Juvenile Idiopathic Inflammatory Myopathy and Juvenile Dermatomyositis: Characteristics and outcomes of affected children at a Paediatric Rheumatology Clinic in a tropical climate.

Juvenile Idiopathic Inflammatory Myopathy and Juvenile Dermatomyositis: Characteristics and outcomes of affected children at a Paediatric Rheumatology Clinic in a tropical climate. | 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 Research Article Juvenile Idiopathic Inflammatory Myopathy and Juvenile Dermatomyositis: Characteristics and outcomes of affected children at a Paediatric Rheumatology Clinic in a tropical climate. Zafir Wasim Latchan, Shanice Ali, Patrick Chin-Kong, Haramnauth Dyaanand This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-1764777/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 Background Juvenile Idiopathic Inflammatory Myopathy (JIIM) is a rare autoimmune condition of which Juvenile Dermatomyositis (JDM) is the most common subtype. To this date there has been no published data focusing on this condition in Trinidad nor the English-speaking Caribbean. Methods A retrospective folder analysis was done on patients with JIIM who attended the recently formed Paediatric Rheumatology Multi-Disciplinary Clinic over a 6 months period from May 2021 at San Fernando Teaching Hospital, Trinidad and Tobago. Demographics, investigations and diagnoses were analysed. A secondary comparative analysis of two diagnostic criteria was performed and treatment approach was examined. Results 5 notes were reviewed in this period. The male: female ratio was 1:4 and the average age of onset was 6.8 years. 80% of JIIM cases were JDM. The most common presenting complaints were: skin rash and weakness (62%). The most common additional symptoms were: joint pain (60%) and pruritis (60%). Previous upper respiratory tract infection was recognized in 40% of JIIM patients and one patient had Kawasaki disease 6 years prior. Creatine Kinase showed modest elevations in JDM, whilst it was extremely elevated in one patient with myositis. There was an 80% concordance between the EULAR/ACR 2017 and the Peter and Bohan 1975 criteria. 60% tested positive for ANA. Anti PM, Anti Ku, Anti RP155, Anti Mi2A, Anti Mi2B and Anti RP11 antibodies were identified. 40% were positive for AntiRP155. 60% of patients utilized MRI imaging which detected myositis in all reports. Methotrexate and prednisolone were used in 100%. Parenteral drugs used included: intravenous immunoglobulin and methylprednisolone. 80% had vitamin D supplementation and 100% were prescribed physiotherapy. One child had intractable calcinosis. Conclusions Whilst the study size was arguably small, patterns were seen which are in keeping with international data on epidemiology and treatment. Recommendations from this study include: greater utilization of MRI in diagnostic workup, further evaluating anti RP155 antibody as a new myositis associated antibody in a Caribbean population and exploring further options for calcinosis treatment in a developing nation. Juvenile Idiopathic Inflammatory Myopathy Juvenile Dermatomyositis Trinidad Figures Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Figure 7 Figure 8 Figure 9 Introduction: Juvenile Idiopathic Inflammatory Myopathy (JIIM) is considered a group of rare autoimmune diseases characterised by muscle weakness and characteristic skin rashes. (1) Of the various subtypes of JIIM, Juvenile Dermatomyositis (JDM) is the most common and accounts for approximately 80% of all JIIM. (1) The incidence reported in the literature is estimated to be 2–3 per million. (1) Over the last two decades, there has been a magnitude of research into causation, associations and pathophysiology of JDM. (1,2,3) The current thinking is environment plays a key role in a genetically susceptible host. (2) There is evidence of an inverse relationship of latitude and prevalence of JDM in European cities. (3) However, there is no data on prevalence in a Caribbean population to corroborate this finding in our latitude. In fact, there is currently no published evidence on Juvenile Idiopathic Inflammatory Myopathy and Juvenile Dermatomyositis in Trinidad and Tobago, or in the English-speaking Caribbean. In our small twin island of Trinidad and Tobago, there are five Regional Health Authorities serving a population of 1.4 million people. They are: North West, North Central, Eastern, South West and the Tobago Regional Health Authorities. The South West Regional Health Authority (SWRHA) serves an estimated catchment of 600 000 people and there is one tertiary centre for paediatrics. Of note, there are no double qualified Paediatric Rheumatologists at present. As such, all paediatric rheumatology patients are seen under General Paediatrics with consultation and input from the Adult Rheumatology service. In February of 2021, a decision was made to establish a Multi-Disciplinary Paediatric Rheumatology Clinic at SWRHA, with an aim to optimise care for these children. Here, children up to 18 years would be seen together by both Paediatric Medical and Adult Rheumatology with an aim to enhance the care for children with rheumatological conditions. Thus, the few paediatric rheumatology patients at the SWRHA have been brought into one clinic. Preliminary analysis of these patients revealed that together with lupus, JIIM had the highest number of cases in the clinic (unpublished data). This stimulated the idea of looking more closely at the JIIM/JDM population at this institution. Methodology: After gaining ethical approval from the Bioethics Committee of the South West Regional Health Authority; a retrospective folder review on the clinical characteristics of JIIM/JDM patients at the San Fernando Teaching Hospital over a 6-month period commenced. Inclusion criteria were: Children less than eighteen years, AND Attending the Paediatric Rheumatology Multi-Disciplinary Clinic of South West Regional Health Authority, AND A diagnosis of possible, probable or definite idiopathic inflammatory myopathy OR A diagnosis of possible, probable or definite juvenile dermatomyositis Patients’ notes alone were analysed who attended this clinic during the period from May to October 2021. The information was extracted and placed onto a Microsoft Excel Spreadsheet from which data analysis was done and descriptive statistics were reported. Demographic data, month of presentation and time lag between symptom onset and presentation were analysed. Additionally, examination findings, laboratory and diagnostic investigations were extracted from the notes. These were used in a secondary analysis to compare the Peter and Bohan criteria of 1975 with the European Union League Against Rheumatism/ American College of Rheumatology (EULAR/ACR) classification criteria of 2017. (4,5) The classification criteria were calculated with the assistance of an online calculator found at www.imm.ki.se/biostatistics/calculators/iim/ . (6) The number of relapses and also immunosuppressive drugs used in management of these children with JIIM and JDM at SWRHA were examined. In addition, utilization of physiotherapy, skin protection and vitamin d supplementation were described together with calcinosis and the treatment options utilized. We also assessed if patients had clinically inactive disease, at the last visit, based on a modified Paediatric Rheumatology INternational Trials Organisation (PRINTO) Criteria similar to Okong'o et al 's study from Cape Town. (7,8) Our criterion included a Childhood Myositis Assessment Score (CMAS) > 47 and a serum Creatinine Kinase > 150 U/L. Results: 5 notes were reviewed during this time. It must be highlighted that several gaps in data were present in the notes reviewed. Demographics: Of these five children with JIIM/JDM, 4 were female and one was male. No clusters were seen in terms of location of these children. See map below. The age at presentation ranged from 2 years to 11 years. Average age at presentation was 6.8years with a median of 6 years. Month of presentation: Whilst 2 patients had their first symptom in November, there was no observable pattern of month of presentation. The average time lag between first symptom and presentation was 3.8 months. Table 1 Month of first symptom Month of presentation Time lag to presentation November May 6 months August September 1 month November December 1 month May July 2 months April January 9 months Presenting complaint: The most common presenting complaint were skin rash and weakness which accounted for 62% of all presenting complaints of these children who had JIIM/JDM followed by joint pains, fever and weight loss. The most common additional symptom was joint pain (3/5) and pruritis (3/5). Followed by fever (2/5) and fatigue (2/5). One patient with 5 additional symptom was male and presented at 2 years of age. Examination findings at diagnosis: 4 out of 5 children had some documentation of muscle weakness on examination. In terms of skin findings, 4 out of 5 had documented pathognomonic changes of JDM. Gottron’s papule was the most common pathognomonic (50%) skin finding in these 4 children with JDM. Environmental factors and genetics: In terms of environmental factors, 2 out of 5 had previous infections in the 6 months prior to presentation. 1 out of 5 had the potential stressor of entering final year of primary school. 3 out of 5 had been fully vaccinated; however, we were unable to ascertain if any were given in the 6 months prior to presentation. Additionally, 4 out of 5 had previous medical conditions which included Kawasaki disease. 