease presentation and treatment modalities may help improve care and outcome in patients with inflammatory myopathies. Materials and Methods Study subject: The patient cohort and data collection methods for this study are the same as those described in our previous publication regarding malignancies in patients with DM or PM, which we described as follows [ 2 ]. We conducted a registry analysis using electronic medical records (EMRs) from the outpatient clinic and admission data from a tertiary medical center (the largest hospital in Taiwan) from January 2001 to May 2019. EMRs included demographic data, dates of clinical visits, diagnostic codes, and details of examinations. This study was based in part on data from the Chang Gung Research Database provided by Chang Gung Memorial Hospital. Subjects below 18 years of age were excluded. Diagnoses of DM and PM were based on the Bohan and Peter diagnostic criteria. The study protocol was approved by the Chang Gung Memorial Hospital Institutional Review Board approved the waiver of the participants’ consent (CGMHIRB201901511B0). All methods were performed in accordance with the relevant guidelines and regulations. Diagnostic coding: Patients with DM with the International Classification of Diseases (ICD), ninth revision (ICD-9) code 7103 and ICD, tenth revision (ICD-10) codes M33, M3310, M3319, M3390, M3399, and M360 or PM (or ICD-9 code 7104; or ICD-10 codes M3320 or M3329) were recruited. Amyopathic DM and inclusion body myositis were not identified due to a lack of specific diagnostic codes. To study the associated comorbidities, we selected diabetes mellitus (ICD-9: 250; ICD-10: I110, I101, I109, I110, I111, and I119), and hypertension (ICD-9: 401 and 402; ICD-10: I10-I16). Outcome measures: Patients were linked to the National Death Registry using their unique identification number. The date of death and cause of death were extracted, with causes coded by ICD-9 and ICD-10. All patients were followed from the index date until the date of death or May 31st, 2019, whichever came first. Statistical analysis: All statistical analyses were performed using SPSS (version 21.0; IBM, New York, USA). Continuous variables are expressed as the means ± standard deviations. Categorical variables are presented as numbers and ratios. The standardized mortality ratio (SMR) was used to compare the mortality of patients with DM/PM to the general population of Taiwan. The top 10 causes of death that are present in at least 9 years of the study period were used to calculate expected mortality rates. Kaplan-Meier analysis was used for to compare the survival probability in patients with DM or PM, with or without malignancy and major infection. A major infection was defined as one that necessitated the use of intravenous and/or prolonged course of antimicrobial treatment for more than 1 week. A Cox regression model was used to examine the hazard ratios of risk factors of mortality in patients with DM and PM. Statistical significance was defined as P < 0.05. Declarations Author contributions statement Lung-Sun Ro and Jung Lung Hsu conceived the idea. Jung Lung Hsu, Ming-Feng Liao, Chun-Che Chu, Hung-Chou Kuo, Mei-Yun Cheng, Mei-Lan Chen collected and checked the data. Pin-Hsuan Huang performed the data analysis. Gin Hoong Lee wrote the manuscript. Jung Lung Hsu revised the manuscript. All authors reviewed the manuscript and approved the final version of the manuscript. Competing interests statement The authors declare no competing interests. Fundings The authors declared no funding support for this research. Author Contribution Lung-Sun Ro and Jung Lung Hsu conceived the idea. Jung Lung Hsu, Ming-Feng Liao, Chun-Che Chu, Hung-Chou Kuo, Mei-Yun Cheng, Mei-Lan Chen collected and checked the data. Pin-Hsuan Huang performed the data analysis. Gin Hoong Lee wrote the manuscript. Jung Lung Hsu revised the manuscript. All authors reviewed the manuscript and approved the final version of the manuscript. Acknowledgement The authors thank Ping-Hsuan Huang for the statistical assistance and wish to acknowledge the support of the Maintenance Project of the Center for Big Data Analytics and Statistics (Grant: CLRPG3N0011) at Chang Gung Memorial Hospital for study design and monitor, data analysis and interpretation. Data Availability All data and codes generated used in this study will be available upon request to the corresponding author. References Dalakas, M. C. & Hohlfeld, R. Polymyositis and dermatomyositis. Lancet 362 (9388), 971–982 (2003). Hsu, J. L. et al. 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Gender differences in patients with anti-MDA5-positive dermatomyositis: a cohort study of 251 cases. Clin. Rheumatol. 43 (1), 339–347 (2024). Aono, K. et al. Testosterone deficiency promotes the development of pulmonary emphysema in orchiectomized mice exposed to elastase. Biochem. Biophys. Res. Commun. 558 , 94–101 (2021). Additional & Information. Tables Table 1. Standardized mortality ratio (SMR) of patients with dermatomyositis and polymyositis. Dermatomyositis Overall n = 1100 Females n = 705 Males n = 395 Malignant neoplasms 1.80 (1.40 – 2.19) * 1.42 (0.93 – 1.91) 2.46 (1.77 – 3.16) * Disease of heart 1.52 (0.93 – 2.12) 1.58 (0.72 – 2.44) 1.65 (0.72 – 2.58) Pneumonia 2.46 (1.41 – 3.51) * 2.19 (0.83 – 3.55) 2.76 (1.13 – 4.40) * Cerebrovascular disease 1.08 (0.49 – 1.67) 0.99 (0.20 – 1.79) 1.27 (0.33 – 2.21) Diabetes mellitus 1.34 (0.61 – 2.07) 1.94 (0.60 – 3.29) 1.05 (0.13 – 1.97) Chronic lower respiratory diseases 0.74 (0.01 – 1.46) 0.28 (0.00 – 0.84) * 1.20 (0.00 – 2.56) Chronic liver disease and cirrhosis 1.68 (0.58 – 2.77) 1.48 (0.03 – 2.93) 1.26 (0.16 – 2.37) Nephritis, nephrotic syndrome and nephrosis 1.27 (0.25 – 2.29) 1.54 (0.03 – 3.06) 1.09 (0.00 – 2.59) Intentional self-harm (suicide) 1.29 (0.03 – 2.56) 1.18 (0.00 – 3.50) 3.75 (0.00 – 7.99) Accidents and adverse effects 0.57 (0.01 – 1.13) 0.50 (0.00 – 1.19) 0.67 (0.00 – 1.60) Polymyositis Overall n = 1164 Females n = 701 Males n = 463 Malignant neoplasms 1.15 (0.84 – 1.46) 0.72 (0.39 – 1.05) 1.82 (1.21 – 2.42) * Disease of heart 2.48 (1.73 – 3.23) * 2.31 (1.32 – 3.30) * 2.95 (1.69 – 4.21) * Pneumonia 2.74 (1.64 – 3.83) * 1.59 (0.49 – 2.68) 4.11 (2.09 – 6.12) * Cerebrovascular diseases 0.56 (0.15 – 0.98) * 0.15 (0.00 – 0.44) * 1.11 (0.22 – 2.00) Diabetes mellitus 1.30 (0.59 – 2.01) 2.16 (0.82 – 3.50) 0.64 (0.00 – 1.37) Chronic lower respiratory diseases 0.54 (0.00 – 1.15) 0.52 (0.00 – 1.23) 0.41 (0.00 – 1.21) Chronic liver disease and cirrhosis 1.24 (0.32 – 2.17) 0.98 (0.00 – 2.09) 1.03 (0.02 – 2.03) Nephritis, nephrotic syndrome and nephrosis 2.27 (0.93 – 3.61) 2.39 (0.62 – 4.17) 2.21 (0.04 – 4.39) Intentional self-harm (suicide) 2.41 (0.74 – 4.08) 3.90 (0.08 – 7.71) 5.00 (0.10 – 9.91) Accidents and adverse effects 0.54 (0.01 – 1.07) 0.44 (0.00 – 1.06) 0.68 (0.00 – 1.62) * indicates statistical significance at p < 0.05. Table 2. Causes of death in patients with dermatomyositis and polymyositis Cause of death Dermatomyositis n (%) Polymyositis n (%) Overall n (%) Total death number 319 (100) 316 (100) 635 (100) Malignant neoplasm 80 (25.1) 53 (16.8) 133 (20.9) Diseases of the musculoskeletal system and connective tissue 48 (15.1) 31 (9.8) 79 (12.4) Diseases of heart (except hypertensive diseases) 25 (7.8) 42 (13.3) 67 (10.6) Pneumonia 21 (6.6) 24 (7.6) 45 (7.1) Diabetes Mellitus 13 (4.1) 13 (4.1) 26 (4.1) Cerebrovascular diseases 13 (4.1) 7 (2.2) 20 (3.2) Nephritis, nephrotic syndrome and nephrosis 6 (1.9) 11(3.5) 17 (2.7) Chronic liver disease and cirrhosis 9 (2.8) 7 (2.2) 16 (2.5) Suicide 4 (1.3) 8 (2.5) 12 (1.9) Chronic lower respiratory diseases 4 (1.3) 3 (0.9) 7 (1.1) Accidents and adverse effects 4 (1.3) 4 (1.3) 8 (1.3) Others 92 (28.8) 113 (35.8) 205 (32.3) Data are number (%) Table 3. Observed and expected deaths and standardized mortality ratios in patient with dermatomyositis/polymyositis stratified by age and sex at incidence. Person-years Observed deaths (rate per 1000) Expected deaths SMR (95% CI) Overall 21991.32 635 (28.88) 241.73 2.63 (2.42-2.83) Males 7983.55 293 (36.7) 108.81 2.69 (2.38-3.00) <45 3918.5 46 (11.74) 7.23 6.36 (4.52-8.2.0) 45-64 2988.57 125 (41.83) 44.04 2.84 (2.34-3.34) ≧65 1076.45 122 (113.34) 111.33 1.10 (0.90-1.29) Females 14007.77 342 (24.42) 107.05 3.19 (2.86-3.53) <45 6779.65 69 (10.18) 3.85 17.94 (13.71-22.18) 45-64 5917.74 160 (27.04) 34.93 4.58 (3.87-5.29) ≧65 1310.38 113 (86.23) 91.55 1.23 (1.01-1.46) Abbreviations: SMR, standardized mortality ratio; CI, confidence interval. Table 4. Estimated overall survival following the diagnosis of dermatomyositis and polymyositis. 