Rare, Spesific, Systemic Organ Involvement in a Patient with a History of Oligoarthritis and Sjögren’s Syndrome: Case Report

Rare, Spesific, Systemic Organ Involvement in a Patient with a History of Oligoarthritis and Sjögren’s Syndrome: Case Report | 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 Case Report Rare, Spesific, Systemic Organ Involvement in a Patient with a History of Oligoarthritis and Sjögren’s Syndrome: Case Report Murat AY This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-6874147/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 Objective: Sjögren's syndrome is a chronic autoimmune disease characterized by involvement of exocrine glands and lymphocytic infiltrates. The presented case demonstrates the rare findings of systemic involvement in Sjögren’s syndrome. Case: A 45-year-old woman, diagnosed with Sjögren's syndrome in the rheumatology clinic, presented with mildly raised erythematous skin lesions, joint pain and intermittent cough. Physical examination arthralgia, polycyclic cutaneous lesions and bibasilar rales. Ultrasonography revealed parotitis, electromyography revealed peripheral nervous system involvement and high resolution tomography revealed lymphocytic interstitial pneumonia. Azathioprine and Rituximab 1000 mg on days 0 and 14 were started every 6 months. There was significant response in joint involvement, acute phase reactans and near complete response in signs of interstitial lung disease findings. Discussion: Considering that it is a systemic disease, clinical findings including nonspecific findings should be carefully evaluated and targeted treatment algorithms in systemic involvement should be organized specifically according to the organ involvement. Sjögren's Syndrome lymphocytic interstitial pneumonia arthritis lymphoma Figures Figure 1 Figure 2 Figure 3 Introduction Primary Sjögren's syndrome is a common, systemic autoimmune disease with a female: male incidence ratio of approximately 9:1, reaching its peak incidence at approximately 50 years of age (1). The disease is characterized by exocrinopathy and is usually characterized by the classic triad of dry mouth and eyes, fatigue and arthralgia (2) . These three symptoms in particular are present in more than 80% of patients with Sjögren's syndrome. It is diagnosed with the 2017 American College of Rheumatology (ACR)/European Alliance of Rheumatology Societies (EULAR) criteria for primary Sjögren's syndrome, which requires the presence of immunologic abnormalities (positive serum anti-SSA antibodies or focal lymphocytic sialadenitis on salivary gland biopsy) (3). Systemic involvement can be seen in 30-40% of primary Sjögren's syndrome(4). EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) is used to assess systemic complications of the disease (5). The incidence of B-cell lymphoma in primary Sjögren's syndrome is significantly increased compared to the general population. In general, low-grade, marginal zone histologic subtype B-cell non-Hodgkin's lymphomas are common. The incidence of lung disease and involvement in Sjögren's syndrome ranges from 9-50%. As non-sicca systemic manifestations in Sjögren's syndrome; arthralgia in 50% and intermittent symmetric arthritis in 16% in joint involvement, cutaneous vasculitis and polycyclic photosensitive cutaneous lesions in 30% in the presence of cryoglobulins, bronchiectasis/bronchiolar abnormalities in 50% in tomography findings of pulmonary involvement, ground-glass opacities/interstitial changes in 49%, pulmonary involvement was most commonly histopathologically diagnosed as non-specific interstitial pneumonia (NSIP) in 45%, bronchiolitis in 25%, and usual interstitial pneumonia (OIP) in 16%, LIP in 15%, Raynaud's phenomenon in 10-20% as cardiovascular findings, rarely mild pericardial effusion, primary biliary cholangitis, type-1 autoimmune hepatitis and mucosa-associated lymphoid tissue (MALT) lymphoma in gastrointestinal findings, interstitial renal diseases most commonly in nephrological findings, neurologically, ganglionopathy, peripheral nervous