1 patient had a positive family history of autoimmune disease. Table 2 Past Medical History Family history Kawasaki disease 6 years prior Nephrotic Syndrome Lichen striatus one year prior Rheumatoid Arthritis Lower respiratory tract infection at birth and constipation Sickle cell Trait Asthma Laboratory investigations at diagnosis- muscle enzymes: In terms of muscle enzymes at time of diagnosis, Creatinine Kinase (CK) and Lactate Dehydrogenase (LDH) were the most elevated muscle enzyme at presentation. Alanine transferase (ALT) was the least elevated of all the muscle enzymes in any of these children. Two patients had missing LDH in the notes. Autoantibodies: With respect to Autoantibodies, 3 out of 5 had Anti-Nuclear Antibody (ANA) positivity documented. 2 out of 5 had documented Myositis Specific Antibodies sent off- both were reported as negative. 1 out of 5 had Myositis Associated Antibodies documented and was reported as negative. 4 out of 5 had documented Extractable Nuclear Antigen (ENA), see Image 2 below. 3 out of these 4 ENA were positive. Of the positive ENA autoantibodies, they included the following: One with JIIM had PM75 and Ku positive whilst two with JDM had RP 155 and Mi2A, Mi2B, RP 11 and RP155 positive. ENA Autoantibodies in JIIM/JDM patients documented in notes included the following: Diagnostic Imaging and investigations: -Magnetic Resonance Imaging (MRI) of the thigh- 4 MRI Thighs were documented in the notes of 3 patients. There was a 100% ability to identify inflammation on short tau inversion recovery sequence on these images reported by specialist radiologists. However, it was done at a subsequent date after diagnosis. All 4 MRI thigh were able to identify features of myositis. One child had a repeat MRI as a tool to measure response to escalated therapy and this was also successful in showing improvement, in comparison with the previous MRI done 3 and a half years earlier. -Electromyography (EMG)- 60% of patients (3/5) had an EMG done. One was able to identify acute myositis. One suggested a proximal myopathy with a coexisting neuropathy and one suggested inflammatory myopathy but thought the pattern was non-specific and other etiologies should be considered. -Skin Biopsy- One child had a skin punch biopsy which showed interface dermatitis -Muscle Biopsy 60% (3/5) had a muscle biopsy done. 2 were reported as non-specific features and one as connective tissue elements only. The histopathology report did state that they are not equipped to do all the required staining and that this is a subspecialist field. Comparison of Diagnostic and Classification Criteria Based on the data available at the time of presentation in the folders, a secondary analysis was done to assess the Peter and Bohan diagnostic criteria 1975 and the EULAR/ACR Classification Criteria 2017. 80% of patients had concordance in diagnosis and classification as seen in Table 3 below. Table 3 Comparison of Peter and Bohan 1975 diagnostic criteria versus EULAR/ ACR 2017 Classification criteria based on data extracted from notes at time of diagnosis. Peter and Bohan criteria (1975) EULAR/ACR (2017) Probability Percent Classification Subtype Patient 1 Probable Juvenile Dermatomyositis 62–91% Probable IIM Juvenile Dermatomyositis Patient 2 Probable polymyositis 57% Probable IIM Juvenile myositis Patient 3 Probable Juvenile Dermatomyositis 99–100% Definite IIM Juvenile Dermatomyositis Patient 4 Definite Juvenile Dermatomyositis 92–92% Definite IIM Juvenile Dermatomyositis Patient 5 Definite Juvenile Dermatomyositis 100% Definite IIM Juvenile Dermatomyositis Immunosuppressive drugs used: 100% of patients received steroids and methotrexate. Intravenous Immunoglobulin and methylprednisolone were the two most common parenteral drugs used. At the time of collection of data, 2 patients were on more than one immunosuppressant therapy other than prednisolone. One child was on 5 immunosuppressive therapies simultaneously for severe calcinosis, whilst on prednisolone. No child received cyclophosphamide. Additional treatment 4 out of 5 had vitamin d and calcium supplementation, 3 out of 5 had sunscreen or protective clothing. 100% had physiotherapy prescribed. Disease flares number and type: 3 out of 5 had documented flares. One child had 4 flares- 2 of which were skin and 2 were muscle weakness. The other 2 children had one muscle weakness flare each documented. Disease activity In assessing inactive disease, we looked at the last documented CMAS and CK levels (Table 4 ) as MMT 8 and physician global assessment were not documented. Patient 3 had a missing CK level, however the CMAS was the lowest meaning that there was still active disease. 3 patients had CMAS greater than 47 and 3 had CK less than 150 at the last visit. Of note, only 40% (2 out of 5) had both CMAS < 48 and CK less than 150 at the last clinic visit. Table 4 Patient 1 Patient 2 Patient 3 Patient 4 Patient 5 Last CMAS 46 52 29 51 51 Last CK 105 81 62 184 Last ESR 31 22 104 14 Physiological changes: 60% (3 out of 5) patients had physiological changes at last visit. These included alopecia, muscle atrophy, abdominal pain, contractures, calcinosis and growth failure. The child with calcinosis had 4 physiological changes. The calcinosis treatment included diltiazem, intralesional steroids and colchicine. No patient had ECHO, Spirometry or CT chest. 3 had ECGs which were documented as normal. One had an ophthalmology review but no cataracts were seen. Discussion: JIIM/JDM is rare but is a well-known entity. It should be noted from the outset, that the number of notes reviewed in this study was only 5 and as such, it would be difficult to make any general statements on prevalence or incidence in our population. The authors did reach out to colleagues at another Regional Health Authority, however they reported that research was halted during the COVID-19 pandemic at that time. Nevertheless, there were certain observations that was worth reporting. We did see a female predominance in our study (male: female ratio of 1:4) which is in keeping with most of the international literature on this disease. (9,10). Additionally, the peak age of onset is approximately 7 year (9,10) and we found an average of 6.8 years with a median age of 6 years in our study. The 5 patients were not clustered together. Additionally, there was no specific pattern of month presentation. Whilst it is recognized that there is an increasing prevalence as one moves closer to the equator, as well as increased flares in exposure to increase UV radiation (2,3), our tropical climate did not reveal any specific month of presentation or onset of disease. Meteorological data would be required to compare UV radiation in each month to make an objective assessment with a larger sample size. In terms of environmental triggers, 40% had a previous upper respiratory tract infection in 6 months prior to presentation which is a recognized trigger for disease flares in children with JDM (2). Interestingly, there was one patient who had a past medical history of Kawasaki disease which is now a recognized risk factor for autoimmunity. In a 2020 registry-based cohort study by Danish researchers, they found that patients with previous Kawasaki disease had an increased risk of developing autoimmune diseases including dermatomyositis and polymyositis after 10 years of the disease. (11) Of note, this patient developed JIIM, 6 years after presenting with Kawasaki disease. This should make us rethink how Kawasaki disease is viewed- beyond just its cardiac sequelae. A recommendation would be to refer these children to the rheumatology service as is done in Singapore. (12) The most common presenting complaint were skin rash and weakness which accounted for 62% of all presenting complaints. An interesting point to note is that joint pain and pruritis were the most common additional features present. However, the range of varying symptoms highlights the fact that JIIM/JDM can present with many other constitutional features. (9) In terms of pathognomonic features, Gottron’s papule and heliotropic rash were the most present feature in our JDM patients, which is usually the findings that help distinguish JDM from polymyositis. (13) Of note, with respect to muscle enzymes, Creatinine Kinase was 5 to 6 times higher in the one child who had probable myositis by Peter and Bohan’s Criteria. This is in keeping with the fact that CK in IIM patients tend to be the higher in Juvenile Polymyositis compared to JDM. (14) This study found a 60% ANA Positivity rate which is similar to the 70% reported by Shah M. et al. (14) Myositis Specific antibody panels and Myositis Associated Antibodies panels are not available in the public setting at SWRHA and may account for the low testing seen in the notes reviewed. However, a few notable observations on autoantibodies done from Extractable Nuclear Antigen-23 testing was discovered. One child with probable IIM, tested positive for Anti PM and Anti Ku which can be found in myositis and systemic sclerosis overlap. (14) Another patient with probable polymyositis, was positive for Anti RP155 only, which is not one of the usual autoantibodies as identified by Rider and Nistala in their overview (1). One patient with definite JDM tested positive for 4 Autoantibodies which included: Anti Mi2 A, Anti Mi2 B, Anti RP11 and Anti RP155. Anti Mi2 is well documented as a myositis specific antibody, however, Anti RP11 and RP155 can be associated with systemic sclerosis (15). As such, it raises the question whether these 2 children with Anti RP155 positive Antibodies, may have JDM overlapping with another connective tissue disease, sometimes termed “overlap myositis” (1,16). It may also be possible that this is a new autoantibody association of JDM in a Trinidadian population. However, the study size was too small to definitively pronounce that Anti RP155 is a new myositis associated antibody in a Trinidadian population. It is noteworthy that MRI was utilized in 60% of patients, as it offers