1-year survival, % (95% CI) 5-year survival, % (95% CI) 10-year survival, % (95% CI) 15-year survival, % (95% CI) Overall 91.8 (90.6-92.9) 82.0 (80.3-83.5) 75.3 (73.3-77.1) 67.9 (65.6-70.1) Polymyositis 93.4 (91.8-94.7) 84.4 (82.2-86.4) 77.0 (74.3-79.5) 68.8 (65.5-71.8) Dermatomyositis 90.2 (88.3-91.8) 79.4 (76.8-81.7) 73.4 (70.5-76.1) 67.1 (63.7-70.2) Abbreviations: CI, confidence interval. Table 5. Cox regression analyses of risk factors in the dermatomyositis and polymyositis patients. Univariate analysis Dermatomyositis Polymyositis HR (95% CI) P value HR (95% CI) P value Age at onset 1.05 (1.05 - 1.06) <0.0001 1.06 (1.05 - 1.07) <0.0001 Gender(M) 1.54 (1.23 - 1.94) 0.0002 1.41 (1.12 - 1.78) 0.0031 Malignancy 2.81 (2.01 - 3.93) <0.0001 3.03 (2.00 - 4.60) <0.0001 Hypertension 1.43 (1.13 - 1.81) 0.0028 1.54 (1.22 - 1.95) 0.0003 Infection 1.81 (1.44 - 2.29) <0.0001 1.63 (1.30 - 2.05) <0.0001 Stratified multivariate analysis Dermatomyositis Polymyositis HR (95% CI) P value HR (95% CI) P value Age at onset 1.05 (1.05 - 1.06) <0.0001 1.06 (1.05 - 1.07) <0.0001 Gender(M) 1.40 (1.11 - 1.76) 0.0041 1.14 (0.90 - 1.44) 0.2687 Malignancy 1.40 (0.98 – 2.00) 0.0664 1.67 (1.08 - 2.58) 0.022 Infection 1.54 (1.21 - 1.98) 0.0005 1.41 (1.10 - 1.80) 0.006 Bold denotes statistical significance. Abbreviations: HR, hazard ratio; CI, confidence interval. Additional Declarations No competing interests reported. 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Lewis College of Nursing and Health Professions, Georgia State University","correspondingAuthor":false,"prefix":"","firstName":"Mei-Lan","middleName":"","lastName":"Chen","suffix":""},{"id":532722711,"identity":"60bd0396-ed82-4658-84c2-7e592ce3674b","order_by":6,"name":"Pin-Hsuan Huang","email":"","orcid":"","institution":"Chang Gung Memorial Hospital","correspondingAuthor":false,"prefix":"","firstName":"Pin-Hsuan","middleName":"","lastName":"Huang","suffix":""},{"id":532722712,"identity":"f6c5199f-d7ff-478f-a299-f4ef4bc6857e","order_by":7,"name":"Long-Sun Ro","email":"","orcid":"","institution":"Chang Gung Memorial Hospital Linkou Medical Center and College of Medicine, Chang-Gung University","correspondingAuthor":false,"prefix":"","firstName":"Long-Sun","middleName":"","lastName":"Ro","suffix":""},{"id":532722713,"identity":"3eabd4d8-76c3-4b8c-ac7e-2bcd61a4ae67","order_by":8,"name":"Jung Lung Hsu","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA2UlEQVRIiWNgGAWjYFAC5gaGDwwMdvwMPAwHiNLAw8DYwDiDgSFZsgGsxQBkCGEtzDwMDIwbgFoYiNJiL5HYJm2bU8dswMB78HBBxR8Gc/Z+/A7kAWnJ3XaYz5yBL+HwjDMGDJY9hwk4DKLlALNlA4/BYd42AwaDG8lEaLHcVse44QBIyz+glvuPidDCuI0ZqqUBZAuhEDvzsNmyd9vhZMlmoBaeY8Y8BmeSDfBqYW9PPnjj57Y6O372HuPPPDVycgbHDz7Abw0cQJ3DQ6TyUTAKRsEoGAX4AABEtT+I57WN1AAAAABJRU5ErkJggg==","orcid":"","institution":"New Taipei Municipal TuCheng Hospital (Built and Operated by Chang Gung Medical Foundation), Chang Gung Memorial Hospital and Chang Gung University","correspondingAuthor":true,"prefix":"","firstName":"Jung","middleName":"Lung","lastName":"Hsu","suffix":""}],"badges":[],"createdAt":"2025-08-29 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02:45:09","extension":"html","order_by":9,"title":"","display":"","copyAsset":false,"role":"acdc-reference","size":103900,"visible":true,"origin":"","legend":"","description":"","filename":"earlyproof.html","url":"https://assets-eu.researchsquare.com/files/rs-7485056/v1/9b3a72b83c9b79cb93ae5079.html"},{"id":94156242,"identity":"69707db1-de33-47ee-91f1-b6d35a1d273d","added_by":"auto","created_at":"2025-10-23 02:53:08","extension":"jpg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":1047262,"visible":true,"origin":"","legend":"\u003cp\u003eKaplan-Meier Plot of the survival of the patients with dermatomyositis (DM) and polymyositis (PM). The survival curve of the total population stratified by with or without malignancy (A), and with or without major infection (B). The survival curve of the patients with DM stratified by with or without malignancy (C), and with or without major infection (D). The survival curve of the patients with PM stratified by with or without malignancy (E), and with or without major infection (F).\u003c/p\u003e","description":"","filename":"Figure1.jpg","url":"https://assets-eu.researchsquare.com/files/rs-7485056/v1/dcbdf11c1a0da2f03a6dc0c2.jpg"},{"id":94672730,"identity":"38a05e6c-5a42-4977-a027-2f166cea41b3","added_by":"auto","created_at":"2025-10-29 13:40:54","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":2242602,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-7485056/v1/557ad061-5556-465e-9c99-d96bc47acce8.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Survival Trends and Causes of Death in Dermatomyositis and Polymyositis: A 19-year Longitudinal Study in Taiwan","fulltext":[{"header":"Introduction","content":"\u003cp\u003eDermatomyositis (DM) and polymyositis (PM) are idiopathic inflammatory muscle disorders characterized by skin rashes, progressive proximal muscle weakness, and involvement of other organs such as the lungs and heart [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. Serious comorbidities, including interstitial lung disease, malignancies and cardiopulmonary complications, are frequently associated with DM and PM [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e, \u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e].\u003c/p\u003e\u003cp\u003eStudies in Isreal and China reported with DM/PM had a five to nine times the risk of death compared to general population [\u003cspan additionalcitationids=\"CR5\" citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. Mortality rates in patients with DM/PM have been reported to range from 12.3% to 60.5% at 5 years [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e, \u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e, \u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. While recent studies in Canada and the UK revealed that mortality rates of patients with DM/PM were improving in recent cohorts compared to previous cohorts. However, mortality remained significantly elevated in patients with DM/PM compared to the general population [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e, \u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e]. Prior studies showed that malignancies, interstitial lung disease, cariovascular disease and infections to be major causes of deaths in patients with DM/PM [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e, \u003cspan additionalcitationids=\"CR11\" citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e].\u003c/p\u003e\u003cp\u003eCurrent mainstay treatments for DM and PM include glucocorticoid and cytostatic drugs, which suppresses the immune system and further increase the risks of major infections [\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e]. A cohort study in China reported infection as a leading cause of mortality in patients with idiopathic inflammatory myositis, where pulmonary infection was the most common infection, followed by bacteremia without focus [\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e]. DM/PM patient with major infection was found to have poorer survival rate compared to patients without infections [\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e].\u003c/p\u003e\u003cp\u003eHowever, there are limited recent reports on mortality rates and causes of death in DM/PM patients in Taiwan. We conducted this study to evaluate and update the recent mortality situation in the Taiwanese population, examining mortality rates, causes of death, and associated risk factors of DM/PM.\u003c/p\u003e"},{"header":"Results","content":"\u003cdiv id=\"Sec3\" class=\"Section2\"\u003e\n \u003ch2\u003eBaseline characteristic:\u003c/h2\u003e\n \u003cp\u003eFrom 2001\u0026ndash;2019, a total of 1100 patients with DM and a total of 1164 patients with PM were identified. The mean of the age of diagnosis of patients with DM and PM were 49.11 \u0026plusmn; 15.47 years and 48.53 \u0026plusmn; 15.03 years, respectively. Among DM patients, 395 (35.91%) were male and 705 (64.09%) were female; while among PM patients, 463 (39.77%) were male and 701 (60.23%) were female. The mean follow-up duration in DM and PM patients were 8.77 \u0026plusmn; 5.39 years and 9.35 \u0026plusmn; 5.26 years respectively.\u003c/p\u003e\n\u003c/div\u003e\n\u003ch3\u003eMortality\u003c/h3\u003e\n\u003cp\u003eDuring the 21991.32 person-years of observation, 635 deaths were recorded, with 319 in DM patients and 316 in PM patients. Table \u003cspan class=\"InternalRef\"\u003e3\u003c/span\u003e shows the observed and expected deaths and calculated SMR for patients with dermatomyositis and polymyositis, stratified by sex and age group at diagnosis. The highest number of observed deaths (125 males, 160 females) occurred in patients aged 45\u0026ndash;64 years. The highest crude mortality rate (113.34 and 86.23/1000 person-years for male and female, respectively) was observed in patients aged 65 and older. However, compared to the general population, patient aged below 45 had the highest SMR, 6.36 (95% CI: 4.52\u0026ndash;8.20) for males and 17.94 (95% CI: 13.71\u0026ndash;22.18) for females.\u003c/p\u003e\n\u003ch3\u003eDermatomyositis\u003c/h3\u003e\n\u003cp\u003eIn patients with DM, 319 deaths were observed (180 females, 139 males). The estimated survival rate at 1, 5, 10 and 15 years were 90.2% (95% CI: 88.3\u0026ndash;91.8), 79.4 (95% CI: 76.8\u0026ndash;81.7), 73.4 (95% CI: 70.5\u0026ndash;76.1) and 67.1 (95% CI: 63.7\u0026ndash;70.2), respectively.