system involvement in 2-50%, mononoritis multiplex, clubfoot, transverse myelitis, optic neuritis and neuromyelitis optica, finally subclinical hypothyroidism can be seen in one third of Sjögren's syndrome patients (6). Case A 45-year-old woman, diagnosed with Sjögren's syndrome in the rheumatology clinic, presented to our rheumatology clinic with mild erythematous skin lesions, joint pain and intermittent cough. The patient has a history of right knee arthrocentesis after oligoarthritis; hydroxychloroquine, short-term prednisolone and methotrexate use. Physical examination revealed arthralgia, polycyclic cutaneous lesions, bibasilar rales in respiratory sounds; no lympadenopathy was detected by palpation. B-symptoms were evaluated as negative. Hyporeflexia was not detected but the patient described intermittent paresthesia in the anamnesis. In laboratory findings, antinuclear antibody (ANA): 3+ (>1/1000-<1/3200, in fine speckled pattern), anti-SSA: positive, anti-SSB: positive, Ro-52: positive, rheumatoid factor: 24.8 IU/mL positive, sedimentation: 96 mm/hour and C-reactive protein (CRP): 11.2 mg/L. Hypocomplementemia was not detected and anti-double stranded DNA (anti-dsDNA) was negative. Investigations revealed subclinical hypothyroidism. Hepatitis and Human Immunodeficiency Virus (HIV) serology were negative. Quantiferon test was found negative. Schirmer's test 2/3 mm, salivary gland biopsy pathology: lymphocytic infiltration in multiple foci, focal lymphocytic sialadenitis compatible with Sjögren's syndrome. Although no lymphadenopathy was detected on ultrasonography, neck ultrasonography was evaluated in favor of parotitis. Electromyography was consistent with demyelinating type polyneuropathy. High-resolution tomography (HRCT) findings were evaluated in favor of lymphocytic interstitial pneumonia (LIP). The first HRCT findings evaluated at the diagnostic stage are shown in Figure 1. No additional findings were found on magnetic resonance imaging in terms of central nervous system involvement and possible demyelinating diseases. Azathioprine 100 mg/day with titration with a dose target of 2 mg/kg/day, prednisolone 0.3 mg/kg/day and pilocarpine were started. Endobronchial ultrasonography (EBUS) was performed and lymphoma was not considered with histopathologic confirmation. In the following period, the patient developed arthritis in bilateral symmetrical proximal interphalangeal joints and wrists, no regression of interstitial lung disease (ILD) findings was observed on imaging and Rituximab treatment was planned. The findings of active arthritis in the wrist are shown in Figure 2. The first dose of Rituximab 1000 mg was administered intravenously and 1000 mg dose was planned on the 14th day. Two days after the Rituximab dose, the patient was admitted to the emergency department due to sudden onset of dyspnea. Echocardiography revealed severe tricuspid regurgitation, severe mitral stenosis and pulmonary artery pressure: 85 mm Hg. The patient was followed up as asymptomatic after volume control was achieved and 1000 mg iv Rituximab infusion was administered on the 14th day and then mitral valve replacement surgery was performed by Cardiovascular Surgery and Warfarin sodium was started as permanent anticoagulant therapy. Rituximab infusion doses of 1000 mg on days 0 and 14 every 6 months and Azathioprine 2 mg/kg/day were continued and the patient's joint involvement findings showed a significant regression, acute phase reactants showed a significant response and ILD findings showed a near complete response. The control HRCT findings after follow-up and treatment are shown in Figure 3. Discussion Systemic manifestations seen in Sjögren's syndrome are classified into two subgroups: non-visceral (skin, arthralgia, myalgia) and visceral (lung, heart, kidney, gastrointestinal, endocrine, central and peripheral nervous system) (7). In Sjögren's syndrome, arthralgia occurs equally in small and large joints, whereas arthritis is an intermittent polyarticular arthropathy with symmetrical involvement of small joints (8). Arthritis