Constrictive-effusive pericarditis as presenting feature of IgG4 - Related Disease (IgG4-RD): a case report

Constrictive-effusive pericarditis as presenting feature of IgG4 - Related Disease (IgG4-RD): a 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 Constrictive-effusive pericarditis as presenting feature of IgG4 - Related Disease (IgG4-RD): a case report Robert Fekete This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-6341057/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 IgG4 - Related Disease (IgG4-RD) is a fibroinflammatory disorder initially identified as autoimmune pancreatitis but subsequently shown in numerous other organs with common pathological hallmarks. Constrictive pericarditis is an unusual presentation of IgG4-RD. Case report This case report is about a 45-year-old previously healthy white male patient with complicated course of IgG4-Related Disease (IgG4-RD) with pancreatic cyst, constrictive effusive pericarditis likely triggered by 3 month antecedent Coxsackie virus infection with hand foot and mouth syndrome, who had 495 cc pericardial fluid drained, bilateral pleural effusions with 1 liter fluid drained from the right side. He was treated with methylprednisolone oral up to 48 mg daily. With each decrease in steroid dose (given maximum glucose on metabolic panel to 903) or holiday in steroid up to six weeks, he experienced worsening of positional and exertional shortness of breath. Rilonarcept treatment was not effective in improving cardiac constriction. Final diagnosis and resolution of symptoms were obtained after pericardiectomy showing abundant fibrosis and plasma cells staining for IgG4. Remission was maintained on rituximab infusion. Conclusion This case highlights difficulty in diagnosing and treating IgG4-RD. His initial IgG4 serum level was normal to only mildly elevated. Definitive diagnosis was via tissue biopsy which required open heart surgery to access. Prior to the diagnosis, he was treated with methylprednisolone which led to severely elevated glucose levels. Oral steroids and rilonarcept could not stop the progression of constrictive pericarditis. Pericardiectomy and subsequent treatment with rituximab led to continued remission. Rheumatology IgG4-RD constrictive effusive pericarditis rilonarcept rituximab case report Figures Figure 1 Figure 2 Figure 3 Figure 4 Background IgG4 - Related Disease (IgG4-RD) is a fibroinflammatory disorder initially identified as autoimmune pancreatitis but subsequently shown in numerous other organs with common pathological hallmarks [1,2]. Constrictive pericarditis is an unusual presentation of IgG4-RD. Case report This case report is about a 45-year-old previously healthy white male patient with complicated course of IgG4-Related Disease (IgG4-RD) with pancreatic cyst, constrictive effusive pericarditis likely triggered by 3 month antecedent Coxsackie virus infection with hand foot and mouth syndrome. Serum testing was positive for Coxsackie A7, A9, A16, A24 IgG at 1:1600. Patient began to develop progressive ascites, increased abdominal girth, as well as progressive bilateral lower extremity edema. He presented to the emergency room with exertional and positional shortness of breath. He had nighttime orthopnea. Physical examination was significant for anasarca, jugular venous distention, Kussmaul sign, tachycardia, enlarged and pulsatile liver, normal blood pressure. Electrocardiogram had decreased QRS amplitude but without electrical alternans. Emergent echocardiogram showed large pericardial effusion and signs of constriction including septal bounce. He had 495 cc pericardial fluid drained, which was sanguinous. There were also bilateral pleural effusions (Figure 1). Pleurocentesis was performed with 920 cc of yellow serous fluid which was transudative by Light’s criteria: lactate dehydrogenase fluid 74, serum 234 (units per liter), total protein fluid 1.9, serum 6.5, (grams per deciliter), albumin fluid 1.3, serum 3.3 (grams per deciliter). CD38 positive T cells and B cells were demonstrated on cytology. Bone marrow aspiration, X ray skeletal survey, and whole-body positron emission tomography were normal. He was discharged from the hospital and placed on indomethacin 50 mg BID and colchicine 0.6 mg BID. Given the return of exertional shortness of breath on this treatment for one month, he was switched to methylprednisolone