the advantage of being non-invasive and is now being utilized more frequently by paediatric rheumatologists and dermatologists in diagnostic workup as well as in monitoring response to therapy in JIIM. (17,18) Specifically, in our study, muscle biopsy and EMG was documented in 60% of patients, which could be due to the invasiveness of these procedure. Moreover, muscle biopsy did not add much information to the diagnosis in this cohort. Additionally, our institution does not have the specialized histopathology services available to effectively report on JIIM/JDM specimens. Whilst it would be beneficial to have further trained personnel to offer this service, MRI may offer a better solution in our setting to assist in diagnosis. As such, a recommendation would be to utilize Magnetic Resonance Imaging earlier, in children being worked up for JIIM. The diagnosis of JDM has long been based on the works of Bohan and Peters in 1975. (4) Although it still remains widely in use by residents and clinicians across the board, there have been many attempts to revamp this criterion, with the latest being the EULAR/ACR Classification of 2017. (5) The advantage of this new criteria is that it can be scored with or without a muscle biopsy recognising the shift away from invasive testing. Additionally, there is an online calculator which facilitates an easier scoring. (6) Leclair and Lundberg (19) compared sensitivities and specificities of six varying IIM criteria and found that, when compared to Peter and Bohan’s criteria, EULAR/ACR 2017 had a slightly lower sensitivity (87–93% compared with 94–98%) but much better specificity (82–88% compared with 29–55%). A secondary analysis to compare these 2 criteria and found an 80% concordance between them. The reason for the discordance was that one child had neither an Electromyogram nor a biopsy documented in the notes. Therefore, the Peter and Bohan 1975 criteria should be continued to be utilized in clinical practice. However, in children without an EMG or a Muscle Biopsy, the EULAR/ACR 2017 offers a better classification criterion. In terms of drugs used, all children received Methotrexate and prednisolone which is in keeping with the latest consensus guidelines as the therapeutic agent of choice to commence. (20) The only parental drugs used were Intravenous Immunoglobulin and methylprednisolone. In attempting to stratify prednisolone dosage and duration, there were gaps in the notes and this presented some difficulty to report. As such, a recommendation would be to place notes on an electronic database for easier access and analysis. All children were prescribed physiotherapy, and 80% were on vitamin D supplementation which is in keeping with current recommendations. (20) The treatment options in this retrospective analysis at this centre were in keeping with guidelines. With respect to clinically inactive disease at the last visit, a modified PRINTO criteria similar to that used by O’kongo et al was used. (7) 40% had clinically inactive disease by our criteria of analysis. 60% had physiological changes at last visit and one patient who had the earliest disease onset in this data set also had calcinosis. His calcinosis appeared to be unresponsive to multiple therapy. Of note, there is no established guideline on JDM calcinosis management in the literature, however a survey of paediatric rheumatologists in 2017, found that optimising immunosuppressants and adding adjunct therapies like bisphosphonates and calcium channel blockers were the top two additional therapies recommended. (21) A recommendation would be for this institution to look at establishing a protocol for adjuvant therapy- like bisphosphonates, in children with JDM as another option for intractable calcinosis. Conclusion: The recently formed and established Paediatric Rheumatology Multi-Disciplinary Clinic at the South West Regional Health Authority has brought an advantageous opportunity for research into this field. The analysis of the JIIM/ JDM patients over this time frame has given us some useful insights that are in keeping with international epidemiological data in terms of gender and age of presentation. We note the important association of Kawasaki disease and future development of autoimmunity and suggest that future patients with Kawasaki disease be offered rheumatology follow up. Moreover, because MRI offered a 100% ability to find myositis, we recommend that this less invasive modality be utilised in our setting. Furthermore, we recommend that Peter and Bohan 1975 criterion still be used for all patients and the EULAR/ACR 2017 criterion can be used for those without muscle biopsy in light of our 80% concordance between the two criteria. We recognise also the advantage for EULAR/ACR 2017 criteria to be scored with or without muscle biopsy with its web-based calculator. In terms of antibodies, 60 percent of our patients were ANA positive. Interestingly, we did report 40 percent had Anti RP155 positive which may indicate overlap myositis or the potential for a new myositis associated antibody in our Trinidadian JIIM population. A larger sample size would lend strength to this statement. First line therapy in these patients were found to be in keeping with the latest consensus guidelines on JDM management. We would also recommend establishing a new protocol for bisphosphonate treatment for difficult to treat calcinosis in JDM as none had it used in our setting thus far. Overall, we recommend that this paediatric rheumatology service continue to develop and improve so as to offer highly specialised care to these rare patients that require extra attention. List Of Abbreviations: SWRHA- South West Regional Health Authority EULAR- European Union League Against Rheumatism ACR- American College of Rheumatology PRINTO- Paediatric Rheumatology INternational Trials Organisation CMAS- Childhood Myositis Assessment Score JIIM- Juvenile Idiopathic Inflammatory Myopathy JDM- Juvenile Dermatomyositis MRI- Magnetic Resonance Imaging EMG- Electromyography ANA- Anti Nuclear Antibody ENA- Extractable Nuclear Antigen CK- Creatinine Kinase LDH- Lactate Dehydrogenase AST- Aspartate Transaminase ALT- Alanine Transaminase Declarations: Ethics approval and consent to participate- Ethics approval attained from the Bioethics Committee of the South West Regional Health Authority. Reference number 1/3/40-103. Consent for publication- Not applicable Availability of data and materials- Attached Documents Competing interests- Not applicable Funding- Nil provided Authors' contributions- ZL- concept, design, data collection, introduction, methodology, analysis, results, discussion, conclusion SA- Data collection PC- Data Collection HD- Senior review of paper. All authors have approved the final version for submission Acknowledgements- Not applicable References: Rider LG, Nistala K. The juvenile idiopathic inflammatory myopathies: pathogenesis, clinical and autoantibody phenotypes, and outcomes. Journal of Internal Medicine . 2016;280(1):24-38. Mamyrova G, Rider LG, Ehrlich A, et al. Environmental factors associated with disease flare in juvenile and adult dermatomyositis. Rheumatology (Oxford) . 2017;56(8):1342-1347 Hengstman GJ, van Venrooij WJ, Vencovsky J, Moutsopoulos HM, van Engelen BG. The relative prevalence of dermatomyositis and polymyositis in Europe exhibits a latitudinal gradient. 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Curr Rheumatol Rep . 2018;20(4):18. Bellutti Enders F, Bader-Meunier B, Baildam E, et al. Consensus-based recommendations for the management of juvenile dermatomyositis. Ann Rheum Dis . 2017;76(2):329-340. Orandi AB, Baszis KW, Dharnidharka VR, Huber AM, Hoeltzel MF; CARRA Juvenile Myositis subgroup. Assessment, classification and treatment of calcinosis as a complication of juvenile dermatomyositis: a survey of pediatric rheumatologists by the childhood arthritis and rheumatology research alliance (CARRA). Pediatr Rheumatol Online J . 2017;15(1):71. Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-1764777","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Research Article","associatedPublications":[],"authors":[{"id":115094072,"identity":"81071f6f-cd88-440d-821f-02f29a1f35a1","order_by":0,"name":"Zafir Wasim Latchan","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAABBklEQVRIiWNgGAWjYFACxgcHGBgsgAweIK44ABY78ACvFmYDoDIJoAaQljMHwNSBBAJaGOBaGNsgWhjwaZFvP8x4uIJBQt6e/ezBz4Xz7sjZix1+CLTFTk63AYdPepIZDp5hkDDs4clLlp657Zkxj3SaAVBLsrHZARzOYsg/cLCBQYKxR4LHQJp32+HEHukEkJYDidtwaGHjf8wA0mIP1GL8m3cOSEv6B7xaeCSSwVoSgVrMpHkbQFpy8NsiIQGxJbnnTI6ZNc+xw8Y8t3MKDiQY4PaLfH8y88cGBhvb9vYzxrd5ag7Lsc9O3/zhQ4WdHC4tYMD4D0PIAI/yUTAKRsEoGAUEAQD9eVogSnSGOAAAAABJRU5ErkJggg==","orcid":"https://orcid.org/0000-0002-9859-8770","institution":"San Fernando General Hospital","correspondingAuthor":true,"submittingAuthor":false,"prefix":"","firstName":"Zafir","middleName":"Wasim","lastName":"Latchan","suffix":""},{"id":115094073,"identity":"6de68c08-f659-462a-98d9-1c5e1f3e8914","order_by":1,"name":"Shanice Ali","email":"","orcid":"","institution":"San Fernando General Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Shanice","middleName":"","lastName":"Ali","suffix":""},{"id":115094074,"identity":"9ded9bd9-2969-4ae5-935a-0933ed6ae65d","order_by":2,"name":"Patrick Chin-Kong","email":"","orcid":"","institution":"San Fernando General Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Patrick","middleName":"","lastName":"Chin-Kong","suffix":""},{"id":115094075,"identity":"53a9f683-4e03-4cfd-9aba-235b4f9c67f0","order_by":3,"name":"Haramnauth Dyaanand","email":"","orcid":"","institution":"San Fernando General Hospital","correspondingAuthor":false,"submittingAuthor":false,"prefix":"","firstName":"Haramnauth","middleName":"","lastName":"Dyaanand","suffix":""}],"badges":[],"createdAt":"2022-06-16 11:27:37","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-1764777/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-1764777/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":23183974,"identity":"111c8c67-1cc0-4411-bb88-87262993f53c","added_by":"auto","created_at":"2022-06-28 13:51:42","extension":"jpg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":32343,"visible":true,"origin":"","legend":"\u003cp\u003eGraph 1.