often occurs in small joints such as the joints of the hand and less commonly in large joints such as the knee, shoulder and ankle. Compared to RA, arthritis in Sjögren's syndrome goes through more remissions and relapses and morning stiffness is less common. Differential diagnosis between RA and Sjögren's syndrome is important in arthritis and arthralgia due to RF positivity. Atzeni et al. demonstrated that anti cyclic citrullinated peptide (anti-CCP) antibodies are important in the differential diagnosis of arthritis in early RA and Sjögren's syndrome (9). One important difference is that synovitis is rare in primary Sjögren's syndrome, in addition, arthritis is milder and tends to resolve without deformity. One important difference with RA is that primary Sjögren's syndrome does not show erosive changes in the joints on imaging. The most common neurologic finding in Sjögren's syndrome is pure sensory or sensory predominant polyneuropathy. Peripheral nervous system involvement is more common than central nervous system involvement in primary Sjögren's syndrome (10). The incidence of respiratory system involvement in Sjögren's syndrome varies (9-24%). Although dry mouth, dry nose and cough due to tracheal involvement may be observed in upper and lower airway findings, Sjögren's syndrome should be considered in differential diagnosis as it is a nonspecific finding. Diffuse parenchymal lung diseases such as NSIP, OIP and bronchiolitis obliterans organizing pneumonia (BOOP) can be seen; LIP, follicular bronchiolitis, nodular lymphoid hyperplasia can be seen as pulmonary lymphoproliferative diseases. Especially LIP pattern is rarely seen in patients with Sjögren's syndrome. It may present with a slow progression and increasing cough and shortness of breath. On physical examination, lymphadenopathy and bibasilar rales are common. On HRCT, ground glass appearance, centrilobular nodules, septal and bronchovascular thickening, pulmonary cysts and mediastinal lymphadenopathy are most prominent. In Sjögren's syndrome, very few cases with LIP pattern show complete recovery (11). In the diagnostic approach of Sjögren's syndrome, first of all, differential diagnoses should be evaluated and primary and secondary differentiation should be made. Clinical findings suggestive of systemic involvement should be carefully evaluated and spesific treatment protocols should be planned. Declarations Written and verbal informed consent was obtained from the patient prior to the preparation of this case report. The patient was fully informed about the case presentation and publication process, and consented voluntarily to the publication of the report. Compliance with Ethical Standards Conflict of Interest The author declare that they have no competing financial interests or personal relationships that may have influenced the work reported in this study. Funding: This research did not receive any specific grants from funding agencies in the public, commercial, or not-for-profit sectors. Data Availability Statement: Data is available upon reasonable request. Ethical approval All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Decloration of Helsinki and its later amendments or comparable ethical standards. Informed Consent Statement: Written informed consent has been obtained from the participant involved in the study. References Qin B, Wang J, Yang Z, Yang M, Ma N, Huang F, et al. Epidemiology of primary Sjögren's syndrome: a systematic review and meta-analysis. Ann Rheum Dis. 2015;74(11):1983-9. Meijer JM, Meiners PM, Huddleston Slater JJ, Spijkervet FK, Kallenberg CG, Vissink A, et al. Health-related quality of life, employment and disability in patients with Sjogren's syndrome. Rheumatology (Oxford). 2009;48(9):1077-82. Mariette X, Criswell LA. Primary Sjögren's Syndrome. New England Journal of Medicine. 2018;378(10):931-9. Fox RI. Sjögren's syndrome. The Lancet. 2005;366(9482):321-31. Seror R, Ravaud P, Mariette X, Bootsma H, Theander E, Hansen A, et al. EULAR Sjögren's Syndrome Patient Reported Index (ESSPRI): development of a consensus patient index for primary Sjögren's syndrome. Annals of the rheumatic diseases. 