oral up to 48 mg daily. Unfortunately, he developed excessive thirst, frequent urination, and tiredness on this steroid dose. Serum glucose was elevated to 903. He was admitted to the medical intensive care unit with glucose of 423 and was treated with intravenous insulin. Blood glucose subsequently stabilized on methylprednisolone 2 mg twice a day and weekly rilonarcept 220 mg subcutaneous treatment. Rilonarcept is a soluble receptor which traps interleukin-1 [3]. Diagnosis of probable IgG4-RD was made via pericardial biopsy during curative pericardiectomy 11 months after emergency room presentation. It showed fibrosis and abundant plasma cells (Figure 2) with more than 10% of plasma cells staining for IgG4 (Figure 3). IgG4 level increased to 162.5 mg/dl after complete cessation of steroid. Remission was maintained with intravenous rituximab treatment of 1000 mg weekly for two doses every six months. Treated IgG4-RD has good prognosis with normal quality of life. The patient likely had additional congestive hepatopathy with elevated GGT and alkaline phosphatase which resolved after pericardiectomy. There was also significant proteinuria with improvement on steroids, worsening after steroid holidays, and complete resolution after pericardiectomy, possibly due to minimal change disease from the initial Coxsackie infection. The highest random total protein to creatinine ratio was 6058 mg protein / gram creatinine. Renal biopsy showed mild chronic changes with about 9% global glomerulosclerosis and mild interstitial fibrosis and tubular atrophy. Potential biomarkers of IgG-RD were present before pericardial biopsy was obtained. Chemokine (C-X-C Motif) Ligand 9, (CXCL9), at 1180 pg/ml, normal <647 pg/mL, shown to be a putative biomarker of IgG4-RD and follicular lymphoma [2]. There was also elevation of cytokines. Interleukin-6 peaked at 24.5 pg/ml (normal <5 pg/ml) during the initial hospitalization for drainage of pericardial fluid and then resolved. About 8 months after presentation, during a six-week steroid holiday, soluble interleukin 2-receptor alpha (sIL-2Ralpha) peaked at 13285 pg/ml (normal up to 1891 pg/ml), indicating the T cell component of the disease [4]. Transforming growth factor Beta-1 (TGF-Beta-1) which is shown to be a part of models of signaling in IgG-RD was elevated to 21400 pg/ml (pre-11/19/2024 reference range 344-2382 pg/mL) [2]. Discussion In the initial phase of the disease, serum IgG4 levels were normal to mildly elevated. The highest pre-surgical level was 132 mg/dl (nl 2-96 mg/dl). Profoundly elevated IgG4 levels that are a hallmark of the disease at least above the 135 mg/dl cutoff of the 2020 revised comprehensive diagnostic criteria for IgG4-RD were not present until after pericardiectomy and complete cessation of steroid therapy [1]. Diagnosis of IgG4 Related Disease in the early phase with the main lesion being in a difficult to access area such as the pericardium is very challenging. Small salivary gland biopsy after the patient was already started on steroids showed sialadenitis but not IgG4 staining. Clinicians can be alert to clues such as clinical deterioration with each steroid holiday or dose reduction and elevation of putative biomarkers such as CXCL9, sIL-2Ralpha, and TGF-beta-1 until the diagnosis can be made by pathology. Declarations Patient perspective The patient is grateful for the care received and normalization of his quality of life. Acknowledgements None. Declarations Ethics approval and consent to participate Written informed consent was obtained from the patient for participation in this case report however, no ethical approval was needed. "The institution’s policy, therefore, is that a case report is not research that must be approved by the IRB." New York Medical College Policies and Procedures for the Conduct of Research Involving Human Subjects. [https://www.nymc.edu/media/schools-andcolleges/nymc/pdf/human-subjects-research/IRBmanual.pdf] Accessed on 3/24/2025. Consent for publication Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. Availability of data All supporting data were included in this manuscript. Patient clinical data is protected by the United States Health Insurance Portability and Accountability Act (HIPAA) of 1996. Competing interests The author declares that he does not have any competing interests Funding None. Author contributions RF: conception, data acquisition, and writing. References Umehara H, Okazaki K, Kawa S, et al. The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD. Modern Rheumatology. 