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一种罕见的重叠情况:干燥综合征合并自身免疫性肝炎。

A Rare Overlap: Sjögren's Syndrome With Autoimmune Hepatitis.

作者信息

Prasaanth Sharan, Sivasubramanian Dhiran

机构信息

Internal Medicine, Coimbatore Medical College and Hospital, Coimbatore, IND.

Critical Care Medicine, Christian Medical College, Vellore, Vellore, IND.

出版信息

Cureus. 2024 Nov 24;16(11):e74334. doi: 10.7759/cureus.74334. eCollection 2024 Nov.

Abstract

Sjögren's syndrome (SS) is a chronic autoimmune disorder primarily affecting exocrine glands, leading to symptoms such as dry mouth and dry eyes. While SS can occur as a primary condition, it may overlap with other autoimmune diseases, complicating management. Autoimmune hepatitis (AIH), a liver disorder characterized by elevated serum globulins and liver-specific autoantibodies, can co-occur with SS, although this overlap is rare. We report a case of a 45-year-old woman with primary SS who developed abdominal distension, pedal edema, and abnormal liver function tests, raising suspicion of an overlap syndrome. Autoimmune profiling confirmed primary SS, while liver biopsy indicated AIH and chronic liver disease with portal hypertension. The patient received immunosuppressive therapy, including corticosteroids and azathioprine, along with supportive care for cirrhosis led to stabilization. This case highlights the diagnostic challenges and the need for clinical vigilance in identifying autoimmune overlap syndromes to ensure appropriate management.

摘要

干燥综合征(SS)是一种主要影响外分泌腺的慢性自身免疫性疾病,会导致口干、眼干等症状。虽然SS可作为原发性疾病出现,但它可能与其他自身免疫性疾病重叠,使治疗变得复杂。自身免疫性肝炎(AIH)是一种以血清球蛋白升高和肝脏特异性自身抗体为特征的肝脏疾病,可与SS同时发生,尽管这种重叠情况很少见。我们报告一例45岁患有原发性SS的女性病例,该患者出现腹胀、足部水肿和肝功能检查异常,引发了对重叠综合征的怀疑。自身免疫谱分析证实为原发性SS,而肝活检显示为AIH以及伴有门静脉高压的慢性肝病。患者接受了包括皮质类固醇和硫唑嘌呤在内的免疫抑制治疗,同时针对肝硬化进行了支持性治疗,病情得以稳定。该病例凸显了诊断方面的挑战以及在识别自身免疫重叠综合征时临床保持警惕以确保恰当治疗的必要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2d69/11667356/7b0f11fa2166/cureus-0016-00000074334-i01.jpg

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