\u003c/p\u003e","description":"","filename":"Graph1.jpg","url":"https://assets-eu.researchsquare.com/files/rs-1764777/v1/d5551786c881332065bb7591.jpg"},{"id":23184870,"identity":"65cf188a-a9a9-443e-bad7-662c7d7f9f47","added_by":"auto","created_at":"2022-06-28 13:56:42","extension":"jpg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":98807,"visible":true,"origin":"","legend":"\u003cp\u003eImage 1. (Map taken from Google maps)\u003c/p\u003e","description":"","filename":"Image1.jpg","url":"https://assets-eu.researchsquare.com/files/rs-1764777/v1/f49443d9bf1848c3e2a26d92.jpg"},{"id":23183975,"identity":"3045b3b3-3e46-4fd9-81c6-740bdb72907b","added_by":"auto","created_at":"2022-06-28 13:51:42","extension":"jpg","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":33481,"visible":true,"origin":"","legend":"\u003cp\u003eGraph 2.\u003c/p\u003e","description":"","filename":"Graph2.jpg","url":"https://assets-eu.researchsquare.com/files/rs-1764777/v1/f623cd03d884fb1a51697882.jpg"},{"id":23185866,"identity":"abdf6b72-3ab4-4ca4-be31-3d4699b0ac12","added_by":"auto","created_at":"2022-06-28 14:01:42","extension":"jpg","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":40161,"visible":true,"origin":"","legend":"\u003cp\u003eGraph 3.\u003c/p\u003e","description":"","filename":"Graph3.jpg","url":"https://assets-eu.researchsquare.com/files/rs-1764777/v1/d615734a2f36193e874fb878.jpg"},{"id":23184871,"identity":"cace6970-6b8a-4d69-97b7-7aa7daa77a74","added_by":"auto","created_at":"2022-06-28 13:56:42","extension":"jpg","order_by":5,"title":"Figure 5","display":"","copyAsset":false,"role":"figure","size":44658,"visible":true,"origin":"","legend":"\u003cp\u003eGraph 4\u003c/p\u003e","description":"","filename":"Graph4.jpg","url":"https://assets-eu.researchsquare.com/files/rs-1764777/v1/2c64ef8f5da70d68f5207846.jpg"},{"id":23183978,"identity":"bc7aa28f-7a88-4e1f-bf2b-861f4b546137","added_by":"auto","created_at":"2022-06-28 13:51:42","extension":"jpg","order_by":6,"title":"Figure 6","display":"","copyAsset":false,"role":"figure","size":39087,"visible":true,"origin":"","legend":"\u003cp\u003eGraph 5.\u003c/p\u003e","description":"","filename":"Graph5.jpg","url":"https://assets-eu.researchsquare.com/files/rs-1764777/v1/2f97b9d7ddc9002c57de8d62.jpg"},{"id":23183982,"identity":"7ef22ddb-b0fe-4b96-b6ed-2c99dcc0b306","added_by":"auto","created_at":"2022-06-28 13:51:42","extension":"jpg","order_by":7,"title":"Figure 7","display":"","copyAsset":false,"role":"figure","size":94877,"visible":true,"origin":"","legend":"\u003cp\u003eGraph 6.\u003c/p\u003e","description":"","filename":"Graph6.jpg","url":"https://assets-eu.researchsquare.com/files/rs-1764777/v1/d4dade22273e9b2e4e71c0be.jpg"},{"id":23183977,"identity":"c939dd51-0332-4f1b-b1df-4806c5ad11c9","added_by":"auto","created_at":"2022-06-28 13:51:42","extension":"jpg","order_by":8,"title":"Figure 8","display":"","copyAsset":false,"role":"figure","size":25263,"visible":true,"origin":"","legend":"\u003cp\u003eImage 2.\u003c/p\u003e","description":"","filename":"Image2.jpg","url":"https://assets-eu.researchsquare.com/files/rs-1764777/v1/7dba998c880dd7ec5abe00bf.jpg"},{"id":23183981,"identity":"59694b1e-7691-4406-b76d-95d9f334b17d","added_by":"auto","created_at":"2022-06-28 13:51:42","extension":"jpg","order_by":9,"title":"Figure 9","display":"","copyAsset":false,"role":"figure","size":45935,"visible":true,"origin":"","legend":"\u003cp\u003eGraph 7.\u003c/p\u003e","description":"","filename":"Graph7.jpg","url":"https://assets-eu.researchsquare.com/files/rs-1764777/v1/e8b3ae88f4ec8101ba3abc15.jpg"},{"id":23690122,"identity":"323b46ae-8a50-476a-bae5-51bf889e41b0","added_by":"auto","created_at":"2022-07-11 03:40:39","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":724498,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-1764777/v1/9eb66189-84e6-4db1-a75e-f7143a9f1c53.pdf"}],"financialInterests":"","formattedTitle":"Juvenile Idiopathic Inflammatory Myopathy and Juvenile Dermatomyositis: Characteristics and outcomes of affected children at a Paediatric Rheumatology Clinic in a tropical climate.","fulltext":[{"header":"Introduction:","content":"\u003cp\u003eJuvenile Idiopathic Inflammatory Myopathy (JIIM) is considered a group of rare autoimmune diseases characterised by muscle weakness and characteristic skin rashes. (1) Of the various subtypes of JIIM, Juvenile Dermatomyositis (JDM) is the most common and accounts for approximately 80% of all JIIM. (1) The incidence reported in the literature is estimated to be 2\u0026ndash;3 per million. (1)\u003c/p\u003e \u003cp\u003eOver the last two decades, there has been a magnitude of research into causation, associations and pathophysiology of JDM. (1,2,3) The current thinking is environment plays a key role in a genetically susceptible host. (2) There is evidence of an inverse relationship of latitude and prevalence of JDM in European cities. (3) However, there is no data on prevalence in a Caribbean population to corroborate this finding in our latitude. In fact, there is currently no published evidence on Juvenile Idiopathic Inflammatory Myopathy and Juvenile Dermatomyositis in Trinidad and Tobago, or in the English-speaking Caribbean.\u003c/p\u003e \u003cp\u003eIn our small twin island of Trinidad and Tobago, there are five Regional Health Authorities serving a population of 1.4\u0026nbsp;million people. They are: North West, North Central, Eastern, South West and the Tobago Regional Health Authorities. The South West Regional Health Authority (SWRHA) serves an estimated catchment of 600 000 people and there is one tertiary centre for paediatrics. Of note, there are no double qualified Paediatric Rheumatologists at present. As such, all paediatric rheumatology patients are seen under General Paediatrics with consultation and input from the Adult Rheumatology service.\u003c/p\u003e \u003cp\u003eIn February of 2021, a decision was made to establish a Multi-Disciplinary Paediatric Rheumatology Clinic at SWRHA, with an aim to optimise care for these children. Here, children up to 18 years would be seen together by both Paediatric Medical and Adult Rheumatology with an aim to enhance the care for children with rheumatological conditions. Thus, the few paediatric rheumatology patients at the SWRHA have been brought into one clinic. Preliminary analysis of these patients revealed that together with lupus, JIIM had the highest number of cases in the clinic (unpublished data). This stimulated the idea of looking more closely at the JIIM/JDM population at this institution.\u003c/p\u003e"},{"header":"Methodology:","content":"\u003cp\u003eAfter gaining ethical approval from the Bioethics Committee of the South West Regional Health Authority; a retrospective folder review on the clinical characteristics of JIIM/JDM patients at the San Fernando Teaching Hospital over a 6-month period commenced.\u003c/p\u003e\n\u003cp\u003eInclusion criteria were:\u003c/p\u003e\n\u003col style=\"list-style-type: lower-alpha;\"\u003e\n\u003cli\u003e\n\u003cp\u003eChildren less than eighteen years, AND\u003c/p\u003e\n\u003c/li\u003e\n\u003cli\u003e\n\u003cp\u003eAttending the Paediatric Rheumatology Multi-Disciplinary Clinic of South West Regional Health Authority, AND\u003c/p\u003e\n\u003c/li\u003e\n\u003cli\u003e\n\u003cp\u003eA diagnosis of possible, probable or definite idiopathic inflammatory myopathy OR\u003c/p\u003e\n\u003c/li\u003e\n\u003cli\u003e\n\u003cp\u003eA diagnosis of possible, probable or definite juvenile dermatomyositis\u003c/p\u003e\n\u003c/li\u003e\n\u003c/ol\u003e\n\u003cp\u003ePatients\u0026rsquo; notes alone were analysed who attended this clinic during the period from May to October 2021. The information was extracted and placed onto a Microsoft Excel Spreadsheet from which data analysis was done and descriptive statistics were reported.\u003c/p\u003e\n\u003cp\u003eDemographic data, month of presentation and time lag between symptom onset and presentation were analysed. Additionally, examination findings, laboratory and diagnostic investigations were extracted from the notes. These were used in a secondary analysis to compare the Peter and Bohan criteria of 1975 with the European Union League Against Rheumatism/ American College of Rheumatology (EULAR/ACR) classification criteria of 2017. (4,5) The classification criteria were calculated with the assistance of an online calculator found at \u003cspan class=\"ExternalRef\"\u003e\u003cspan class=\"RefSource\"\u003e\u003ca href=\"http://www.imm.ki.se/biostatistics/calculators/iim/\" target=\"_blank\"\u003ewww.imm.ki.se/biostatistics/calculators/iim/\u003c/a\u003e\u003c/span\u003e\u003c/span\u003e. (6)\u003c/p\u003e\n\u003cp\u003eThe number of relapses and also immunosuppressive drugs used in management of these children with JIIM and JDM at SWRHA were examined. In addition, utilization of physiotherapy, skin protection and vitamin d supplementation were described together with calcinosis and the treatment options utilized.\u003c/p\u003e\n\u003cp\u003eWe also assessed if patients had clinically inactive disease, at the last visit, based on a modified Paediatric Rheumatology INternational Trials Organisation (PRINTO) Criteria similar to Okong'o \u003cem\u003eet al\u003c/em\u003e's study from Cape Town. (7,8) Our criterion included a Childhood Myositis Assessment Score (CMAS)\u0026thinsp;\u0026gt;\u0026thinsp;47 and a serum Creatinine Kinase\u0026thinsp;\u0026gt;\u0026thinsp;150 U/L.