\u003c/p\u003e\n\u003cp\u003eThe leading causes of death were malignant neoplasms (80 deaths), diseases of the musculoskeletal system and connective tissues (48 deaths), and pneumonia (21 deaths). Pneumonia and malignant neoplasm had significantly higher SMR of 2.46 (95% CI: 1.41\u0026ndash;3.51) and 1.80 (95% CI: 1.40\u0026ndash;2.19), respectively. In the male population, pneumonia and malignant neoplasm also had an even higher SMR than expected of 2.76 (95% CI: 1.13\u0026ndash;4.40) and 2.46 (95% CI: 1.77\u0026ndash;3.16), respectively. However, in females a significantly lower SMR was observed for chronic lower respiratory diseases 0.28 (95% CI: 0.00-0.84).\u003c/p\u003e\n\u003ch3\u003ePolymyositis\u003c/h3\u003e\n\u003cp\u003eIn PM patients, a total of 316 deaths were observed (162 females, 154 males). The estimated survival rate at 1, 5, 10 and 15 years were 93.4 (95% CI: 91.8\u0026ndash;94.7), 84.4 (95% CI: 82.2\u0026ndash;86.4), 77.0 (95% CI: 74.3\u0026ndash;79.5), and 68.8 (95% CI: 65.5\u0026ndash;71.8), respectively.\u003c/p\u003e\n\u003cp\u003eThe top three causes of death were malignant neoplasms (53 deaths), diseases of the musculoskeletal system and connective tissues (31 deaths), and pneumonia (24 deaths). Pneumonia and diseases of heart had the significantly higher SMRs: 2.74 (95% CI: 1.64\u0026ndash;3.83) and 2.48 (95% CI: 1.73\u0026ndash;3.23) respectively. In the male population, significantly higher SMRs were observed in pneumonia, (4.11; 95% CI: 2.09\u0026ndash;6.12), diseases of heart (2.95; 95% CI: 1.69\u0026ndash;4.21), and malignant neoplasm (1.82; 95% CI: 1.21\u0026ndash;2.42). However, in females, a significantly lower SMR was observed for cerebrovascular disease 0.15 (95% CI: 0.00-0.44).\u003c/p\u003e\n\u003cp\u003e\u003cspan type=\"BoldUnderline\" class=\"BoldUnderline\" name=\"Emphasis\"\u003eSurvival analysis in DM/PM regarding malignancy and major infection status.\u003c/span\u003e\u003c/p\u003e\n\u003cp\u003eThe Kaplan-Meier survival analysis was done to estimate the survival probabilities of patient with dermatomyositis or polymyositis (Fig. \u003cspan class=\"InternalRef\"\u003e1\u003c/span\u003e). The overall 15-year survival rate in DM patients is lower than PM patients, though not statistically significant, P\u0026thinsp;=\u0026thinsp;0.0753.\u003c/p\u003e\n\u003cp\u003eIn DM patients, those with malignancy had significantly lower 5-, 10- and 15-year survival rate than those without malignancy (55.7%, 40.3%, and 33.7% vs 80.8%, 75.6% and 69.4%, respectively, log rank test P\u0026thinsp;\u0026lt;\u0026thinsp;0.0001). Patients with major infections also had significantly lower 5-, 10- and 15-year survival rate than those without major infections as well (74.4%, 66.9% and 58.9% vs 84.5%, 80.1% and 75.7%, respectively, log rank test P\u0026thinsp;\u0026lt;\u0026thinsp;0.0001).\u003c/p\u003e\n\u003cp\u003eIn PM patients, those with malignancy had significantly lower 5-, 10- and 15-year survival rate than those without malignancy (68.4%, 49.3% and 30.6% vs 85.0%, 78.1% and 70.3%, respectively, log rank test P\u0026thinsp;\u0026lt;\u0026thinsp;0.0001). Patients with major infections also had significantly lower 5-, 10- and 15-year survival rate than those without major infections as well (81.1%, 71.4% and 60.2% vs 86.5%, 80.7% and 74.4%, respectively, log rank test P\u0026thinsp;\u0026lt;\u0026thinsp;0.0001).\u003c/p\u003e\n\u003ch3\u003eRisk factors for mortality in DM/PM patients\u003c/h3\u003e\n\u003cp\u003eCox proportional hazard regression analysis revealed that age at onset, male gender, malignancy, hypertension, and major infection were significant prognostic factors for mortality in both DM and PM patients (Table \u003cspan class=\"InternalRef\"\u003e5\u003c/span\u003e). The presence of malignancy had the highest hazard ratio (HR) for mortality: 2.81 (95% CI: 2.01\u0026ndash;3.93) in DM patients and 3.03 (95% CI: 2.00\u0026ndash;4.60) in PM patients.\u003c/p\u003e\n\u003cp\u003eStratified multivariate analysis revealed that age at onset (HR 1.05, 95% CI 1.05\u0026ndash;1.06, P\u0026thinsp;\u0026lt;\u0026thinsp;0.0001), male gender (HR 1.40, 95% CI 1.11\u0026ndash;1.76, P\u0026thinsp;=\u0026thinsp;0.0041) and presence of major infection (HR 1.54, 95% CI 1.21\u0026ndash;1.98, P\u0026thinsp;=\u0026thinsp;0.0005) were independent predictors of mortality in DM patients. While in PM patients, age at onset (HR 1.06, 95% CI 1.05\u0026ndash;1.07, P\u0026thinsp;\u0026lt;\u0026thinsp;0.0001), presence of malignancy (HR 1.67, 95% CI 1.08\u0026ndash;2.58, P\u0026thinsp;=\u0026thinsp;0.022) and presence of major infection (HR 1.41, 95% CI 1.10\u0026ndash;1.80, P\u0026thinsp;=\u0026thinsp;0.006) were independent predictors of mortality.\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eThis study provides comprehensive data and analysis on the causes of death and survival outcomes of DM/PM patients in Taiwan. The 5-year mortality rate of 18.0% in this cohort is within the range observed in previous studies [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e, \u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e, \u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. Advances in disease understanding and treatments may have contributed to this improved survival outcome, compared to older cohorts.\u003c/p\u003e\u003cp\u003eMalignancy was identified as the leading cause of death in both DM and PM patients, with significantly elevated SMRs of 1.80 in DM patients. Poor survival outcome is noted in DM/PM patients with malignancies, where most mortality occurs within the first 5 years. Previous studies showed an increased cancer risk in patients with DM/PM and most cancers were diagnosed within 1 year of diagnosis of DM/PM [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e, \u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. In contrast to several previous DM/PM cohorts such as in Finland, Hungary and US which reported higher mortality due to cardiovascular complications [\u003cspan additionalcitationids=\"CR16\" citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e\u0026ndash;\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e]. This could be due to ethinicity and regional difference, where western population more commonly had cardiovascular risk factors [\u003cspan citationid=\"CR18\" class=\"CitationRef\"\u003e18\u003c/span\u003e].\u003c/p\u003e\u003cp\u003eWe also found that malignancy and major infections were independent prognostic factor of mortality in patients with DM/PM. Pneumonia was found to have two-and-a-half-fold increased mortality risk in patients with DM/PM compared to the general populations. Lung complications such as interstitial lung diseases are common in dermatomyositis and polymyositis patients, while cancer treatments and immunosuppressant uses expose patients to higher risk of infection [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e, \u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e]. These factors combined, major infections such as pneumonia in patients with DM/PM brings higher mortality risks compared to the general populations.\u003c/p\u003e\u003cp\u003eCompared to general population, disease of heart had significantly higher SMR in patients with polymyositis, which is consistent with prior research showing cardiac complications such as arrhythmias, myocarditis and heart failure in PM [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e, \u003cspan citationid=\"CR19\" class=\"CitationRef\"\u003e19\u003c/span\u003e, \u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e]. Cardiac involvement in patients with PM are not uncommon, including complications such as ischemic heart disease, myocardial infarction, heart failure, cardiac arrhythmia, pericardial effusions and even pulmonary hypertensions [\u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e, \u003cspan citationid=\"CR20\" class=\"CitationRef\"\u003e20\u003c/span\u003e]. Chronic inflammation from DM/PM may also damage the myocardium causing further cardiac complications. Additionally, corticosteroid use and reduced physical activity from muscle weakness may exacerbate cardiovascular risk factors like hypertension and dyslipidemia, further contributing to mortality. Proactive cardiac surveillance in PM management is needed to improve the mortality condition.\u003c/p\u003e\u003cp\u003eWhile the overall prevalence and demographic characteristics of dermatomyositis (DM) and polymyositis (PM), such as age and sex distribution, appear similar across Western and Asian populations, significant disparities exist in disease subtypes, associated malignancies, and autoantibody profiles [\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e]. Notably, DM is more prevalent in Asia compared to PM, and while the overall cancer risk is comparable, Asian patients exhibit a higher incidence of nasopharyngeal and lung cancers, in contrast to the ovarian, lung, gastric, and colorectal cancers more commonly observed in Western cohorts [\u003cspan citationid=\"CR21\" class=\"CitationRef\"\u003e21\u003c/span\u003e, \u003cspan citationid=\"CR22\" class=\"CitationRef\"\u003e22\u003c/span\u003e]. These regional differences also extend to the prevalence of specific autoantibodies (e.g., anti-p155/140 in Asia versus anti-Jo-1 in the West) and a higher incidence of interstitial lung disease in Asian DM/PM patients [\u003cspan citationid=\"CR23\" class=\"CitationRef\"\u003e23\u003c/span\u003e]. These differences underscored the importance of ethnicity-specific considerations in clinical screening and management strategies.