4 May 2021;31(3):529-33. https://doi.org/10.1080/14397595.2020.1859710 Hara A, Watanabe T, Minaga K, et al. A positive cytokine/chemokine feedback loop establishes plasmacytoid DC–driven autoimmune pancreatitis in IgG4- related disease Akane Hara, JCI Insight. 2024;9(20):e167910. https://doi.org/10.1172/jci.insight.167910 Imazio M, Lazaros G, Gattorno M, LeWinter M, Abbate A, Brucato A, Klein A. Anti-interleukin-1 agents for pericarditis: a primer for cardiologists. European Heart Journal.14 August 2022;43(31):2946–57. https://doi.org/10.1093/eurheartj/ehab452 Karim AF, Eurelinks LEM, Bansie RD, et al. Soluble Interleukin-2 Receptor: A Potential Marker for Monitoring Disease Activity in IgG4-Related Disease. Mediators Inflamm. 2018 Mar 1:2018:6103064. doi: 10.1155/2018/6103064. eCollection 2018. PMID: 29686532 PMCID: PMC5854105 Masunaga A, Ishibashi F, Koh E. et al. Possible relationship between fibrosis of IgG4-related thymitis and the profibrotic cytokines, transforming growth factor beta 1, interleukin 1 beta and interferon gamma: a case report. Diagn Pathol. 2018 Jan 17;13:6. doi: 10.1186/s13000-018-0684-1 PMCID: PMC5811959 PMID: 29439708 Additional Declarations The authors declare no competing interests. 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-6341057","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":436067815,"identity":"6f2ca9a6-3b29-4edf-a7c6-b1893c66b6ce","order_by":0,"name":"Robert Fekete","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA9klEQVRIiWNgGAWjYFACHhAhxyABoj42gEjGxgOEtSQYA7UwMzDObABpZWwgXgszbwPENrxazNt7Dz6u/GEgJ9nef/Cx7Q6bOt32w0BbamyicWmROXMu2fBMgoGxNM9hZuPcM2kSZmcSgVqOpeU24NAiIZFjJtmQ8CdxnkQym3Ru22EJswNALYwNh/FpMf/ZkGBQP0/+MftvS5CW8w8JajFjBGpJkJZgZmNmBGm5QcgWnnPJkg1pBoYze5KNJXvb0iS33QDakoDPL+y9Bz822BjISxw/+PDDzzYbfrPz6Q8ffKixwakFB0ggTfkoGAWjYBSMAjQAAO/dXF4eaCYtAAAAAElFTkSuQmCC","orcid":"https://orcid.org/0000-0002-0627-8340","institution":"New York Medical College","correspondingAuthor":true,"prefix":"","firstName":"Robert","middleName":"","lastName":"Fekete","suffix":""}],"badges":[],"createdAt":"2025-03-31 02:49:49","currentVersionCode":1,"declarations":{"humanSubjects":true,"vertebrateSubjects":false,"conflictsOfInterestStatement":false,"humanSubjectEthicalGuidelines":true,"humanSubjectConsent":true,"humanSubjectClinicalTrial":false,"humanSubjectCaseReport":true,"vertebrateSubjectEthicalGuidelines":false},"doi":"10.21203/rs.3.rs-6341057/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-6341057/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":79675004,"identity":"9c8a7e7b-0c28-499f-bc06-0664cbb8f0d9","added_by":"auto","created_at":"2025-04-01 11:59:38","extension":"jpg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":233075,"visible":true,"origin":"","legend":"\u003cp\u003eCT chest showing large bilateral pleural effusions as well as pericardial effusion and thickened pericardium.\u003c/p\u003e","description":"","filename":"figure1.jpg","url":"https://assets-eu.researchsquare.com/files/rs-6341057/v1/79fafdeea962e443bd9f3ecb.jpg"},{"id":79675005,"identity":"94a77007-76d0-40ad-9e63-b825cc87c271","added_by":"auto","created_at":"2025-04-01 11:59:38","extension":"jpg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":805055,"visible":true,"origin":"","legend":"\u003cp\u003eHematoxylin - eosin stain showing abundant plasma cells and fibrosis\u003c/p\u003e","description":"","filename":"figure2.jpg","url":"https://assets-eu.researchsquare.com/files/rs-6341057/v1/40c3af494996bc498daebfd7.jpg"},{"id":79673982,"identity":"540db93f-8efb-47fe-a665-63449a61e1d3","added_by":"auto","created_at":"2025-04-01 11:51:38","extension":"jpg","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":638576,"visible":true,"origin":"","legend":"\u003cp\u003eIgG4 immunostaining of pericardium\u003c/p\u003e","description":"","filename":"figure3.jpg","url":"https://assets-eu.researchsquare.com/files/rs-6341057/v1/a6821d7655322e1f4c34d71c.jpg"},{"id":79673979,"identity":"c488287e-9e41-4f4a-8637-75f4b08a60c4","added_by":"auto","created_at":"2025-04-01 11:51:38","extension":"jpg","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":1022636,"visible":true,"origin":"","legend":"\u003cp\u003eIgG total immunostaining