2011;70(6):968-72. Ramos-Casals M, Brito-Zerón P, Bombardieri S, Bootsma H, De Vita S, Dörner T, et al. EULAR recommendations for the management of Sjögren's syndrome with topical and systemic therapies. Ann Rheum Dis. 2020;79(1):3-18. Fox RI. Sjögren's syndrome. The Lancet. 2005;366(9482):321-31. Kassan SS, Moutsopoulos HM. Clinical manifestations and early diagnosis of Sjögren syndrome. Arch Intern Med. 2004;164(12):1275-84. Atzeni F, Sarzi-Puttini P, Lama N, Bonacci E, Bobbio-Pallavicini F, Montecucco C, et al. Anti-cyclic citrullinated peptide antibodies in primary Sjögren's syndrome may be associated with non-erosive synovitis. Arthritis Res Ther. 2008;10(3):R51. Mori K, Iijima M, Koike H, Hattori N, Tanaka F, Watanabe H, et al. The wide spectrum of clinical manifestations in Sjögren's syndrome-associated neuropathy. Brain. 2005;128(Pt 11):2518-34. Cha SI, Fessler MB, Cool CD, Schwarz MI, Brown KK. Lymphoid interstitial pneumonia: clinical features, associations and prognosis. Eur Respir J. 2006;28(2):364-9. Additional Declarations No competing interests reported. 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. We do this by developing innovative software and high quality services for the global research community. Our growing team is made up of researchers and industry professionals working together to solve the most critical problems facing scientific publishing. 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-6874147","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":472410691,"identity":"25bf8d78-8098-4178-b6fb-a0535ce81b4c","order_by":0,"name":"Murat AY","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA2UlEQVRIiWNgGAWjYBADHn72BiBlYEGk+gMMDHKSPQdAWiSI12JscCMBxCRCi+6M3IefP9QcTtxw8/nVDT8KJBj427sT8Goxu5FuLHHg2OHEmbdzym72AB0mcebsBgJa0hgkDrAdTuy7nZN2gweoxUAil6AW5h8H/h1ObLh5Ju3mHyK1sEkcbDtsLHCD/dht4mw584zN4mxfOjCQc9huyxhI8BD2y/E05hsV36yBUXn82c03f2zk+Nt78WuBgmYg5jEAsXiIUQ4CdUDM/oBY1aNgFIyCUTDCAABF7E64yGUACgAAAABJRU5ErkJggg==","orcid":"","institution":"Kütahya Sağlık Bilimleri Üniversitesi","correspondingAuthor":true,"prefix":"","firstName":"Murat","middleName":"","lastName":"AY","suffix":""}],"badges":[],"createdAt":"2025-06-11 17:53:08","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-6874147/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-6874147/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":85619492,"identity":"b2c27886-797a-4687-96fc-0e4b888e3406","added_by":"auto","created_at":"2025-06-29 15:01:49","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":635787,"visible":true,"origin":"","legend":"\u003cp\u003eSystemic involvement of lymphocytic interstitial pneumonia at diagnosis. Interlobular septal thickening in both lung parenchyma, bibasilar subpleural reticular density increases, multiple cystic appearances in both lungs, bronchiectatic changes, scattered solid parenchymal nodules.\u003c/p\u003e","description":"","filename":"1.png","url":"https://assets-eu.researchsquare.com/files/rs-6874147/v1/4042d389d22c44ab55dfa08c.png"},{"id":85620248,"identity":"b35ab1bc-70b4-43d7-8d0a-3612cfa48248","added_by":"auto","created_at":"2025-06-29 15:09:49","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":325294,"visible":true,"origin":"","legend":"\u003cp\u003eArthralgia in the proximal interphalangeal joints and active arthritis in bilateral wrists\u003c/p\u003e","description":"","filename":"2.png","url":"https://assets-eu.researchsquare.com/files/rs-6874147/v1/91e3360f51a1159101a6a423.png"},{"id":85619493,"identity":"a95fd4fe-a74f-4dcf-bb47-04252d71cb1a","added_by":"auto","created_at":"2025-06-29 15:01:49","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":479472,"visible":true,"origin":"","legend":"\u003cp\u003eControl tomography section 2 years after the first high-resolution tomography, which was evaluated as a near complete response to Azathioprine and Rituximab treatments. Although near complete regression is seen in diffuse ground glass areas, pneumocyst appearance in the lung parenchyma is remarkable.