\u003c/p\u003e\u003cp\u003eThis study highlights that patient under 45 years of age have markedly higher SMRs compared to the general population, with SMRs of 6.36 for males and 17.94 for females, which was similarly reported in the study by Kridin et al [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. This finding is striking because it suggests that despite the relatively low crude mortality rates in younger patients, the relative risk of death compared to the general population is disproportionately high. Potential causes for this significant disparities include delayed diagnosis, more aggressive disease phenotypes or complications like malignancy or infections that may disproportionately affect younger patients with DM/PM, compared to the general populations. This finding underscores the need for heightened clinical vigilance and tailored management strategies for reduction of this significant mortality risk posed upon the younger DM/PM patients.\u003c/p\u003e\u003cp\u003eInterestingly, we found that older age of onset and male gender were both independent prognostic factor of mortality in DM patients in the Cox regression analysis. Male sex was also previously reported as a risk factor of mortality in China and Italy [\u003cspan citationid=\"CR24\" class=\"CitationRef\"\u003e24\u003c/span\u003e, \u003cspan citationid=\"CR25\" class=\"CitationRef\"\u003e25\u003c/span\u003e]. Previous study showed that male patients had higher prevelance of severe interstitial lung disease as well as rapidly progressive interstitial lung disease, as well as higher inflammatory biomarkers than the female patients [\u003cspan citationid=\"CR26\" class=\"CitationRef\"\u003e26\u003c/span\u003e]. A group in Japan showed that androgen deficiency promotes development of pulmonary emphysema, and androgen replacement therapy reverses this condition in mice [\u003cspan citationid=\"CR27\" class=\"CitationRef\"\u003e27\u003c/span\u003e]. This suggests sex hormones may play a role in the disease process of interstitial lung disease and inflammatory myositis.\u003c/p\u003e"},{"header":"Limitations","content":"\u003cp\u003eThis study was conducted in the largest tertiary referral medical center in Taiwan and included a relatively large sample size of DM/PM patients with long observational period (19 years). We used the National Death Registry data to have a comprehensive overview of mortality causes and patterns in DM/PM patients across Taiwan, which allowed us to examine SMR and survival outcomes in detail. However, several limitations in this study needed to be addressed. Firstly, relying on National Death Registry data for analysis, potential coding errors in cause-of-death reporting may be present which could affect the accuracy of analysis. Interstitial lung disease is not recorded as a cause-of-death in the National Death Registry and such data could not be retrieved for analysis. Secondly, a cohort of myositis patients in a single center tertiary referral center may not be fully representative of all DM/PM patients in Taiwan and may likely to include a higher proportion of more severe or complicated cases. Thirdly, we did not include patients with amyopathic dermatomyositis and inclusion body myositis due to ICD diagnostic coding constraints. Lastly, due to the retrospective nature of our study, we were unable to collect complete clinical data such as use of immunosuppressive agents and rate of smoking in the enrolled patients, which are important factors affecting the mortality and morbidity outcomes of these patients.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eOur study provides valuable insights into the detailed mortality situation of DM/PM patients in Taiwan. High mortality rate is seen in DM/PM patients, and infection and malignancy are the leading cause of death. Older age of onset, male, presence of malignancy and major infections were all poor prognosis factors of mortality. Vigilant surveillance for malignancy and steps to mitigate aspiration risk are important to reduce mortality in this population. Further study in characteristics of different autoantibody disease presentation and treatment modalities may help improve care and outcome in patients with inflammatory myopathies.\u003c/p\u003e"},{"header":"Materials and Methods","content":"\u003cp\u003eStudy subject:\u003c/p\u003e\u003cp\u003eThe patient cohort and data collection methods for this study are the same as those described in our previous publication regarding malignancies in patients with DM or PM, which we described as follows [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. We conducted a registry analysis using electronic medical records (EMRs) from the outpatient clinic and admission data from a tertiary medical center (the largest hospital in Taiwan) from January 2001 to May 2019. EMRs included demographic data, dates of clinical visits, diagnostic codes, and details of examinations. This study was based in part on data from the Chang Gung Research Database provided by Chang Gung Memorial Hospital. Subjects below 18 years of age were excluded. Diagnoses of DM and PM were based on the Bohan and Peter diagnostic criteria. The study protocol was approved by the Chang Gung Memorial Hospital Institutional Review Board approved the waiver of the participants\u0026rsquo; consent (CGMHIRB201901511B0). All methods were performed in accordance with the relevant guidelines and regulations.\u003c/p\u003e\u003cp\u003eDiagnostic coding:\u003c/p\u003e\u003cp\u003ePatients with DM with the International Classification of Diseases (ICD), ninth revision (ICD-9) code 7103 and ICD, tenth revision (ICD-10) codes M33, M3310, M3319, M3390, M3399, and M360 or PM (or ICD-9 code 7104; or ICD-10 codes M3320 or M3329) were recruited. Amyopathic DM and inclusion body myositis were not identified due to a lack of specific diagnostic codes. To study the associated comorbidities, we selected diabetes mellitus (ICD-9: 250; ICD-10: I110, I101, I109, I110, I111, and I119), and hypertension (ICD-9: 401 and 402; ICD-10: I10-I16).\u003c/p\u003e\u003cp\u003eOutcome measures:\u003c/p\u003e\u003cp\u003ePatients were linked to the National Death Registry using their unique identification number. The date of death and cause of death were extracted, with causes coded by ICD-9 and ICD-10. All patients were followed from the index date until the date of death or May 31st, 2019, whichever came first.\u003c/p\u003e\u003cdiv id=\"Sec12\" class=\"Section2\"\u003e\u003ch2\u003eStatistical analysis:\u003c/h2\u003e\u003cp\u003eAll statistical analyses were performed using SPSS (version 21.0; IBM, New York, USA). Continuous variables are expressed as the means\u0026thinsp;\u0026plusmn;\u0026thinsp;standard deviations. Categorical variables are presented as numbers and ratios. The standardized mortality ratio (SMR) was used to compare the mortality of patients with DM/PM to the general population of Taiwan. The top 10 causes of death that are present in at least 9 years of the study period were used to calculate expected mortality rates. Kaplan-Meier analysis was used for to compare the survival probability in patients with DM or PM, with or without malignancy and major infection. A major infection was defined as one that necessitated the use of intravenous and/or prolonged course of antimicrobial treatment for more than 1 week. A Cox regression model was used to examine the hazard ratios of risk factors of mortality in patients with DM and PM. Statistical significance was defined as P\u0026thinsp;\u0026lt;\u0026thinsp;0.05.\u003c/p\u003e\u003c/div\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003ch2\u003eAuthor contributions statement\u003c/h2\u003e\u003cp\u003eLung-Sun Ro and Jung Lung Hsu conceived the idea. Jung Lung Hsu, Ming-Feng Liao, Chun-Che Chu, Hung-Chou Kuo, Mei-Yun Cheng, Mei-Lan Chen collected and checked the data. Pin-Hsuan Huang performed the data analysis. Gin Hoong Lee wrote the manuscript. Jung Lung Hsu revised the manuscript. All authors reviewed the manuscript and approved the final version of the manuscript.\u003c/p\u003e\u003c/p\u003e\u003cp\u003e\u003ch2\u003eCompeting interests statement\u003c/h2\u003e\u003cp\u003eThe authors declare no competing interests.\u003c/p\u003e\u003c/p\u003e\u003ch2\u003eFundings\u003c/h2\u003e\u003cp\u003eThe authors declared no funding support for this research.\u003c/p\u003e\u003ch2\u003eAuthor Contribution\u003c/h2\u003e\u003cp\u003eLung-Sun Ro and Jung Lung Hsu conceived the idea. Jung Lung Hsu, Ming-Feng Liao, Chun-Che Chu, Hung-Chou Kuo, Mei-Yun Cheng, Mei-Lan Chen collected and checked the data. Pin-Hsuan Huang performed the data analysis. Gin Hoong Lee wrote the manuscript. Jung Lung Hsu revised the manuscript. All authors reviewed the manuscript and approved the final version of the manuscript.\u003c/p\u003e\u003ch2\u003eAcknowledgement\u003c/h2\u003e\u003cp\u003eThe authors thank Ping-Hsuan Huang for the statistical assistance and wish to acknowledge the support of the Maintenance Project of the Center for Big Data Analytics and Statistics (Grant: CLRPG3N0011) at Chang Gung Memorial Hospital for study design and monitor, data analysis and interpretation.