Two Concomitant Rare Extraglandular Manifestations of Primary Sjögren's Syndrome: IgA Nephropathy and Autoimmune Hepatitis
- PMID: 39211028
- PMCID: PMC11350420
- DOI: 10.31138/mjr.260123.ina
Two Concomitant Rare Extraglandular Manifestations of Primary Sjögren's Syndrome: IgA Nephropathy and Autoimmune Hepatitis
Abstract
Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease and can rarely present with multiple extraglandular manifestations. Here we report a case of pSS with concomitant IgA nephropathy and autoimmune hepatitis as the initial manifestations. She presented with polyarthralgia, sicca symptoms and persistent fatigue but was asymptomatic for renal and liver involvement. Autoimmune diseases can have overlapping clinical features and occasionally, manifest nonspecific symptoms leading to delay in diagnosis. It is therefore imperative to thoroughly evaluate any patient of pSS for early recognition of the diverse extraglandular features and initiate prompt treatment to improve outcome.
Keywords: IgA nephropathy; Sjögren’s syndrome; autoimmune; glomerulonephritis; hepatitis.
© 2024 The Mediterranean Journal of Rheumatology (MJR).
Conflict of interest statement
None of the authors have any potential conflicts of interest to disclose.
Figures
Similar articles
-
Glomerulonephritis with severe nephrotic syndrome induced by immune complexes composed of galactose-deficient IgA1 in primary Sjögren's syndrome: a case report.BMC Nephrol. 2021 Mar 25;22(1):108. doi: 10.1186/s12882-021-02306-0. BMC Nephrol. 2021. PMID: 33765955 Free PMC article. Review.
-
Primary Sjögren's syndrome: Extraglandular manifestations and hydroxychloroquine therapy.Clin Rheumatol. 2017 Nov;36(11):2455-2460. doi: 10.1007/s10067-017-3822-3. Epub 2017 Sep 14. Clin Rheumatol. 2017. PMID: 28913747
-
[Analysis of clinical features and prognosis in patients with primary Sjögren's syndrome and autoimmune liver disease].Beijing Da Xue Xue Bao Yi Xue Ban. 2020 Oct 18;52(5):886-891. doi: 10.19723/j.issn.1671-167X.2020.05.015. Beijing Da Xue Xue Bao Yi Xue Ban. 2020. PMID: 33047724 Free PMC article. Chinese.
-
Ten-year observation of patients with primary Sjögren's syndrome: Initial presenting characteristics and the associated outcomes.Int J Rheum Dis. 2019 May;22(5):929-933. doi: 10.1111/1756-185X.13464. Epub 2018 Dec 26. Int J Rheum Dis. 2019. PMID: 30588773
-
Anti-Sjögren's-syndrome-related antigen A autoantibodies (Anti-SSA antibody) and meningoencephalitis: Sjögren's syndrome waiting to be unveiled? A case series and review of literature.Rheumatol Int. 2021 Oct;41(10):1855-1866. doi: 10.1007/s00296-020-04716-z. Epub 2020 Oct 11. Rheumatol Int. 2021. PMID: 33040168 Review.
Cited by
-
Hypokalemic Periodic Paralysis in a Patient With Primary Sjögren's Syndrome and Distal Renal Tubular Acidosis: A Case Report.Clin Med Insights Case Rep. 2025 Aug 30;18:11795476251372407. doi: 10.1177/11795476251372407. eCollection 2025. Clin Med Insights Case Rep. 2025. PMID: 40894112 Free PMC article.
References
-
- Baldini C, Pepe P, Quartuccio L, Priori R, Bartoloni E, Alunno A, et al. Primary Sjogren’s syndrome as a multi-organ disease: impact of the serological profile on the clinical presentation of the disease in a large cohort of Italian patients. Rheumatology (Oxford) 2014. May;53(5):839–44. - PubMed
-
- Pattrapornpisut P, Avila-Casado C, Reich HN. IgA Nephropathy: Core Curriculum 2021. Am J Kidney Dis 2021. Sep;78(3):429–41. - PubMed
-
- Hennes EM, Zeniya M, Czaja AJ, Parés A, Dalekos GN, Krawitt EL, et al. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology 2008;48:169–76. - PubMed
-
- Chen Y, Zhao X, Tang D, Xu C, Sun L, Sun L, et al. IgA nephropathy in two patients with Sjögren’s syndrome: one with concomitant autoimmune hepatitis. Intern Med 2010;49(1):37–43. - PubMed
-
- Markowitz G. Updated Oxford Classification of IgA nephropathy: a new MEST-C score. Nat Rev Nephrol 2017;13(7):385–6. - PubMed
Publication types
LinkOut - more resources
Full Text Sources
Miscellaneous