\u003c/p\u003e","description":"","filename":"3.png","url":"https://assets-eu.researchsquare.com/files/rs-6874147/v1/4adbe5ed9bd7eaae612bc8fb.png"},{"id":85620249,"identity":"a8b10a79-1657-4711-a079-e19295aed0ae","added_by":"auto","created_at":"2025-06-29 15:09:54","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":2303220,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-6874147/v1/0558b86a-0635-4da7-bb16-d76da815d44e.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Rare, Spesific, Systemic Organ Involvement in a Patient with a History of Oligoarthritis and Sjögren’s Syndrome: Case Report","fulltext":[{"header":"Introduction","content":"\u003cp\u003ePrimary Sjögren's syndrome is a common, systemic autoimmune disease with a female: male incidence ratio of approximately 9:1, reaching its peak incidence at approximately 50 years of age (1). The disease is characterized by exocrinopathy and is usually characterized by the classic triad of dry mouth and eyes, fatigue and arthralgia (2) . These three symptoms in particular are present in more than 80% of patients with Sjögren's syndrome. It is diagnosed with the 2017 American College of Rheumatology (ACR)/European Alliance of Rheumatology Societies (EULAR) criteria for primary Sjögren's syndrome, which requires the presence of immunologic abnormalities (positive serum anti-SSA antibodies or focal lymphocytic sialadenitis on salivary gland biopsy) (3).\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eSystemic involvement can be seen in 30-40% of primary Sjögren's syndrome(4). EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) is used to assess systemic complications of the disease (5).\u003c/p\u003e\n\u003cp\u003eThe incidence of B-cell lymphoma in primary Sjögren's syndrome is significantly increased compared to the general population. In general, low-grade, marginal zone histologic subtype B-cell non-Hodgkin's lymphomas are common.\u003c/p\u003e\n\u003cp\u003eThe incidence of lung disease and involvement in Sjögren's syndrome ranges from 9-50%. As non-sicca systemic manifestations in Sjögren's syndrome; arthralgia in 50% and intermittent symmetric arthritis in 16% in joint involvement, cutaneous vasculitis and polycyclic photosensitive cutaneous lesions in 30% in the presence of cryoglobulins, bronchiectasis/bronchiolar abnormalities in 50% in tomography findings of pulmonary involvement, ground-glass opacities/interstitial changes in 49%, pulmonary involvement was most commonly histopathologically diagnosed as non-specific interstitial pneumonia (NSIP) in 45%, bronchiolitis in 25%, and usual interstitial pneumonia (OIP) in 16%, LIP in 15%, Raynaud's phenomenon in 10-20% as cardiovascular findings, rarely mild pericardial effusion, primary biliary cholangitis, type-1 autoimmune hepatitis and mucosa-associated lymphoid tissue (MALT) lymphoma in gastrointestinal findings, interstitial renal diseases most commonly in nephrological findings, neurologically, ganglionopathy, peripheral nervous system involvement in 2-50%, mononoritis multiplex, clubfoot, transverse myelitis, optic neuritis and neuromyelitis optica, finally subclinical hypothyroidism can be seen in one third of Sjögren's syndrome patients (6).\u0026nbsp;\u003c/p\u003e"},{"header":"Case","content":"\u003cp\u003eA 45-year-old woman, diagnosed with Sj\u0026ouml;gren\u0026apos;s syndrome in the rheumatology clinic, presented to our rheumatology clinic with mild erythematous skin lesions, joint pain and intermittent cough. The patient has a history of right knee arthrocentesis after oligoarthritis; hydroxychloroquine, short-term prednisolone and methotrexate use.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003ePhysical examination revealed arthralgia, polycyclic cutaneous lesions, bibasilar rales in respiratory sounds; no lympadenopathy was detected by palpation. B-symptoms were evaluated as negative. Hyporeflexia was not detected but the patient described intermittent paresthesia in the anamnesis.