[A case of auto-immune hepatitis associated with primary Sjogren's syndrome].

Yong Dae Kwon, Hong Sik Lee, Chul Hee Park, Yoon Tae Jeen, Hoon Jai Chun, Sang Woo Lee, Jai Hyun Choi, Chang Duck Kim, Ho Sang Ryu, Jin Hai Hyun
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Abstract

Auto-immune hepatitis is a chronic necroinflammatory liver disorder that is characterized by hypergammaglobulinemia, auto-antibodies in serum, and, on histological examination, the presence of periportal hepatitis. Although it can be associated with a number of other auto-immune diseases, Sjogren's syndrome is rarely associated with auto-immune hepatitis. We herein report an unusual case of auto-immune hepatitis associated with primary Sjogren's syndrome. A 39-year-old woman was admitted to our hospital due to jaundice. Laboratory data showed negative viral hepatitis marker, increased serum IgG level, positive anti- nuclear antibody, and an increased rheumatoid factor titer. The patient had no history of taking medications and alcohol. Based on characteristic clinical features, liver biopsy findings, positive Schirmer's test, and salivary scintigraph, she was diagnosed as having auto-immune hepatitis and Sjogren's syndrome. The patient achieved complete remission with steroid monotherapy.

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自身免疫性肝炎合并原发性干燥综合征1例。
自身免疫性肝炎是一种慢性坏死性炎症性肝脏疾病,其特征是高γ球蛋白血症,血清中存在自身抗体,组织学检查显示存在门静脉周围肝炎。虽然干燥综合征可能与许多其他自身免疫性疾病有关,但它很少与自身免疫性肝炎有关。我们在此报告一例罕见的自身免疫性肝炎合并原发性干燥综合征。一名39岁女性因黄疸入院。实验室数据显示病毒性肝炎标志物阴性,血清IgG水平升高,抗核抗体阳性,类风湿因子滴度升高。患者无药物和酒精史。根据特征性的临床表现、肝活检结果、阳性的Schirmer试验和唾液扫描图,诊断为自身免疫性肝炎和干燥综合征。患者通过类固醇单药治疗获得完全缓解。
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