of pericardium\u003c/p\u003e","description":"","filename":"figure4.jpg","url":"https://assets-eu.researchsquare.com/files/rs-6341057/v1/1d9e1dc42f909f95d61d670f.jpg"},{"id":79675006,"identity":"3310a52e-5d2f-4a3c-b17a-14a3285450c0","added_by":"auto","created_at":"2025-04-01 11:59:43","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":2946064,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-6341057/v1/c55a34c2-03df-4bfb-adff-3ab701b314d4.pdf"}],"financialInterests":"The authors declare no competing interests.","formattedTitle":"\u003cp\u003eConstrictive-effusive pericarditis as presenting feature of IgG4 - Related Disease (IgG4-RD): a case report\u003c/p\u003e","fulltext":[{"header":"Background","content":"\u003cp\u003eIgG4 - Related Disease (IgG4-RD) is a fibroinflammatory disorder initially identified as autoimmune pancreatitis but subsequently shown in numerous other organs with common pathological hallmarks [1,2]. Constrictive pericarditis is an unusual presentation of IgG4-RD.\u003c/p\u003e"},{"header":"Case report","content":"\u003cp\u003eThis case report is about a 45-year-old previously healthy white male patient with complicated course of IgG4-Related Disease (IgG4-RD) with pancreatic cyst, constrictive effusive pericarditis likely triggered by 3 month antecedent Coxsackie virus infection with hand foot and mouth syndrome. Serum testing was positive for Coxsackie A7, A9, A16, A24 IgG at 1:1600. Patient began to develop progressive ascites, increased abdominal girth, as well as progressive bilateral lower extremity edema.\u003c/p\u003e\n\u003cp\u003eHe presented to the emergency room with exertional and positional shortness of breath. He had nighttime orthopnea. Physical examination was significant for anasarca, jugular venous distention, Kussmaul sign, tachycardia, enlarged and pulsatile liver, normal blood pressure. Electrocardiogram had decreased QRS amplitude but without electrical alternans. Emergent echocardiogram showed large pericardial effusion and signs of constriction including septal bounce.\u003c/p\u003e\n\u003cp\u003eHe had 495 cc pericardial fluid drained, which was sanguinous. There were also bilateral pleural effusions (Figure 1). \u0026nbsp;Pleurocentesis was performed with 920 cc of yellow serous fluid which was transudative by Light’s criteria:\u003c/p\u003e\n\u003cp\u003elactate dehydrogenase fluid 74, serum 234 (units per liter), total protein fluid 1.9, serum 6.5, (grams per deciliter), albumin fluid 1.3, serum 3.3 (grams per deciliter). CD38 positive T cells and B cells were demonstrated on cytology.\u003c/p\u003e\n\u003cp\u003eBone marrow aspiration, X ray skeletal survey, and whole-body positron emission tomography were normal. \u0026nbsp; He was discharged from the hospital and placed on indomethacin 50 mg BID and colchicine 0.6 mg BID. Given the return of exertional shortness of breath on this treatment for one month, he was switched to methylprednisolone oral up to 48 mg daily.\u003c/p\u003e\n\u003cp\u003eUnfortunately, he developed excessive thirst, frequent urination, and tiredness on this steroid dose. Serum glucose was elevated to 903. He was admitted to the medical intensive care unit with glucose of 423 and was treated with intravenous insulin. Blood glucose subsequently stabilized on methylprednisolone 2 mg twice a day and weekly rilonarcept 220 mg subcutaneous treatment. Rilonarcept is a soluble receptor which traps interleukin-1 [3].\u003c/p\u003e\n\u003cp\u003eDiagnosis of probable IgG4-RD was made via pericardial biopsy during curative pericardiectomy 11 months after emergency room presentation. It showed fibrosis and abundant plasma cells (Figure 2) with more than 10% of plasma cells staining for IgG4 (Figure 3). IgG4 level increased to 162.5 mg/dl after complete cessation of steroid. Remission was maintained with intravenous rituximab treatment of 1000 mg weekly for two doses every six months. Treated IgG4-RD has good prognosis with normal quality of life.\u003c/p\u003e\n\u003cp\u003eThe patient likely had additional congestive hepatopathy with elevated GGT and alkaline phosphatase which resolved after pericardiectomy. There was also significant proteinuria with improvement on steroids, worsening after steroid holidays, and complete resolution after pericardiectomy, possibly due to minimal change disease from the initial Coxsackie infection. The highest random total protein to creatinine ratio was 6058 mg protein / gram creatinine. Renal biopsy showed mild chronic changes with about 9% global glomerulosclerosis and mild interstitial fibrosis and tubular atrophy.