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eIn laboratory findings, antinuclear antibody (ANA): 3+ (\u0026gt;1/1000-\u0026lt;1/3200, in fine speckled pattern), anti-SSA: positive, anti-SSB: positive, Ro-52: positive, rheumatoid factor: 24.8 IU/mL positive, sedimentation: 96 mm/hour and C-reactive protein (CRP): 11.2 mg/L. Hypocomplementemia was not detected and anti-double stranded DNA (anti-dsDNA) was negative. Investigations revealed subclinical hypothyroidism. Hepatitis and Human Immunodeficiency Virus (HIV) serology were negative. Quantiferon test was found negative.\u003c/p\u003e\n\u003cp\u003eSchirmer\u0026apos;s test 2/3 mm, salivary gland biopsy pathology: lymphocytic infiltration in multiple foci, focal lymphocytic sialadenitis compatible with Sj\u0026ouml;gren\u0026apos;s syndrome.\u003c/p\u003e\n\u003cp\u003eAlthough no lymphadenopathy was detected on ultrasonography, neck ultrasonography was evaluated in favor of parotitis. Electromyography was consistent with demyelinating type polyneuropathy. High-resolution tomography (HRCT) findings were evaluated in favor of lymphocytic interstitial pneumonia (LIP). The first HRCT findings evaluated at the diagnostic stage are shown in Figure 1. No additional findings were found on magnetic resonance imaging in terms of central nervous system involvement and possible demyelinating diseases.\u003c/p\u003e\n\u003cp\u003eAzathioprine 100 mg/day with titration with a dose target of 2 mg/kg/day, prednisolone 0.3 mg/kg/day and pilocarpine were started. Endobronchial ultrasonography (EBUS) was performed and lymphoma was not considered with histopathologic confirmation. In the following period, the patient developed arthritis in bilateral symmetrical proximal interphalangeal joints and wrists, no regression of interstitial lung disease (ILD) findings was observed on imaging and Rituximab treatment was planned. The findings of active arthritis in the wrist are shown in Figure 2.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThe first dose of Rituximab 1000 mg was administered intravenously and 1000 mg dose was planned on the 14th day. Two days after the Rituximab dose, the patient was admitted to the emergency department due to sudden onset of dyspnea. Echocardiography revealed severe tricuspid regurgitation, severe mitral stenosis and pulmonary artery pressure: 85 mm Hg. The patient was followed up as asymptomatic after volume control was achieved and 1000 mg iv Rituximab infusion was administered on the 14th day and then mitral valve replacement surgery was performed by Cardiovascular Surgery and Warfarin sodium was started as permanent anticoagulant therapy. Rituximab infusion doses of 1000 mg on days 0 and 14 every 6 months and Azathioprine 2 mg/kg/day were continued and the patient\u0026apos;s joint involvement findings showed a significant regression, acute phase reactants showed a significant response and ILD findings showed a near complete response. The control HRCT findings after follow-up and treatment are shown in Figure 3.\u0026nbsp;\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eSystemic manifestations seen in Sjögren's syndrome are classified into two subgroups: non-visceral (skin, arthralgia, myalgia) and visceral (lung, heart, kidney, gastrointestinal, endocrine, central and peripheral nervous system) (7).\u003c/p\u003e\n\u003cp\u003eIn Sjögren's syndrome, arthralgia occurs equally in small and large joints, whereas arthritis is an intermittent polyarticular arthropathy with symmetrical involvement of small joints (8). Arthritis often occurs in small joints such as the joints of the hand and less commonly in large joints such as the knee, shoulder and ankle. Compared to RA, arthritis in Sjögren's syndrome goes through more remissions and relapses and morning stiffness is less common. Differential diagnosis between RA and Sjögren's syndrome is important in arthritis and arthralgia due to RF positivity. Atzeni et al. demonstrated that anti cyclic citrullinated peptide (anti-CCP) antibodies are important in the differential diagnosis of arthritis in early RA and Sjögren's syndrome (9). One important difference is that synovitis is rare in primary Sjögren's syndrome, in addition, arthritis is milder and tends to resolve without deformity. One important difference with RA is that primary Sjögren's syndrome does not show erosive changes in the joints on imaging.