\u003c/p\u003e\u003ch2\u003eData Availability\u003c/h2\u003e\u003cp\u003eAll data and codes generated used in this study will be available upon request to the corresponding author.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eDalakas, M. C. \u0026amp; Hohlfeld, R. Polymyositis and dermatomyositis. \u003cem\u003eLancet\u003c/em\u003e \u003cb\u003e362\u003c/b\u003e (9388), 971\u0026ndash;982 (2003).\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eHsu, J. L. et al. 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H. et al. Survival analysis of patients with dermatomyositis and polymyositis: analysis of 192 Chinese cases. \u003cem\u003eClin. Rheumatol.\u003c/em\u003e \u003cb\u003e30\u003c/b\u003e (12), 1595\u0026ndash;1601 (2011).\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eCobos, G. A., Femia, A. \u0026amp; Vleugels, R. A. Dermatomyositis: An Update on Diagnosis and Treatment. \u003cem\u003eAm. J. Clin. Dermatol.\u003c/em\u003e \u003cb\u003e21\u003c/b\u003e (3), 339\u0026ndash;353 (2020).\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eGe, Y. P. et al. Infection is not rare in patients with idiopathic inflammatory myopathies. \u003cem\u003eClin. Exp. Rheumatol.\u003c/em\u003e \u003cb\u003e40\u003c/b\u003e (2), 254\u0026ndash;259 (2022).\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eAirio, A., Kautiainen, H. \u0026amp; Hakala, M. Prognosis and mortality of polymyositis and dermatomyositis patients. \u003cem\u003eClin. Rheumatol.\u003c/em\u003e \u003cb\u003e25\u003c/b\u003e (2), 234\u0026ndash;239 (2006).\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eSchiopu, E. et al. \u003cem\u003ePredictors of survival in a cohort of patients with polymyositis and dermatomyositis: effect of corticosteroids, methotrexate and azathioprine\u003c/em\u003e14p. R22 (Arthritis Research \u0026amp; Therapy, 2012). 1.\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eDank\u0026oacute;, K. et al. Long-term survival of patients with idiopathic inflammatory myopathies according to clinical features: a longitudinal study of 162 cases. \u003cem\u003eMed. (Baltim).\u003c/em\u003e \u003cb\u003e83\u003c/b\u003e (1), 35\u0026ndash;42 (2004).\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eIrawati, S. et al. Long-term incidence and risk factors of cardiovascular events in Asian populations: systematic review and meta-analysis of population-based cohort studies. \u003cem\u003eCurr. Med. Res. Opin.\u003c/em\u003e \u003cb\u003e35\u003c/b\u003e (2), 291\u0026ndash;299 (2019).\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eMaugars, Y. M. et al. Long-term prognosis of 69 patients with dermatomyositis or polymyositis. \u003cem\u003eClin. Exp. Rheumatol.\u003c/em\u003e \u003cb\u003e14\u003c/b\u003e (3), 263\u0026ndash;274 (1996).\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eLu, Z. et al. Cardiac Involvement in Adult Polymyositis or Dermatomyositis: A Systematic Review. \u003cem\u003eClin. Cardiol.\u003c/em\u003e \u003cb\u003e35\u003c/b\u003e (11), 685\u0026ndash;691 (2012).\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eUngprasert, P. et al. \u003cem\u003eClinical features of inflammatory myopathies and their association with malignancy: a systematic review in asian population.\u003c/em\u003e ISRN Rheumatol, 2013: p. 509354. (2013).\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eOhta, A. et al. Prevalence and incidence of polymyositis and dermatomyositis in Japan. \u003cem\u003eMod. Rheumatol.\u003c/em\u003e \u003cb\u003e24\u003c/b\u003e (3), 477\u0026ndash;480 (2014).\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eOpinc-Rosiak, A. H. \u0026amp; Makowska, J. S. Environmental exposures as risk factors for idiopathic inflammatory myopathies. \u003cem\u003eJ. Autoimmun.\u003c/em\u003e \u003cb\u003e140\u003c/b\u003e, 103095 (2023).\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eHe, S. et al. Differences in sex- and age-associated mortality in patients with anti-MDA5-positive dermatomyositis. \u003cem\u003eMod. Rheumatol.\u003c/em\u003e \u003cb\u003e33\u003c/b\u003e (5), 975\u0026ndash;981 (2022).\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eDanieli, M. G. et al. Impact of treatment on survival in polymyositis and dermatomyositis. A single-centre long-term follow-up study. \u003cem\u003eAutoimmun. Rev.\u003c/em\u003e \u003cb\u003e13\u003c/b\u003e (10), 1048\u0026ndash;1054 (2014).\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eCheng, L. et al. Gender differences in patients with anti-MDA5-positive dermatomyositis: a cohort study of 251 cases. \u003cem\u003eClin. Rheumatol.\u003c/em\u003e \u003cb\u003e43\u003c/b\u003e (1), 339\u0026ndash;347 (2024).\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eAono, K. et al. Testosterone deficiency promotes the development of pulmonary emphysema in orchiectomized mice exposed to elastase. \u003cem\u003eBiochem. Biophys. Res. Commun.\u003c/em\u003e \u003cb\u003e558\u003c/b\u003e, 94\u0026ndash;101 (2021).\u003c/span\u003e\u003c/li\u003e\u003cli\u003e\u003cspan\u003eAdditional \u0026amp; Information.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"},{"header":"Tables","content":"\u003cp\u003eTable 1. Standardized mortality ratio (SMR) of patients with dermatomyositis and polymyositis.\u003c/p\u003e\n\u003ctable border=\"1\" cellspacing=\"0\" cellpadding=\"0\" width=\"656\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eDermatomyositis\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eOverall\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u003cstrong\u003en = 1100\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eFemales\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u003cstrong\u003en = 705\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eMales\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u003cstrong\u003en = 395\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eMalignant neoplasms\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.80 (1.40 \u0026ndash; 2.19) *\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.42 (0.93 \u0026ndash; 1.91)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e2.46 (1.77 \u0026ndash; 3.16) *\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eDisease of heart\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.52 (0.93 \u0026ndash; 2.12)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.58 (0.72 \u0026ndash; 2.44)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.65 (0.72\u0026nbsp;\u0026ndash; 2.58)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003ePneumonia\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e2.46 (1.41 \u0026ndash; 3.51) *\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e2.19 (0.83 \u0026ndash; 3.55)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e2.76 (1.13 \u0026ndash; 4.40) *\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eCerebrovascular disease\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.08\u0026nbsp;(0.49 \u0026ndash; 1.67)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e0.99\u0026nbsp;(0.20 \u0026ndash; 1.79)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.27\u0026nbsp;(0.33 \u0026ndash; 2.21)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eDiabetes mellitus\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.34 (0.61 \u0026ndash; 2.07)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.94\u0026nbsp;(0.60 \u0026ndash; 3.29)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.05\u0026nbsp;(0.13 \u0026ndash; 1.97)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eChronic lower respiratory diseases\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e0.74\u0026nbsp;(0.01 \u0026ndash; 1.46)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e0.28\u0026nbsp;(0.00 \u0026ndash; 0.84) *\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.20\u0026nbsp;(0.00 \u0026ndash; 2.56)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eChronic liver disease and cirrhosis\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.68 (0.58 \u0026ndash; 2.77)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.48 (0.03 \u0026ndash; 2.93)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.26 (0.16 \u0026ndash; 2.37)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eNephritis, nephrotic syndrome and nephrosis\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.27 (0.25 \u0026ndash; 2.29)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.54 (0.03 \u0026ndash; 3.06)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.09 (0.00 \u0026ndash; 2.59)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eIntentional self-harm (suicide)\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.29 (0.03 \u0026ndash; 2.56)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.18 (0.00 \u0026ndash; 3.50)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e3.75 (0.00 \u0026ndash; 7.99)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eAccidents and adverse effects\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e0.57 (0.01 \u0026ndash; 1.13)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e0.50 (0.00 \u0026ndash; 