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
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