\u003c/p\u003e"},{"header":"Results:","content":"\u003cp\u003e5 notes were reviewed during this time. It must be highlighted that several gaps in data were present in the notes reviewed.\u003c/p\u003e\n\u003cdiv id=\"Sec4\" class=\"Section2\"\u003e\n\u003ch2\u003eDemographics:\u003c/h2\u003e\n\u003cp\u003eOf these five children with JIIM/JDM, 4 were female and one was male.\u003c/p\u003e\n\u003cp\u003eNo clusters were seen in terms of location of these children. See map below.\u003c/p\u003e\n\u003cp\u003eThe age at presentation ranged from 2 years to 11 years. Average age at presentation was 6.8years with a median of 6 years.\u003c/p\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec5\" class=\"Section2\"\u003e\n\u003ch2\u003eMonth of presentation:\u003c/h2\u003e\n\u003cp\u003eWhilst 2 patients had their first symptom in November, there was no observable pattern of month of presentation. The average time lag between first symptom and presentation was 3.8 months.\u003c/p\u003e\n\u003cdiv class=\"gridtable\"\u003e\n\u003ctable id=\"Tab1\" border=\"1\"\u003e\u003ccaption\u003e\n\u003cdiv class=\"CaptionNumber\"\u003eTable 1\u003c/div\u003e\n\u003c/caption\u003e\n\u003cthead\u003e\n\u003ctr\u003e\n\u003cth align=\"left\"\u003e\n\u003cp\u003eMonth of first symptom\u003c/p\u003e\n\u003c/th\u003e\n\u003cth align=\"left\"\u003e\n\u003cp\u003eMonth of presentation\u003c/p\u003e\n\u003c/th\u003e\n\u003cth align=\"left\"\u003e\n\u003cp\u003eTime lag to presentation\u003c/p\u003e\n\u003c/th\u003e\n\u003c/tr\u003e\n\u003c/thead\u003e\n\u003ctbody\u003e\n\u003ctr\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eNovember\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eMay\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003e6 months\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eAugust\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eSeptember\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003e1 month\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eNovember\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eDecember\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003e1 month\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eMay\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eJuly\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003e2 months\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eApril\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eJanuary\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003e9 months\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003e\u0026nbsp;\u003c/p\u003e\n\u003c/div\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec6\" class=\"Section2\"\u003e\n\u003ch2\u003ePresenting complaint:\u003c/h2\u003e\n\u003cp\u003eThe most common presenting complaint were skin rash and weakness which accounted for 62% of all presenting complaints of these children who had JIIM/JDM followed by joint pains, fever and weight loss.\u003c/p\u003e\n\u003cp\u003eThe most common additional symptom was joint pain (3/5) and pruritis (3/5). Followed by fever (2/5) and fatigue (2/5). One patient with 5 additional symptom was male and presented at 2 years of age.\u003c/p\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec7\" class=\"Section2\"\u003e\n\u003ch2\u003eExamination findings at diagnosis:\u003c/h2\u003e\n\u003cp\u003e4 out of 5 children had some documentation of muscle weakness on examination.\u003c/p\u003e\n\u003cp\u003eIn terms of skin findings, 4 out of 5 had documented pathognomonic changes of JDM. Gottron\u0026rsquo;s papule was the most common pathognomonic (50%) skin finding in these 4 children with JDM.\u003c/p\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec8\" class=\"Section2\"\u003e\n\u003ch2\u003eEnvironmental factors and genetics:\u003c/h2\u003e\n\u003cp\u003eIn terms of environmental factors, 2 out of 5 had previous infections in the 6 months prior to presentation. 1 out of 5 had the potential stressor of entering final year of primary school. 3 out of 5 had been fully vaccinated; however, we were unable to ascertain if any were given in the 6 months prior to presentation.\u003c/p\u003e\n\u003cp\u003eAdditionally, 4 out of 5 had previous medical conditions which included Kawasaki disease. 1 patient had a positive family history of autoimmune disease.\u003c/p\u003e\n\u003cdiv class=\"gridtable\"\u003e\n\u003ctable id=\"Tab2\" border=\"1\"\u003e\u003ccaption\u003e\n\u003cdiv class=\"CaptionNumber\"\u003eTable 2\u003c/div\u003e\n\u003c/caption\u003e\n\u003cthead\u003e\n\u003ctr\u003e\n\u003cth align=\"left\"\u003e\n\u003cp\u003ePast Medical History\u003c/p\u003e\n\u003c/th\u003e\n\u003cth align=\"left\"\u003e\n\u003cp\u003eFamily history\u003c/p\u003e\n\u003c/th\u003e\n\u003c/tr\u003e\n\u003c/thead\u003e\n\u003ctbody\u003e\n\u003ctr\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eKawasaki disease 6 years prior\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eNephrotic Syndrome\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eLichen striatus one year prior\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eRheumatoid Arthritis\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eLower respiratory tract infection at birth and constipation\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eSickle cell Trait\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eAsthma\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n\u003c/tr\u003e\n\u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003e\u0026nbsp;\u003c/p\u003e\n\u003c/div\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec9\" class=\"Section2\"\u003e\n\u003ch2\u003eLaboratory investigations at diagnosis- muscle enzymes:\u003c/h2\u003e\n\u003cp\u003eIn terms of muscle enzymes at time of diagnosis, Creatinine Kinase (CK) and Lactate Dehydrogenase (LDH) were the most elevated muscle enzyme at presentation. Alanine transferase (ALT) was the least elevated of all the muscle enzymes in any of these children. Two patients had missing LDH in the notes.\u003c/p\u003e\n\u003c/div\u003e\n\u003ch2\u003eAutoantibodies:\u003c/h2\u003e\n\u003cp\u003eWith respect to Autoantibodies, 3 out of 5 had Anti-Nuclear Antibody (ANA) positivity documented. 2 out of 5 had documented Myositis Specific Antibodies sent off- both were reported as negative. 1 out of 5 had Myositis Associated Antibodies documented and was reported as negative.\u003c/p\u003e\n\u003cp\u003e4 out of 5 had documented Extractable Nuclear Antigen (ENA), see Image 2 below. 3 out of these 4 ENA were positive. Of the positive ENA autoantibodies, they included the following: One with JIIM had PM75 and Ku positive whilst two with JDM had RP 155 and Mi2A, Mi2B, RP 11 and RP155 positive.\u003c/p\u003e\n\u003cp\u003eENA Autoantibodies in JIIM/JDM patients documented in notes included the following:\u003c/p\u003e\n\u003ch2\u003eDiagnostic Imaging and investigations:\u003c/h2\u003e\n\u003cdiv id=\"Sec11\" class=\"Section2\"\u003e\n\u003ch2\u003e-Magnetic Resonance Imaging (MRI) of the thigh-\u003c/h2\u003e\n\u003cp\u003e4 MRI Thighs were documented in the notes of 3 patients. There was a 100% ability to identify inflammation on short tau inversion recovery sequence on these images reported by specialist radiologists. However, it was done at a subsequent date after diagnosis. All 4 MRI thigh were able to identify features of myositis. One child had a repeat MRI as a tool to measure response to escalated therapy and this was also successful in showing improvement, in comparison with the previous MRI done 3 and a half years earlier.\u003c/p\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec12\" class=\"Section2\"\u003e\n\u003ch2\u003e-Electromyography (EMG)-\u003c/h2\u003e\n\u003cp\u003e60% of patients (3/5) had an EMG done. One was able to identify acute myositis. One suggested a proximal myopathy with a coexisting neuropathy and one suggested inflammatory myopathy but thought the pattern was non-specific and other etiologies should be considered.\u003c/p\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec13\" class=\"Section2\"\u003e\n\u003ch2\u003e-Skin Biopsy-\u003c/h2\u003e\n\u003cp\u003eOne child had a skin punch biopsy which showed interface dermatitis\u003c/p\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec14\" class=\"Section2\"\u003e\n\u003ch2\u003e-Muscle Biopsy\u003c/h2\u003e\n\u003cp\u003e60% (3/5) had a muscle biopsy done. 2 were reported as non-specific features and one as connective tissue elements only. The histopathology report did state that they are not equipped to do all the required staining and that this is a subspecialist field.\u003c/p\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec15\" class=\"Section2\"\u003e\n\u003ch2\u003eComparison of Diagnostic and Classification Criteria\u003c/h2\u003e\n\u003cp\u003eBased on the data available at the time of presentation in the folders, a secondary analysis was done to assess the Peter and Bohan diagnostic criteria 1975 and the EULAR/ACR Classification Criteria 2017. 80% of patients had concordance in diagnosis and classification as seen in Table\u0026nbsp;3 below.\u003c/p\u003e\n\u003cdiv class=\"gridtable\"\u003e\n\u003ctable id=\"Taba\" border=\"1\"\u003e\u003ccaption\u003e\n\u003cp\u003eTable 3\u003c/p\u003e\n\u003cp\u003eComparison of Peter and Bohan 1975 diagnostic criteria versus EULAR/ ACR 2017 Classification criteria based on data extracted from notes at time of diagnosis.