Abstract
自身免疫性肝炎(autoimmune hepatitis,AIH)是以肝脏的慢性炎症和免疫学异常为主要病变的一种器官特异性的自身免疫性疾病,其临床特征是女性易患,常伴发肝外自身免疫性疾病,以及血清氨基转移酶升高、高球蛋白血症、血清多种自身抗体、界面性肝炎和汇管区浆细胞浸润,对免疫抑制治疗应答[1-2].近年提出的IgG4相关性自身免疫性肝炎(IgG4-associated autoimmune hepatitis,IgG4-AIH),作为AIH的一个新亚型,由于其独特的临床和病理学特征,因而受到越来越多的关注.IgG4 AIH具有AIH的基本特征,同时又具有新的特点:血清IgG4水平升高,组织学IgG4阳性浆细胞浸润,对激素的治疗反应好且复发率低[3-4].然而IgG4 AIH的发病率和临床特征尚不十分清楚.目前,国内关于IgG4-AIH的病例报道和研究极少,迫切需要对其进行系统的了解和深入的研究,以探讨其流行病学、临床特征和发病机制,更好地指导临床实践. 关键词：肝炎,自身免疫性;IgG4
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