1.19)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e0.67 (0.00 \u0026ndash; 1.60)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003ePolymyositis\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eOverall\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u003cstrong\u003en = 1164\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eFemales\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u003cstrong\u003en = 701\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eMales\u003c/strong\u003e\u003c/p\u003e\n \u003cp\u003e\u003cstrong\u003en = 463\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eMalignant neoplasms\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.15\u0026nbsp;(0.84 \u0026ndash; 1.46)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e0.72\u0026nbsp;(0.39 \u0026ndash; 1.05)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.82\u0026nbsp;(1.21 \u0026ndash; 2.42)\u0026nbsp;*\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eDisease of heart\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e2.48\u0026nbsp;(1.73 \u0026ndash; 3.23) *\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e2.31\u0026nbsp;(1.32 \u0026ndash; 3.30) *\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e2.95\u0026nbsp;(1.69 \u0026ndash; 4.21)\u0026nbsp;*\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003ePneumonia\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e2.74\u0026nbsp;(1.64 \u0026ndash; 3.83) *\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.59\u0026nbsp;(0.49 \u0026ndash; 2.68)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e4.11\u0026nbsp;(2.09 \u0026ndash; 6.12)\u0026nbsp;*\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eCerebrovascular diseases\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e0.56\u0026nbsp;(0.15 \u0026ndash; 0.98) *\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e0.15\u0026nbsp;(0.00 \u0026ndash; 0.44) *\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.11\u0026nbsp;(0.22\u0026nbsp;\u0026ndash; 2.00)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eDiabetes mellitus\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.30\u0026nbsp;(0.59 \u0026ndash; 2.01)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e2.16\u0026nbsp;(0.82 \u0026ndash; 3.50)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e0.64\u0026nbsp;(0.00 \u0026ndash; 1.37)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eChronic lower respiratory diseases\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e0.54\u0026nbsp;(0.00 \u0026ndash; 1.15)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e0.52\u0026nbsp;(0.00 \u0026ndash; 1.23)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e0.41\u0026nbsp;(0.00 \u0026ndash; 1.21)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eChronic liver disease and cirrhosis\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.24 (0.32 \u0026ndash; 2.17)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e0.98 (0.00 \u0026ndash; 2.09)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e1.03 (0.02 \u0026ndash; 2.03)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eNephritis, nephrotic syndrome and nephrosis\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e2.27 (0.93 \u0026ndash; 3.61)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e2.39 (0.62 \u0026ndash; 4.17)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e2.21 (0.04 \u0026ndash; 4.39)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eIntentional self-harm (suicide)\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e2.41 (0.74 \u0026ndash; 4.08)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e3.90 (0.08 \u0026ndash; 7.71)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e5.00 (0.10 \u0026ndash; 9.91)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 28.7671%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eAccidents and adverse effects\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e0.54 (0.01 \u0026ndash; 1.07)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e0.44 (0.00 \u0026ndash; 1.06)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 23.7443%;\"\u003e\n \u003cp\u003e0.68 (0.00 \u0026ndash; 1.62)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003e* indicates statistical significance at p \u0026lt; 0.05.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eTable 2. Causes of death in patients with dermatomyositis and polymyositis\u003c/p\u003e\n\u003ctable border=\"1\" cellspacing=\"0\" cellpadding=\"0\" width=\"643\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 32.3484%;\"\u003e\n \u003cp\u003eCause of death\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 27.9938%;\"\u003e\n \u003cp\u003eDermatomyositis n (%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 22.084%;\"\u003e\n \u003cp\u003ePolymyositis n (%)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 17.5739%;\"\u003e\n \u003cp\u003eOverall n (%)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 32.3484%;\"\u003e\n \u003cp\u003eTotal death number\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 27.9938%;\"\u003e\n \u003cp\u003e319 (100)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 22.084%;\"\u003e\n \u003cp\u003e316 (100)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 17.5739%;\"\u003e\n \u003cp\u003e635 (100)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 32.3484%;\"\u003e\n \u003cp\u003eMalignant neoplasm\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 27.9938%;\"\u003e\n \u003cp\u003e80 (25.1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.084%;\"\u003e\n \u003cp\u003e53 (16.8)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.5739%;\"\u003e\n \u003cp\u003e133 (20.9)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 32.3484%;\"\u003e\n \u003cp\u003eDiseases of the musculoskeletal system and connective tissue\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 27.9938%;\"\u003e\n \u003cp\u003e48 (15.1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.084%;\"\u003e\n \u003cp\u003e31 (9.8)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.5739%;\"\u003e\n \u003cp\u003e79 (12.4)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 32.3484%;\"\u003e\n \u003cp\u003eDiseases of heart (except hypertensive diseases)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 27.9938%;\"\u003e\n \u003cp\u003e25 (7.8)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.084%;\"\u003e\n \u003cp\u003e42 (13.3)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.5739%;\"\u003e\n \u003cp\u003e67 (10.6)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 32.3484%;\"\u003e\n \u003cp\u003ePneumonia\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 27.9938%;\"\u003e\n \u003cp\u003e21 (6.6)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.084%;\"\u003e\n \u003cp\u003e24 (7.6)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.5739%;\"\u003e\n \u003cp\u003e45 (7.1)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 32.3484%;\"\u003e\n \u003cp\u003eDiabetes Mellitus\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 27.9938%;\"\u003e\n \u003cp\u003e13 (4.1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.084%;\"\u003e\n \u003cp\u003e13 (4.1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.5739%;\"\u003e\n \u003cp\u003e26 (4.1)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 32.3484%;\"\u003e\n \u003cp\u003eCerebrovascular diseases\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 27.9938%;\"\u003e\n \u003cp\u003e13 (4.1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.084%;\"\u003e\n \u003cp\u003e7 (2.2)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.5739%;\"\u003e\n \u003cp\u003e20 (3.2)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 32.3484%;\"\u003e\n \u003cp\u003eNephritis, nephrotic syndrome and nephrosis\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 27.9938%;\"\u003e\n \u003cp\u003e6 (1.9)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.084%;\"\u003e\n \u003cp\u003e11(3.5)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.5739%;\"\u003e\n \u003cp\u003e17 (2.7)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 32.3484%;\"\u003e\n \u003cp\u003eChronic liver disease and cirrhosis\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 27.9938%;\"\u003e\n \u003cp\u003e9 (2.8)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.084%;\"\u003e\n \u003cp\u003e7 (2.2)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.5739%;\"\u003e\n \u003cp\u003e16 (2.5)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 32.3484%;\"\u003e\n \u003cp\u003eSuicide\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 27.9938%;\"\u003e\n \u003cp\u003e4 (1.3)\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.084%;\"\u003e\n \u003cp\u003e8 (2.5)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.5739%;\"\u003e\n \u003cp\u003e12 (1.9)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 32.3484%;\"\u003e\n \u003cp\u003eChronic lower respiratory diseases\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 27.9938%;\"\u003e\n \u003cp\u003e4 (1.3)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.084%;\"\u003e\n \u003cp\u003e3 (0.9)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.5739%;\"\u003e\n \u003cp\u003e7 (1.1)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 32.3484%;\"\u003e\n \u003cp\u003eAccidents and adverse effects\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 27.9938%;\"\u003e\n \u003cp\u003e4 (1.3)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.084%;\"\u003e\n \u003cp\u003e4 (1.3)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.5739%;\"\u003e\n \u003cp\u003e8 (1.3)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 32.3484%;\"\u003e\n \u003cp\u003eOthers\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 27.9938%;\"\u003e\n \u003cp\u003e92 (28.8)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.084%;\"\u003e\n \u003cp\u003e113 (35.8)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.5739%;\"\u003e\n \u003cp\u003e205 (32.3)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003eData are number (%)\u003c/p\u003e\n\u003ch3\u003eTable 3. Observed and expected deaths and standardized mortality ratios in patient with dermatomyositis/polymyositis stratified by age and sex at incidence.