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThe most common neurologic finding in Sjögren's syndrome is pure sensory or sensory predominant polyneuropathy. Peripheral nervous system involvement is more common than central nervous system involvement in primary Sjögren's syndrome (10).\u003c/p\u003e\n\u003cp\u003eThe incidence of respiratory system involvement in Sjögren's syndrome varies (9-24%). Although dry mouth, dry nose and cough due to tracheal involvement may be observed in upper and lower airway findings, Sjögren's syndrome should be considered in differential diagnosis as it is a nonspecific finding. Diffuse parenchymal lung diseases such as NSIP, OIP and bronchiolitis obliterans organizing pneumonia (BOOP) can be seen; LIP, follicular bronchiolitis, nodular lymphoid hyperplasia can be seen as pulmonary lymphoproliferative diseases. Especially LIP pattern is rarely seen in patients with Sjögren's syndrome. It may present with a slow progression and increasing cough and shortness of breath. On physical examination, lymphadenopathy and bibasilar rales are common. On HRCT, ground glass appearance, centrilobular nodules, septal and bronchovascular thickening, pulmonary cysts and mediastinal lymphadenopathy are most prominent. In Sjögren's syndrome, very few cases with LIP pattern show complete recovery (11).\u003c/p\u003e\n\u003cp\u003eIn the diagnostic approach of Sjögren's syndrome, first of all, differential diagnoses should be evaluated and primary and secondary differentiation should be made. Clinical findings suggestive of systemic involvement should be carefully evaluated and spesific treatment protocols should be planned.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003eWritten and verbal informed consent was obtained from the patient prior to the preparation of this case report. The patient was fully informed about the case presentation and publication process, and consented voluntarily to the publication of the report.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompliance with Ethical Standards\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConflict of Interest\u0026nbsp;\u003c/strong\u003eThe author declare that they have no competing financial interests or personal relationships that may have influenced the work reported in this study.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding:\u003c/strong\u003e This research did not receive any specific grants from funding agencies in the public, commercial, or not-for-profit sectors.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eData Availability Statement:\u003c/strong\u003e Data is available upon reasonable request.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthical approval\u0026nbsp;\u003c/strong\u003eAll procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Decloration of Helsinki and its later amendments or comparable ethical standards.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eInformed Consent Statement:\u003c/strong\u003e Written informed consent has been obtained from the participant involved in the study.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n \u003cli\u003eQin B, Wang J, Yang Z, Yang M, Ma N, Huang F, et al. Epidemiology of primary Sj\u0026ouml;gren\u0026apos;s syndrome: a systematic review and meta-analysis. Ann Rheum Dis. 2015;74(11):1983-9.\u003c/li\u003e\n \u003cli\u003eMeijer JM, Meiners PM, Huddleston Slater JJ, Spijkervet FK, Kallenberg CG, Vissink A, et al. Health-related quality of life, employment and disability in patients with Sjogren\u0026apos;s syndrome. Rheumatology (Oxford). 2009;48(9):1077-82.\u003c/li\u003e\n \u003cli\u003eMariette X, Criswell LA. Primary Sj\u0026ouml;gren\u0026apos;s Syndrome. New England Journal of Medicine. 2018;378(10):931-9.