\u003c/p\u003e\n\u003c/caption\u003e\n\u003cthead\u003e\n\u003ctr\u003e\n\u003cth rowspan=\"2\" align=\"left\"\u003e\u0026nbsp;\u003c/th\u003e\n\u003cth rowspan=\"2\" align=\"left\"\u003e\n\u003cp\u003ePeter and Bohan criteria (1975)\u003c/p\u003e\n\u003c/th\u003e\n\u003cth colspan=\"3\" align=\"left\"\u003e\n\u003cp\u003eEULAR/ACR (2017)\u003c/p\u003e\n\u003c/th\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003cth align=\"left\"\u003e\n\u003cp\u003eProbability Percent\u003c/p\u003e\n\u003c/th\u003e\n\u003cth align=\"left\"\u003e\n\u003cp\u003eClassification\u003c/p\u003e\n\u003c/th\u003e\n\u003cth align=\"left\"\u003e\n\u003cp\u003eSubtype\u003c/p\u003e\n\u003c/th\u003e\n\u003c/tr\u003e\n\u003c/thead\u003e\n\u003ctbody\u003e\n\u003ctr\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003ePatient 1\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eProbable Juvenile Dermatomyositis\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003e62\u0026ndash;91%\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eProbable IIM\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eJuvenile Dermatomyositis\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003ePatient 2\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eProbable polymyositis\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003e57%\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eProbable IIM\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eJuvenile myositis\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003ePatient 3\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eProbable Juvenile Dermatomyositis\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003e99\u0026ndash;100%\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eDefinite IIM\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eJuvenile Dermatomyositis\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003ePatient 4\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eDefinite Juvenile Dermatomyositis\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003e92\u0026ndash;92%\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eDefinite IIM\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eJuvenile Dermatomyositis\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003ePatient 5\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eDefinite Juvenile Dermatomyositis\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003e100%\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eDefinite IIM\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eJuvenile Dermatomyositis\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003e\u0026nbsp;\u003c/p\u003e\n\u003c/div\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec16\" class=\"Section2\"\u003e\n\u003ch2\u003eImmunosuppressive drugs used:\u003c/h2\u003e\n\u003cp\u003e100% of patients received steroids and methotrexate. Intravenous Immunoglobulin and methylprednisolone were the two most common parenteral drugs used.\u003c/p\u003e\n\u003cp\u003eAt the time of collection of data, 2 patients were on more than one immunosuppressant therapy other than prednisolone. One child was on 5 immunosuppressive therapies simultaneously for severe calcinosis, whilst on prednisolone. No child received cyclophosphamide.\u003c/p\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec17\" class=\"Section2\"\u003e\n\u003ch2\u003eAdditional treatment\u003c/h2\u003e\n\u003cp\u003e4 out of 5 had vitamin d and calcium supplementation, 3 out of 5 had sunscreen or protective clothing. 100% had physiotherapy prescribed.\u003c/p\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec18\" class=\"Section2\"\u003e\n\u003ch2\u003eDisease flares number and type:\u003c/h2\u003e\n\u003cp\u003e3 out of 5 had documented flares. One child had 4 flares- 2 of which were skin and 2 were muscle weakness. The other 2 children had one muscle weakness flare each documented.\u003c/p\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec19\" class=\"Section2\"\u003e\n\u003ch2\u003eDisease activity\u003c/h2\u003e\n\u003cp\u003eIn assessing inactive disease, we looked at the last documented CMAS and CK levels (Table\u0026nbsp;\u003cspan class=\"InternalRef\"\u003e4\u003c/span\u003e) as MMT 8 and physician global assessment were not documented. Patient 3 had a missing CK level, however the CMAS was the lowest meaning that there was still active disease.\u003c/p\u003e\n\u003cp\u003e3 patients had CMAS greater than 47 and 3 had CK less than 150 at the last visit. Of note, only 40% (2 out of 5) had both CMAS\u0026thinsp;\u0026lt;\u0026thinsp;48 and CK less than 150 at the last clinic visit.\u003c/p\u003e\n\u003cdiv class=\"gridtable\"\u003e\n\u003ctable id=\"Tab3\" border=\"1\"\u003e\u003ccaption\u003e\n\u003cdiv class=\"CaptionNumber\"\u003eTable 4\u003c/div\u003e\n\u003c/caption\u003e\n\u003cthead\u003e\n\u003ctr\u003e\n\u003cth align=\"left\"\u003e\u0026nbsp;\u003c/th\u003e\n\u003cth align=\"left\"\u003e\n\u003cp\u003ePatient 1\u003c/p\u003e\n\u003c/th\u003e\n\u003cth align=\"left\"\u003e\n\u003cp\u003ePatient 2\u003c/p\u003e\n\u003c/th\u003e\n\u003cth align=\"left\"\u003e\n\u003cp\u003ePatient 3\u003c/p\u003e\n\u003c/th\u003e\n\u003cth align=\"left\"\u003e\n\u003cp\u003ePatient 4\u003c/p\u003e\n\u003c/th\u003e\n\u003cth align=\"left\"\u003e\n\u003cp\u003ePatient 5\u003c/p\u003e\n\u003c/th\u003e\n\u003c/tr\u003e\n\u003c/thead\u003e\n\u003ctbody\u003e\n\u003ctr\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eLast CMAS\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"char\"\u003e\n\u003cp\u003e46\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"char\"\u003e\n\u003cp\u003e52\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"char\"\u003e\n\u003cp\u003e29\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"char\"\u003e\n\u003cp\u003e51\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"char\"\u003e\n\u003cp\u003e51\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eLast CK\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"char\"\u003e\n\u003cp\u003e105\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"char\"\u003e\n\u003cp\u003e81\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n\u003ctd align=\"char\"\u003e\n\u003cp\u003e62\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"char\"\u003e\n\u003cp\u003e184\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003ctr\u003e\n\u003ctd align=\"left\"\u003e\n\u003cp\u003eLast ESR\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"char\"\u003e\n\u003cp\u003e31\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"char\"\u003e\n\u003cp\u003e22\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"char\"\u003e\n\u003cp\u003e104\u003c/p\u003e\n\u003c/td\u003e\n\u003ctd align=\"left\"\u003e\u0026nbsp;\u003c/td\u003e\n\u003ctd align=\"char\"\u003e\n\u003cp\u003e14\u003c/p\u003e\n\u003c/td\u003e\n\u003c/tr\u003e\n\u003c/tbody\u003e\n\u003c/table\u003e\n\u003cp\u003e\u0026nbsp;\u003c/p\u003e\n\u003c/div\u003e\n\u003c/div\u003e\n\u003cdiv id=\"Sec20\" class=\"Section2\"\u003e\n\u003ch2\u003ePhysiological changes:\u003c/h2\u003e\n\u003cp\u003e60% (3 out of 5) patients had physiological changes at last visit. These included alopecia, muscle atrophy, abdominal pain, contractures, calcinosis and growth failure. The child with calcinosis had 4 physiological changes. The calcinosis treatment included diltiazem, intralesional steroids and colchicine.\u003c/p\u003e\n\u003cp\u003eNo patient had ECHO, Spirometry or CT chest. 3 had ECGs which were documented as normal. One had an ophthalmology review but no cataracts were seen.\u003c/p\u003e\n\u003c/div\u003e"},{"header":"Discussion:","content":"\u003cp\u003eJIIM/JDM is rare but is a well-known entity. It should be noted from the outset, that the number of notes reviewed in this study was only 5 and as such, it would be difficult to make any general statements on prevalence or incidence in our population. The authors did reach out to colleagues at another Regional Health Authority, however they reported that research was halted during the COVID-19 pandemic at that time. Nevertheless, there were certain observations that was worth reporting.\u003c/p\u003e \u003cp\u003eWe did see a female predominance in our study (male: female ratio of 1:4) which is in keeping with most of the international literature on this disease. (9,10). Additionally, the peak age of onset is approximately 7 year (9,10) and we found an average of 6.8 years with a median age of 6 years in our study. The 5 patients were not clustered together. Additionally, there was no specific pattern of month presentation. Whilst it is recognized that there is an increasing prevalence as one moves closer to the equator, as well as increased flares in exposure to increase UV radiation (2,3), our tropical climate did not reveal any specific month of presentation or onset of disease. Meteorological data would be required to compare UV radiation in each month to make an objective assessment with a larger sample size.\u003c/p\u003e \u003cp\u003eIn terms of environmental triggers, 40% had a previous upper respiratory tract infection in 6 months prior to presentation which is a recognized trigger for disease flares in children with JDM (2). Interestingly, there was one patient who had a past medical history of Kawasaki disease which is now a recognized risk factor for autoimmunity. In a 2020 registry-based cohort study by Danish researchers, they found that patients with previous Kawasaki disease had an increased risk of developing autoimmune diseases including dermatomyositis and polymyositis after 10 years of the disease. (11) Of note, this patient developed JIIM, 6 years after presenting with Kawasaki disease. This should make us rethink how Kawasaki disease is viewed- beyond just its cardiac sequelae. A recommendation would be to refer these children to the rheumatology service as is done in Singapore. (12)\u003c/p\u003e \u003cp\u003eThe most common presenting complaint were skin rash and weakness which accounted for 62% of all presenting complaints. An interesting point to note is that joint pain and pruritis were the most common additional features present. However, the range of varying symptoms highlights the fact that JIIM/JDM can present with many other constitutional features. (9) In terms of pathognomonic features, Gottron\u0026rsquo;s papule and heliotropic rash were the most present feature in our JDM patients, which is usually the findings that help distinguish JDM from polymyositis. (13)\u003c/p\u003e \u003cp\u003eOf note, with respect to muscle enzymes, Creatinine Kinase was 5 to 6 times higher in the one child who had probable myositis by Peter and Bohan\u0026rsquo;s Criteria. This is in keeping with the fact that CK in IIM patients tend to be the higher in Juvenile Polymyositis compared to JDM. (14) This study found a 60% ANA Positivity rate which is similar to the 70% reported by Shah M. \u003cem\u003eet al.