\u0026nbsp;\u003c/h3\u003e\n\u003ctable border=\"0\" cellspacing=\"0\" cellpadding=\"0\" width=\"623\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 20%;\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20%;\"\u003e\n \u003cp\u003e\u003cstrong\u003ePerson-years\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eObserved deaths (rate per 1000)\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 17.28%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eExpected deaths\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 22.72%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eSMR (95% CI)\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 20%;\"\u003e\n \u003cp\u003eOverall\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20%;\"\u003e\n \u003cp\u003e21991.32\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20%;\"\u003e\n \u003cp\u003e635 (28.88)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.28%;\"\u003e\n \u003cp\u003e241.73\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.72%;\"\u003e\n \u003cp\u003e2.63 (2.42-2.83)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 20%;\"\u003e\n \u003cp\u003eMales\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20%;\"\u003e\n \u003cp\u003e7983.55\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20%;\"\u003e\n \u003cp\u003e293 (36.7)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.28%;\"\u003e\n \u003cp\u003e108.81\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.72%;\"\u003e\n \u003cp\u003e2.69 (2.38-3.00)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 20%;\"\u003e\n \u003cp\u003e\u0026lt;45\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20%;\"\u003e\n \u003cp\u003e3918.5\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20%;\"\u003e\n \u003cp\u003e46 (11.74)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.28%;\"\u003e\n \u003cp\u003e7.23\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.72%;\"\u003e\n \u003cp\u003e6.36 (4.52-8.2.0)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 20%;\"\u003e\n \u003cp\u003e45-64\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20%;\"\u003e\n \u003cp\u003e2988.57\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20%;\"\u003e\n \u003cp\u003e125 (41.83)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.28%;\"\u003e\n \u003cp\u003e44.04\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.72%;\"\u003e\n \u003cp\u003e2.84 (2.34-3.34)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 20%;\"\u003e\n \u003cp\u003e≧65\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20%;\"\u003e\n \u003cp\u003e1076.45\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20%;\"\u003e\n \u003cp\u003e122 (113.34)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.28%;\"\u003e\n \u003cp\u003e111.33\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.72%;\"\u003e\n \u003cp\u003e1.10 (0.90-1.29)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 20%;\"\u003e\n \u003cp\u003eFemales\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20%;\"\u003e\n \u003cp\u003e14007.77\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20%;\"\u003e\n \u003cp\u003e342 (24.42)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.28%;\"\u003e\n \u003cp\u003e107.05\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.72%;\"\u003e\n \u003cp\u003e3.19 (2.86-3.53)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 20%;\"\u003e\n \u003cp\u003e\u0026lt;45\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20%;\"\u003e\n \u003cp\u003e6779.65\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20%;\"\u003e\n \u003cp\u003e69 (10.18)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.28%;\"\u003e\n \u003cp\u003e3.85\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.72%;\"\u003e\n \u003cp\u003e17.94 (13.71-22.18)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 20%;\"\u003e\n \u003cp\u003e45-64\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20%;\"\u003e\n \u003cp\u003e5917.74\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20%;\"\u003e\n \u003cp\u003e160 (27.04)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.28%;\"\u003e\n \u003cp\u003e34.93\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.72%;\"\u003e\n \u003cp\u003e4.58 (3.87-5.29)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd style=\"width: 20%;\"\u003e\n \u003cp\u003e≧65\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 20%;\"\u003e\n \u003cp\u003e1310.38\u0026nbsp;\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20%;\"\u003e\n \u003cp\u003e113 (86.23)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 17.28%;\"\u003e\n \u003cp\u003e91.55\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 22.72%;\"\u003e\n \u003cp\u003e1.23 (1.01-1.46)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003eAbbreviations: SMR, standardized mortality ratio; CI, confidence interval.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eTable 4. Estimated overall survival following the diagnosis of dermatomyositis and polymyositis.\u003c/p\u003e\n\u003ctable border=\"1\" cellspacing=\"0\" cellpadding=\"0\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd valign=\"bottom\" style=\"width: 19.6013%;\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 19.6013%;\"\u003e\n \u003cp\u003e\u003cstrong\u003e1-year survival, % (95% CI)\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 18.9369%;\"\u003e\n \u003cp\u003e\u003cstrong\u003e5-year survival, % (95% CI)\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20.9302%;\"\u003e\n \u003cp\u003e\u003cstrong\u003e10-year survival, % (95% CI)\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20.9302%;\"\u003e\n \u003cp\u003e\u003cstrong\u003e15-year survival, % (95% CI)\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"bottom\" style=\"width: 19.6013%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eOverall\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 19.6013%;\"\u003e\n \u003cp\u003e91.8 (90.6-92.9)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 18.9369%;\"\u003e\n \u003cp\u003e82.0 (80.3-83.5)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20.9302%;\"\u003e\n \u003cp\u003e75.3 (73.3-77.1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20.9302%;\"\u003e\n \u003cp\u003e67.9 (65.6-70.1)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"bottom\" style=\"width: 19.6013%;\"\u003e\n \u003cp\u003e\u003cstrong\u003ePolymyositis\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 19.6013%;\"\u003e\n \u003cp\u003e93.4 (91.8-94.7)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 18.9369%;\"\u003e\n \u003cp\u003e84.4 (82.2-86.4)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20.9302%;\"\u003e\n \u003cp\u003e77.0 (74.3-79.5)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20.9302%;\"\u003e\n \u003cp\u003e68.8 (65.5-71.8)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"bottom\" style=\"width: 19.6013%;\"\u003e\n \u003cp\u003e\u003cstrong\u003eDermatomyositis\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 19.6013%;\"\u003e\n \u003cp\u003e90.2 (88.3-91.8)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 18.9369%;\"\u003e\n \u003cp\u003e79.4 (76.8-81.7)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20.9302%;\"\u003e\n \u003cp\u003e73.4 (70.5-76.1)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 20.9302%;\"\u003e\n \u003cp\u003e67.1 (63.7-70.2)\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003eAbbreviations: CI, confidence interval.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eTable 5. Cox regression analyses of risk factors in the dermatomyositis and polymyositis patients.