\u003c/li\u003e\n \u003cli\u003eFox RI. Sj\u0026ouml;gren\u0026apos;s syndrome. The Lancet. 2005;366(9482):321-31.\u003c/li\u003e\n \u003cli\u003eSeror R, Ravaud P, Mariette X, Bootsma H, Theander E, Hansen A, et al. EULAR Sj\u0026ouml;gren\u0026apos;s Syndrome Patient Reported Index (ESSPRI): development of a consensus patient index for primary Sj\u0026ouml;gren\u0026apos;s syndrome. Annals of the rheumatic diseases. 2011;70(6):968-72.\u003c/li\u003e\n \u003cli\u003eRamos-Casals M, Brito-Zer\u0026oacute;n P, Bombardieri S, Bootsma H, De Vita S, D\u0026ouml;rner T, et al. EULAR recommendations for the management of Sj\u0026ouml;gren\u0026apos;s syndrome with topical and systemic therapies. Ann Rheum Dis. 2020;79(1):3-18.\u003c/li\u003e\n \u003cli\u003eFox RI. Sj\u0026ouml;gren\u0026apos;s syndrome. The Lancet. 2005;366(9482):321-31.\u003c/li\u003e\n \u003cli\u003eKassan SS, Moutsopoulos HM. Clinical manifestations and early diagnosis of Sj\u0026ouml;gren syndrome. Arch Intern Med. 2004;164(12):1275-84.\u003c/li\u003e\n \u003cli\u003eAtzeni F, Sarzi-Puttini P, Lama N, Bonacci E, Bobbio-Pallavicini F, Montecucco C, et al. Anti-cyclic citrullinated peptide antibodies in primary Sj\u0026ouml;gren\u0026apos;s syndrome may be associated with non-erosive synovitis. Arthritis Res Ther. 2008;10(3):R51.\u003c/li\u003e\n \u003cli\u003eMori K, Iijima M, Koike H, Hattori N, Tanaka F, Watanabe H, et al. The wide spectrum of clinical manifestations in Sj\u0026ouml;gren\u0026apos;s syndrome-associated neuropathy. Brain. 2005;128(Pt 11):2518-34.\u003c/li\u003e\n \u003cli\u003eCha SI, Fessler MB, Cool CD, Schwarz MI, Brown KK. Lymphoid interstitial pneumonia: clinical features, associations and prognosis. Eur Respir J. 2006;28(2):364-9.\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":true,"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":"Sjögren's Syndrome, lymphocytic interstitial pneumonia, arthritis, lymphoma","lastPublishedDoi":"10.21203/rs.3.rs-6874147/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-6874147/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eObjective:\u003c/strong\u003e Sjögren's syndrome is a chronic autoimmune disease characterized by involvement of exocrine glands and lymphocytic infiltrates. The presented case demonstrates the rare findings of systemic involvement in Sjögren’s syndrome. \u003cstrong\u003eCase:\u003c/strong\u003e A 45-year-old woman, diagnosed with Sjögren's syndrome in the rheumatology clinic, presented with mildly raised erythematous skin lesions, joint pain and intermittent cough. Physical examination arthralgia, polycyclic cutaneous lesions and bibasilar rales. Ultrasonography revealed parotitis, electromyography revealed peripheral nervous system involvement and high resolution tomography revealed lymphocytic interstitial pneumonia. Azathioprine and Rituximab 1000 mg on days 0 and 14 were started every 6 months. There was significant response in joint involvement, acute phase reactans and near complete response in signs of interstitial lung disease findings.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eDiscussion:\u003c/strong\u003e Considering that it is a systemic disease, clinical findings including nonspecific findings should be carefully evaluated and targeted treatment algorithms in systemic involvement should be organized specifically according to the organ involvement.\u003c/p\u003e","manuscriptTitle":"Rare, Spesific, Systemic Organ Involvement in a Patient with a History of Oligoarthritis and Sjögren’s Syndrome: Case Report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-06-29 15:01:44","doi":"10.21203/rs.3.rs-6874147/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":"c7b83ab2-c38a-414d-9149-ae74f87ec827","owner":[],"postedDate":"June 29th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2025-06-29T15:01:44+00:00","versionOfRecord":[],"versionCreatedAt":"2025-06-29 15:01:44","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-6874147","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-6874147","identity":"rs-6874147","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}