\u003c/p\u003e\n\u003cp\u003ePotential biomarkers of IgG-RD were present before pericardial biopsy was obtained. Chemokine (C-X-C Motif) Ligand 9, (CXCL9), at 1180 pg/ml, normal \u0026lt;647 pg/mL, shown to be a putative biomarker of IgG4-RD and follicular lymphoma [2]. There was also elevation of cytokines. Interleukin-6 peaked at 24.5 pg/ml (normal \u0026lt;5 pg/ml) during the initial hospitalization for drainage of pericardial fluid and then resolved. About 8 months after presentation, during a six-week steroid holiday, soluble interleukin 2-receptor alpha (sIL-2Ralpha) peaked at 13285 pg/ml (normal up to 1891 pg/ml), indicating the T cell component of the disease [4]. Transforming growth factor Beta-1 (TGF-Beta-1) which is shown to be a part of models of signaling in IgG-RD was elevated to 21400 pg/ml (pre-11/19/2024 reference range 344-2382 pg/mL) [2].\u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eIn the initial phase of the disease, serum IgG4 levels were normal to mildly elevated. The highest pre-surgical level was 132 mg/dl (nl 2-96 mg/dl). Profoundly elevated IgG4 levels that are a hallmark of the disease at least above the 135 mg/dl cutoff of the 2020 revised comprehensive diagnostic criteria for IgG4-RD were not present until after pericardiectomy and complete cessation of steroid therapy [1].\u003c/p\u003e\n\u003cp\u003eDiagnosis of IgG4 Related Disease in the early phase with the main lesion being in a difficult to access area such as the pericardium is very challenging. Small salivary gland biopsy after the patient was already started on steroids showed sialadenitis but not IgG4 staining. Clinicians can be alert to clues such as clinical deterioration with each steroid holiday or dose reduction and elevation of putative biomarkers such as CXCL9, sIL-2Ralpha, and TGF-beta-1 until the diagnosis can be made by pathology.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003ePatient perspective\u003c/p\u003e\n\u003cp\u003eThe patient is grateful for the care received and normalization of his quality of life.\u003c/p\u003e\n\u003cp\u003eAcknowledgements\u003c/p\u003e\n\u003cp\u003eNone.\u003c/p\u003e\n\u003cp\u003eDeclarations\u003c/p\u003e\n\u003cp\u003eEthics approval and consent to participate\u003c/p\u003e\n\u003cp\u003eWritten informed consent was obtained from the patient for participation in this case\u003c/p\u003e\n\u003cp\u003ereport however, no ethical approval was needed.\u003c/p\u003e\n\u003cp\u003e\u0026quot;The institution\u0026rsquo;s policy, therefore, is that a case report is not research that must be\u003c/p\u003e\n\u003cp\u003eapproved by the IRB.\u0026quot;\u003c/p\u003e\n\u003cp\u003eNew York Medical College Policies and Procedures for the Conduct of Research\u003c/p\u003e\n\u003cp\u003eInvolving Human Subjects. [https://www.nymc.edu/media/schools-andcolleges/nymc/pdf/human-subjects-research/IRBmanual.pdf] Accessed on 3/24/2025.\u003c/p\u003e\n\u003cp\u003eConsent for publication\u003c/p\u003e\n\u003cp\u003eWritten informed consent was obtained from the patient for publication of\u003c/p\u003e\n\u003cp\u003ethis case report and any accompanying images. A copy of the written consent\u003c/p\u003e\n\u003cp\u003eis available for review by the Editor-in-Chief of this journal.\u003c/p\u003e\n\u003cp\u003eAvailability of data\u003c/p\u003e\n\u003cp\u003eAll supporting data were included in this manuscript. Patient clinical data is protected by\u003c/p\u003e\n\u003cp\u003ethe United States Health Insurance Portability and Accountability Act (HIPAA) of 1996.\u003c/p\u003e\n\u003cp\u003eCompeting interests\u003c/p\u003e\n\u003cp\u003eThe author declares that he does not have any competing interests\u003c/p\u003e\n\u003cp\u003eFunding\u003c/p\u003e\n\u003cp\u003eNone.\u003c/p\u003e\n\u003cp\u003eAuthor contributions\u003c/p\u003e\n\u003cp\u003eRF: conception, data acquisition, and writing.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eUmehara H, Okazaki K, Kawa S, et al. The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD. Modern Rheumatology. 