\u003c/p\u003e\n\u003ctable border=\"1\" cellspacing=\"0\" cellpadding=\"0\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd rowspan=\"2\" valign=\"top\" style=\"width: 125px;\"\u003e\n \u003cp\u003eUnivariate analysis\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd colspan=\"2\" valign=\"top\" style=\"width: 221px;\"\u003e\n \u003cp\u003eDermatomyositis\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd colspan=\"2\" valign=\"top\" style=\"width: 217px;\"\u003e\n \u003cp\u003ePolymyositis\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 125px;\"\u003e\n \u003cp\u003eHR (95% CI)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 97px;\"\u003e\n \u003cp\u003eP value\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 121px;\"\u003e\n \u003cp\u003eHR (95% CI)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 96px;\"\u003e\n \u003cp\u003eP value\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"bottom\" style=\"width: 125px;\"\u003e\n \u003cp\u003eAge at onset\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 125px;\"\u003e\n \u003cp\u003e1.05 (1.05 - 1.06)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 97px;\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026lt;0.0001\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 121px;\"\u003e\n \u003cp\u003e1.06 (1.05 - 1.07)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 96px;\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026lt;0.0001\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"bottom\" style=\"width: 125px;\"\u003e\n \u003cp\u003eGender(M)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 125px;\"\u003e\n \u003cp\u003e1.54 (1.23 - 1.94)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 97px;\"\u003e\n \u003cp\u003e\u003cstrong\u003e0.0002\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 121px;\"\u003e\n \u003cp\u003e1.41 (1.12 - 1.78)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 96px;\"\u003e\n \u003cp\u003e\u003cstrong\u003e0.0031\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"bottom\" style=\"width: 125px;\"\u003e\n \u003cp\u003eMalignancy\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 125px;\"\u003e\n \u003cp\u003e2.81 (2.01 - 3.93)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 97px;\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026lt;0.0001\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 121px;\"\u003e\n \u003cp\u003e3.03 (2.00 - 4.60)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 96px;\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026lt;0.0001\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"bottom\" style=\"width: 125px;\"\u003e\n \u003cp\u003eHypertension\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 125px;\"\u003e\n \u003cp\u003e1.43 (1.13 - 1.81)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 97px;\"\u003e\n \u003cp\u003e\u003cstrong\u003e0.0028\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 121px;\"\u003e\n \u003cp\u003e1.54 (1.22 - 1.95)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 96px;\"\u003e\n \u003cp\u003e\u003cstrong\u003e0.0003\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 125px;\"\u003e\n \u003cp\u003eInfection\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 125px;\"\u003e\n \u003cp\u003e1.81 (1.44 - 2.29)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 97px;\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026lt;0.0001\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 121px;\"\u003e\n \u003cp\u003e1.63 (1.30 - 2.05)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 96px;\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026lt;0.0001\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003ctable border=\"1\" cellspacing=\"0\" cellpadding=\"0\"\u003e\n \u003ctbody\u003e\n \u003ctr\u003e\n \u003ctd rowspan=\"2\" valign=\"top\" style=\"width: 125px;\"\u003e\n \u003cp\u003eStratified multivariate analysis\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd colspan=\"2\" valign=\"top\" style=\"width: 221px;\"\u003e\n \u003cp\u003eDermatomyositis\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd colspan=\"2\" valign=\"top\" style=\"width: 217px;\"\u003e\n \u003cp\u003ePolymyositis\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 125px;\"\u003e\n \u003cp\u003eHR (95% CI)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 97px;\"\u003e\n \u003cp\u003eP value\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 121px;\"\u003e\n \u003cp\u003eHR (95% CI)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 96px;\"\u003e\n \u003cp\u003eP value\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"bottom\" style=\"width: 125px;\"\u003e\n \u003cp\u003eAge at onset\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 125px;\"\u003e\n \u003cp\u003e1.05 (1.05 - 1.06)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 97px;\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026lt;0.0001\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 121px;\"\u003e\n \u003cp\u003e1.06 (1.05 - 1.07)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 96px;\"\u003e\n \u003cp\u003e\u003cstrong\u003e\u0026lt;0.0001\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"bottom\" style=\"width: 125px;\"\u003e\n \u003cp\u003eGender(M)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 125px;\"\u003e\n \u003cp\u003e1.40 (1.11 - 1.76)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 97px;\"\u003e\n \u003cp\u003e\u003cstrong\u003e0.0041\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 121px;\"\u003e\n \u003cp\u003e1.14 (0.90 - 1.44)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 96px;\"\u003e\n \u003cp\u003e0.2687\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"bottom\" style=\"width: 125px;\"\u003e\n \u003cp\u003eMalignancy\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 125px;\"\u003e\n \u003cp\u003e1.40 (0.98 \u0026ndash; 2.00)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 97px;\"\u003e\n \u003cp\u003e0.0664\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 121px;\"\u003e\n \u003cp\u003e1.67 (1.08 - 2.58)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 96px;\"\u003e\n \u003cp\u003e\u003cstrong\u003e0.022\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003ctr\u003e\n \u003ctd valign=\"top\" style=\"width: 125px;\"\u003e\n \u003cp\u003eInfection\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 125px;\"\u003e\n \u003cp\u003e1.54 (1.21 - 1.98)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 97px;\"\u003e\n \u003cp\u003e\u003cstrong\u003e0.0005\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"bottom\" style=\"width: 121px;\"\u003e\n \u003cp\u003e1.41 (1.10 - 1.80)\u003c/p\u003e\n \u003c/td\u003e\n \u003ctd valign=\"top\" style=\"width: 96px;\"\u003e\n \u003cp\u003e\u003cstrong\u003e0.006\u003c/strong\u003e\u003c/p\u003e\n \u003c/td\u003e\n \u003c/tr\u003e\n \u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003eBold denotes statistical significance.\u003c/p\u003e\n\u003cp\u003eAbbreviations: HR, hazard ratio; CI, confidence interval.\u0026nbsp;\u003c/p\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"","lastPublishedDoi":"10.21203/rs.3.rs-7485056/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-7485056/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003eDermatomyositis (DM) and polymyositis (PM) are rare idiopathic inflammatory myopathies associated with substantial morbidity and mortality. However, data on mortality patterns and prognostic factors in Asian populations, particularly in Taiwan, remain limited. We conducted a retrospective cohort study of 2,264 adult patients diagnosed with DM or PM between 2001 and 2019 using electronic medical records from the largest tertiary referral center in Taiwan. Mortality data were linked with the National Death Registry, and standardized mortality ratios (SMRs) were calculated relative to the general population. During 21,991 person-years of follow-up, 635 deaths were recorded (319 DM, 316 PM), with 5-year mortality rate of 18.0%. Malignancy and pneumonia were the leading causes of death, with significantly elevated SMRs. Patients diagnosed before age 45 had the highest relative risk of death, with SMRs of 6.36 for males and 17.94 for females. Survival analysis revealed significantly reduced survival in patients with malignancies or major infections (log-rank p\u0026thinsp;\u0026lt;\u0026thinsp;0.0001). Cox regression identified older age at onset, male sex, malignancy, and major infection as independent risk factors for mortality. These findings highlight the persistently high mortality burden in DM and PM patients in Taiwan and underscore the need for early malignancy screening, infection prevention, and tailored management strategies to improve long-term outcomes.\u003c/p\u003e","manuscriptTitle":"Survival Trends and Causes of Death in Dermatomyositis and Polymyositis: A 19-year Longitudinal Study in Taiwan","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-10-23 02:45:04","doi":"10.21203/rs.3.rs-7485056/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"be123ce4-310f-4eb1-a1fb-f755f6ec550d","owner":[],"postedDate":"October 23rd, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[{"id":56625097,"name":"Biological sciences/Cancer"},{"id":56625098,"name":"Health sciences/Diseases"},{"id":56625099,"name":"Health sciences/Medical research"},{"id":56625100,"name":"Health sciences/Oncology"},{"id":56625101,"name":"Health sciences/Risk factors"}],"tags":[],"updatedAt":"2025-10-29T10:23:53+00:00","versionOfRecord":[],"versionCreatedAt":"2025-10-23 02:45:04","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-7485056","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-7485056","identity":"rs-7485056","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}