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原发性干燥综合征两种并存的罕见腺外表现:IgA肾病和自身免疫性肝炎。

Two Concomitant Rare Extraglandular Manifestations of Primary Sjögren's Syndrome: IgA Nephropathy and Autoimmune Hepatitis.

作者信息

Roongta Rashmi, Dey Sonali, Mondal Sumantro, Ghosh Alakendu

机构信息

Department of Clinical Immunology and Rheumatology, Institute of Postgraduate Medical Education and Research, Kolkata, West Bengal, India.

出版信息

Mediterr J Rheumatol. 2024 Jan 29;35(2):305-308. doi: 10.31138/mjr.260123.ina. eCollection 2024 Jun.

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.

摘要

原发性干燥综合征(pSS)是一种全身性自身免疫性疾病,很少出现多种腺体外表现。在此,我们报告一例以IgA肾病和自身免疫性肝炎为首发表现的原发性干燥综合征病例。她表现为多关节痛、干燥症状和持续疲劳,但肾脏和肝脏受累无症状。自身免疫性疾病可能有重叠的临床特征,偶尔会出现非特异性症状,导致诊断延迟。因此,必须对任何原发性干燥综合征患者进行全面评估,以便早期识别各种腺体外特征,并及时开始治疗以改善预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9b08/11350420/2f143eadd210/MJR-35-2-305-g001.jpg

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