\u003c/em\u003e (14)\u003c/p\u003e \u003cp\u003eMyositis Specific antibody panels and Myositis Associated Antibodies panels are not available in the public setting at SWRHA and may account for the low testing seen in the notes reviewed. However, a few notable observations on autoantibodies done from Extractable Nuclear Antigen-23 testing was discovered.\u003c/p\u003e \u003cp\u003eOne child with probable IIM, tested positive for Anti PM and Anti Ku which can be found in myositis and systemic sclerosis overlap. (14) Another patient with probable polymyositis, was positive for Anti RP155 only, which is not one of the usual autoantibodies as identified by Rider and Nistala in their overview (1). One patient with definite JDM tested positive for 4 Autoantibodies which included: Anti Mi2 A, Anti Mi2 B, Anti RP11 and Anti RP155. Anti Mi2 is well documented as a myositis specific antibody, however, Anti RP11 and RP155 can be associated with systemic sclerosis (15). As such, it raises the question whether these 2 children with Anti RP155 positive Antibodies, may have JDM overlapping with another connective tissue disease, sometimes termed \u0026ldquo;overlap myositis\u0026rdquo; (1,16). It may also be possible that this is a new autoantibody association of JDM in a Trinidadian population. However, the study size was too small to definitively pronounce that Anti RP155 is a new myositis associated antibody in a Trinidadian population.\u003c/p\u003e \u003cp\u003eIt is noteworthy that MRI was utilized in 60% of patients, as it offers the advantage of being non-invasive and is now being utilized more frequently by paediatric rheumatologists and dermatologists in diagnostic workup as well as in monitoring response to therapy in JIIM. (17,18) Specifically, in our study, muscle biopsy and EMG was documented in 60% of patients, which could be due to the invasiveness of these procedure. Moreover, muscle biopsy did not add much information to the diagnosis in this cohort. Additionally, our institution does not have the specialized histopathology services available to effectively report on JIIM/JDM specimens. Whilst it would be beneficial to have further trained personnel to offer this service, MRI may offer a better solution in our setting to assist in diagnosis. As such, a recommendation would be to utilize Magnetic Resonance Imaging earlier, in children being worked up for JIIM.\u003c/p\u003e \u003cp\u003eThe diagnosis of JDM has long been based on the works of Bohan and Peters in 1975. (4) Although it still remains widely in use by residents and clinicians across the board, there have been many attempts to revamp this criterion, with the latest being the EULAR/ACR Classification of 2017. (5) The advantage of this new criteria is that it can be scored with or without a muscle biopsy recognising the shift away from invasive testing. Additionally, there is an online calculator which facilitates an easier scoring. (6) Leclair and Lundberg (19) compared sensitivities and specificities of six varying IIM criteria and found that, when compared to Peter and Bohan\u0026rsquo;s criteria, EULAR/ACR 2017 had a slightly lower sensitivity (87\u0026ndash;93% compared with 94\u0026ndash;98%) but much better specificity (82\u0026ndash;88% compared with 29\u0026ndash;55%).\u003c/p\u003e \u003cp\u003eA secondary analysis to compare these 2 criteria and found an 80% concordance between them. The reason for the discordance was that one child had neither an Electromyogram nor a biopsy documented in the notes. Therefore, the Peter and Bohan 1975 criteria should be continued to be utilized in clinical practice. However, in children without an EMG or a Muscle Biopsy, the EULAR/ACR 2017 offers a better classification criterion.\u003c/p\u003e \u003cp\u003e In terms of drugs used, all children received Methotrexate and prednisolone which is in keeping with the latest consensus guidelines as the therapeutic agent of choice to commence. (20) The only parental drugs used were Intravenous Immunoglobulin and methylprednisolone. In attempting to stratify prednisolone dosage and duration, there were gaps in the notes and this presented some difficulty to report. As such, a recommendation would be to place notes on an electronic database for easier access and analysis.\u003c/p\u003e \u003cp\u003eAll children were prescribed physiotherapy, and 80% were on vitamin D supplementation which is in keeping with current recommendations. (20) The treatment options in this retrospective analysis at this centre were in keeping with guidelines.\u003c/p\u003e \u003cp\u003eWith respect to clinically inactive disease at the last visit, a modified PRINTO criteria similar to that used by O\u0026rsquo;kongo \u003cem\u003eet al\u003c/em\u003e was used. (7) 40% had clinically inactive disease by our criteria of analysis. 60% had physiological changes at last visit and one patient who had the earliest disease onset in this data set also had calcinosis. His calcinosis appeared to be unresponsive to multiple therapy. Of note, there is no established guideline on JDM calcinosis management in the literature, however a survey of paediatric rheumatologists in 2017, found that optimising immunosuppressants and adding adjunct therapies like bisphosphonates and calcium channel blockers were the top two additional therapies recommended. (21) A recommendation would be for this institution to look at establishing a protocol for adjuvant therapy- like bisphosphonates, in children with JDM as another option for intractable calcinosis.\u003c/p\u003e"},{"header":"Conclusion:","content":"\u003cp\u003eThe recently formed and established Paediatric Rheumatology Multi-Disciplinary Clinic at the South West Regional Health Authority has brought an advantageous opportunity for research into this field. The analysis of the JIIM/ JDM patients over this time frame has given us some useful insights that are in keeping with international epidemiological data in terms of gender and age of presentation. We note the important association of Kawasaki disease and future development of autoimmunity and suggest that future patients with Kawasaki disease be offered rheumatology follow up. Moreover, because MRI offered a 100% ability to find myositis, we recommend that this less invasive modality be utilised in our setting. Furthermore, we recommend that Peter and Bohan 1975 criterion still be used for all patients and the EULAR/ACR 2017 criterion can be used for those without muscle biopsy in light of our 80% concordance between the two criteria. We recognise also the advantage for EULAR/ACR 2017 criteria to be scored with or without muscle biopsy with its web-based calculator. In terms of antibodies, 60 percent of our patients were ANA positive. Interestingly, we did report 40 percent had Anti RP155 positive which may indicate overlap myositis or the potential for a new myositis associated antibody in our Trinidadian JIIM population. A larger sample size would lend strength to this statement. First line therapy in these patients were found to be in keeping with the latest consensus guidelines on JDM management. We would also recommend establishing a new protocol for bisphosphonate treatment for difficult to treat calcinosis in JDM as none had it used in our setting thus far. Overall, we recommend that this paediatric rheumatology service continue to develop and improve so as to offer highly specialised care to these rare patients that require extra attention.\u003c/p\u003e"},{"header":"List Of Abbreviations:","content":"\u003cp\u003eSWRHA- South West Regional Health Authority\u003c/p\u003e\n\u003cp\u003eEULAR- European Union League Against Rheumatism\u003c/p\u003e\n\u003cp\u003eACR- American College of Rheumatology\u003c/p\u003e\n\u003cp\u003ePRINTO- Paediatric Rheumatology INternational Trials Organisation\u003c/p\u003e\n\u003cp\u003eCMAS- Childhood Myositis Assessment Score\u003c/p\u003e\n\u003cp\u003eJIIM- Juvenile Idiopathic Inflammatory Myopathy\u003c/p\u003e\n\u003cp\u003eJDM- Juvenile Dermatomyositis\u003c/p\u003e\n\u003cp\u003eMRI- Magnetic Resonance Imaging\u003c/p\u003e\n\u003cp\u003eEMG- Electromyography\u003c/p\u003e\n\u003cp\u003eANA- Anti Nuclear Antibody\u003c/p\u003e\n\u003cp\u003eENA- Extractable Nuclear Antigen\u003c/p\u003e\n\u003cp\u003eCK- Creatinine Kinase\u003c/p\u003e\n\u003cp\u003eLDH- Lactate Dehydrogenase\u003c/p\u003e\n\u003cp\u003eAST- Aspartate Transaminase\u003c/p\u003e\n\u003cp\u003eALT- Alanine Transaminase\u003c/p\u003e"},{"header":"Declarations:","content":"\u003cul\u003e\n\u003cli\u003e\n\u003cp\u003eEthics approval and consent to participate- Ethics approval attained from the Bioethics Committee of the South West Regional Health Authority. Reference number 1/3/40-103.