4 May 2021;31(3):529-33. https://doi.org/10.1080/14397595.2020.1859710\u003c/li\u003e\n\u003cli\u003eHara A, Watanabe T, Minaga K, et al. A positive cytokine/chemokine feedback loop establishes plasmacytoid DC\u0026ndash;driven autoimmune pancreatitis in IgG4- related disease Akane Hara, JCI Insight. 2024;9(20):e167910. https://doi.org/10.1172/jci.insight.167910\u003c/li\u003e\n\u003cli\u003eImazio M, Lazaros G, Gattorno M, LeWinter M, Abbate A, Brucato A, Klein A. Anti-interleukin-1 agents for pericarditis: a primer for cardiologists. European Heart Journal.14 August 2022;43(31):2946\u0026ndash;57. https://doi.org/10.1093/eurheartj/ehab452\u003c/li\u003e\n\u003cli\u003eKarim AF, Eurelinks LEM, Bansie RD, et al. Soluble Interleukin-2 Receptor: A Potential Marker for Monitoring Disease Activity in IgG4-Related Disease. Mediators Inflamm. 2018 Mar 1:2018:6103064. doi: 10.1155/2018/6103064. eCollection 2018. PMID: 29686532 PMCID: PMC5854105\u003c/li\u003e\n\u003cli\u003eMasunaga A, Ishibashi F, Koh E. et al. Possible relationship between fibrosis of IgG4-related thymitis and the profibrotic cytokines, transforming growth factor beta 1, interleukin 1 beta and interferon gamma: a case report. Diagn Pathol. 2018 Jan 17;13:6. doi: 10.1186/s13000-018-0684-1 PMCID: PMC5811959 PMID: 29439708\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":true,"hideJournal":true,"highlight":"","institution":"New York Medical College","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":"IgG4-RD, constrictive effusive pericarditis, rilonarcept, rituximab, case report","lastPublishedDoi":"10.21203/rs.3.rs-6341057/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-6341057/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eBackground\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eIgG4 - Related Disease (IgG4-RD) is a fibroinflammatory disorder initially identified as autoimmune pancreatitis but subsequently shown in numerous other organs with common pathological hallmarks. Constrictive pericarditis is an unusual presentation of IgG4-RD.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCase report\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis case report is about a 45-year-old previously healthy white male patient with complicated course of IgG4-Related Disease (IgG4-RD) with pancreatic cyst, constrictive effusive pericarditis likely triggered by 3 month antecedent Coxsackie virus infection with hand foot and mouth syndrome, who had 495 cc pericardial fluid drained, bilateral pleural effusions with 1 liter fluid drained from the right side. He was treated with methylprednisolone oral up to 48 mg daily. With each decrease in steroid dose (given maximum glucose on metabolic panel to 903) or holiday in steroid up to six weeks, he experienced worsening of positional and exertional shortness of breath. Rilonarcept treatment was not effective in improving cardiac constriction. Final diagnosis and resolution of symptoms were obtained after pericardiectomy showing abundant fibrosis and plasma cells staining for IgG4. Remission was maintained on rituximab infusion.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusion\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis case highlights difficulty in diagnosing and treating IgG4-RD. His initial IgG4 serum level was normal to only mildly elevated. Definitive diagnosis was via tissue biopsy which required open heart surgery to access. Prior to the diagnosis, he was treated with methylprednisolone which led to severely elevated glucose levels. Oral steroids and rilonarcept could not stop the progression of constrictive pericarditis. Pericardiectomy and subsequent treatment with rituximab led to continued remission.\u003c/p\u003e","manuscriptTitle":"Constrictive-effusive pericarditis as presenting feature of IgG4 - Related Disease (IgG4-RD): a case report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-04-01 11:51:33","doi":"10.21203/rs.3.rs-6341057/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":"73756a78-b3e4-4fac-b1e5-f4625bc80cdf","owner":[],"postedDate":"April 1st, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[{"id":46494208,"name":"Rheumatology"}],"tags":[],"updatedAt":"2025-04-01T11:51:33+00:00","versionOfRecord":[],"versionCreatedAt":"2025-04-01 11:51:33","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-6341057","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-6341057","identity":"rs-6341057","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}