\u003c/p\u003e\n\u003c/li\u003e\n\u003cli\u003e\n\u003cp\u003eConsent for publication- Not applicable\u003c/p\u003e\n\u003c/li\u003e\n\u003cli\u003e\n\u003cp\u003eAvailability of data and materials- Attached Documents\u003c/p\u003e\n\u003c/li\u003e\n\u003cli\u003e\n\u003cp\u003eCompeting interests- Not applicable\u003c/p\u003e\n\u003c/li\u003e\n\u003cli\u003e\n\u003cp\u003eFunding- Nil provided\u003c/p\u003e\n\u003c/li\u003e\n\u003cli\u003e\n\u003cp\u003eAuthors' contributions-\u003c/p\u003e\n\u003c/li\u003e\n\u003cul\u003e\n\u003cli\u003e\n\u003cp\u003eZL- concept, design, data collection, introduction, methodology, analysis, results, discussion, conclusion\u003c/p\u003e\n\u003c/li\u003e\n\u003cli\u003e\n\u003cp\u003eSA- Data collection\u003c/p\u003e\n\u003c/li\u003e\n\u003cli\u003e\n\u003cp\u003ePC- Data Collection\u003c/p\u003e\n\u003c/li\u003e\n\u003cli\u003e\n\u003cp\u003eHD- Senior review of paper.\u003c/p\u003e\n\u003c/li\u003e\n\u003c/ul\u003e\n\u003c/ul\u003e\n\u003cp\u003eAll authors have approved the final version for submission\u003c/p\u003e\n\u003cul\u003e\n\u003cli\u003e\n\u003cp\u003eAcknowledgements- Not applicable\u003c/p\u003e\n\u003c/li\u003e\n\u003c/ul\u003e"},{"header":"References:","content":"\u003col\u003e\n\u003cli\u003eRider LG, Nistala K. The juvenile idiopathic inflammatory myopathies: pathogenesis, clinical and autoantibody phenotypes, and outcomes. \u003cem\u003eJournal of Internal Medicine\u003c/em\u003e. 2016;280(1):24-38.\u003c/li\u003e\n\u003cli\u003eMamyrova G, Rider LG, Ehrlich A, et al. Environmental factors associated with disease flare in juvenile and adult dermatomyositis. \u003cem\u003eRheumatology (Oxford)\u003c/em\u003e. 2017;56(8):1342-1347\u003c/li\u003e\n\u003cli\u003eHengstman GJ, van Venrooij WJ, Vencovsky J, Moutsopoulos HM, van Engelen BG. The relative prevalence of dermatomyositis and polymyositis in Europe exhibits a latitudinal gradient. \u003cem\u003eAnn Rheum Dis\u003c/em\u003e. 2000;59(2):141-142.\u003c/li\u003e\n\u003cli\u003eBohan A, Peter JB. Polymyositis and dermatomyositis. \u003cem\u003eN Engl J Med\u003c/em\u003e. 1975;292(8):403-407.\u003c/li\u003e\n\u003cli\u003eLundberg IE, Tj\u0026auml;rnlund A, Bottai M, et al. 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. \u003cem\u003eAnn Rheum Dis\u003c/em\u003e. 2017;76(12):1955-1964.\u003c/li\u003e\n\u003cli\u003eOnline Web calculator. Classification criteria for Idiopathic Inflammatory Myopathies. www.imm.ki.se/biostatistics/calculators/iim/.\u003c/li\u003e\n\u003cli\u003eOkong\u0026apos;o LO, Esser M, Wilmshurst J, Scott C. Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study. \u003cem\u003ePediatr Rheumatol Online J\u003c/em\u003e. 2016;14(1):60. \u003c/li\u003e\n\u003cli\u003eLazarevic D, Pistorio A, Palmisani E, et al. The PRINTO criteria for clinically inactive disease in juvenile dermatomyositis. \u003cem\u003eAnn Rheum Dis\u003c/em\u003e. 2013;72(5):686-693.\u003c/li\u003e\n\u003cli\u003ePilkington CA, Feldman BM, Sontichai W. Juvenile dermatomyositis and other inflammatory muscle diseases. In: Petty RE\u003cem\u003e, \u003c/em\u003eLaxer RM, Lindsley CB, Wedderburn LR, Mellins ED, Fuhlbrigge RC.\u003cem\u003e \u003c/em\u003eTextbook of Pediatric Rheumatology 8th edition. Philadelphia, Elsevier; 2021.\u003c/li\u003e\n\u003cli\u003eHarris W. Examination Paediatrics 4th Edition. A guide to Paediatric Training. Ch 16 Rheumatology. Juvenile Idiopathic Inflammatory Myopathies (JIIMs): Juvenile Dermatomyositis (JDM). Australia, Churchill Livingstone; 2011, 577- 586.\u003c/li\u003e\n\u003cli\u003eNielsen TM, Andersen NH, Torp-Pedersen C, S\u0026oslash;gaard P, Kragholm KH. Kawasaki disease, autoimmune disorders, and cancer: a register-based study. \u003cem\u003eEur J Pediatr\u003c/em\u003e. 2021;180(3):717-723.\u003c/li\u003e\n\u003cli\u003eTan JHT, Fun HS, Arkachaisri T. Paediatrics Rheumatology Clinic Population in Singapore: The KKH Experience. \u003cem\u003eProceedings of Singapore Healthcare\u003c/em\u003e. 2012;21(4):265-271.\u003c/li\u003e\n\u003cli\u003eShah M, Mamyrova G, Targoff IN, et al. The clinical phenotypes of the juvenile idiopathic inflammatory myopathies. \u003cem\u003eMedicine (Baltimore)\u003c/em\u003e. 2013; 92(1):25-41.\u003c/li\u003e\n\u003cli\u003eMahler M, Satoh M, Fritzler MJ. Anti-Ku antibodies: important points to consider. \u003cem\u003eAnn Rheum Dis\u003c/em\u003e. 2021; 80(11):e182.\u003c/li\u003e\n\u003cli\u003eLiaskos C, Marou E, Simopoulou T, et al. Disease-related autoantibody profile in patients with systemic sclerosis. \u003cem\u003eAutoimmunity\u003c/em\u003e. 2017; 50(7):414-421.\u003c/li\u003e\n\u003cli\u003eLi D, Tansley SL. Juvenile Dermatomyositis-Clinical Phenotypes. \u003cem\u003eCurr Rheumatol Rep\u003c/em\u003e. 2019; 21(12):74. \u003c/li\u003e\n\u003cli\u003eCorral-Maga\u0026ntilde;a O, Bauz\u0026aacute;-Alonso AF, Escudero-G\u0026oacute;ngora MM, Lacruz L, Mart\u0026iacute;n-Santiago A. Juvenile Dermatomyositis: Key Roles of Muscle Magnetic Resonance Imaging and Early Aggressive Treatment. La resonancia magn\u0026eacute;tica muscular y el tratamiento agresivo precoz, claves en la dermatomiositis juvenil. \u003cem\u003eActas Dermosifiliogr (Engl Ed)\u003c/em\u003e. 2018;109(6):e42-e46.\u003c/li\u003e\n\u003cli\u003eThyoka M, Adekunle O, Pilkington C, et al. Introduction of a novel magnetic resonance imaging-based scoring system for assessing disease activity in children with juvenile dermatomyositis. \u003cem\u003eRheumatology (Oxford)\u003c/em\u003e. 2018;57(9):1661-1668.\u003c/li\u003e\n\u003cli\u003eLeclair V, Lundberg IE. New Myositis Classification Criteria-What We Have Learned Since Bohan and Peter. \u003cem\u003eCurr Rheumatol Rep\u003c/em\u003e. 2018;20(4):18.\u003c/li\u003e\n\u003cli\u003eBellutti Enders F, Bader-Meunier B, Baildam E, et al. Consensus-based recommendations for the management of juvenile dermatomyositis. \u003cem\u003eAnn Rheum Dis\u003c/em\u003e. 2017;76(2):329-340.\u003c/li\u003e\n\u003cli\u003eOrandi AB, Baszis KW, Dharnidharka VR, Huber AM, Hoeltzel MF; CARRA Juvenile Myositis subgroup. Assessment, classification and treatment of calcinosis as a complication of juvenile dermatomyositis: a survey of pediatric rheumatologists by the childhood arthritis and rheumatology research alliance (CARRA). \u003cem\u003ePediatr Rheumatol Online J\u003c/em\u003e. 2017;15(1):71.\u003c/li\u003e\n\u003c/ol\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":"Juvenile Idiopathic Inflammatory Myopathy, Juvenile Dermatomyositis, Trinidad","lastPublishedDoi":"10.21203/rs.3.rs-1764777/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-1764777/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eBackground\u003c/h2\u003e \u003cp\u003eJuvenile Idiopathic Inflammatory Myopathy (JIIM) is a rare autoimmune condition of which Juvenile Dermatomyositis (JDM) is the most common subtype. To this date there has been no published data focusing on this condition in Trinidad nor the English-speaking Caribbean.\u003c/p\u003e\u003ch2\u003eMethods\u003c/h2\u003e \u003cp\u003eA retrospective folder analysis was done on patients with JIIM who attended the recently formed Paediatric Rheumatology Multi-Disciplinary Clinic over a 6 months period from May 2021 at San Fernando Teaching Hospital, Trinidad and Tobago. Demographics, investigations and diagnoses were analysed. A secondary comparative analysis of two diagnostic criteria was performed and treatment approach was examined.\u003c/p\u003e\u003ch2\u003eResults\u003c/h2\u003e \u003cp\u003e5 notes were reviewed in this period. The male: female ratio was 1:4 and the average age of onset was 6.8 years. 80% of JIIM cases were JDM. The most common presenting complaints were: skin rash and weakness (62%). The most common additional symptoms were: joint pain (60%) and pruritis (60%). Previous upper respiratory tract infection was recognized in 40% of JIIM patients and one patient had Kawasaki disease 6 years prior. Creatine Kinase showed modest elevations in JDM, whilst it was extremely elevated in one patient with myositis. There was an 80% concordance between the EULAR/ACR 2017 and the Peter and Bohan 1975 criteria. 60% tested positive for ANA. Anti PM, Anti Ku, Anti RP155, Anti Mi2A, Anti Mi2B and Anti RP11 antibodies were identified. 40% were positive for AntiRP155. 60% of patients utilized MRI imaging which detected myositis in all reports. Methotrexate and prednisolone were used in 100%. Parenteral drugs used included: intravenous immunoglobulin and methylprednisolone. 80% had vitamin D supplementation and 100% were prescribed physiotherapy. One child had intractable calcinosis.\u003c/p\u003e\u003ch2\u003eConclusions\u003c/h2\u003e \u003cp\u003eWhilst the study size was arguably small, patterns were seen which are in keeping with international data on epidemiology and treatment. Recommendations from this study include: greater utilization of MRI in diagnostic workup, further evaluating anti RP155 antibody as a new myositis associated antibody in a Caribbean population and exploring further options for calcinosis treatment in a developing nation.\u003c/p\u003e","manuscriptTitle":"Juvenile Idiopathic Inflammatory Myopathy and Juvenile Dermatomyositis: Characteristics and outcomes of affected children at a Paediatric Rheumatology Clinic in a tropical climate.","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2022-06-28 13:51:40","doi":"10.21203/rs.3.rs-1764777/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":"5012a74f-4329-419c-ba77-05a45cd447a2","owner":[],"postedDate":"June 28th, 2022","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2022-07-11T03:40:30+00:00","versionOfRecord":[],"versionCreatedAt":"2022-06-28 13:51:40","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-1764777","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-1764777","identity":"rs-1764777","version":["v1"]},"buildId":"cBFmMYwuxLRRLfASyISRj","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}