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自身免疫性肝炎(AIH)是一种特发性疾病,无明确病因,以高γ-球蛋白血症、存在自身抗体、女性易患为特点。诊断分为三型,Ⅰ型、Ⅱ型间有相互排斥的自身抗体和不同的临床特点,Ⅲ型AIH是不确定的类型。对免疫抑制治疗应答是AIH的特点之一,特别是在完全应答后出现复发时,支持AIH的诊断。IAIHG推荐对确定或可能诊断的AIH患者进行免疫抑制治疗,以改善患者生活质量和预后,对免疫抑制治疗应答较差者也不能排除AIH的诊断,但对皮质类固醇无应答的患者应进行逆行胰胆管造影或磁共振胆管造影术(MRCP),以排除原发性硬化性胆管炎等可能,AIH所致的终末期肝病是肝移植术的最佳指征之一。
Autoimmune hepatitis (AIH) is an idiopathic disease characterized by a high incidence of gammaglobulinemia, the presence of autoantibodies, and predisposition to women, without a definitive cause. Diagnosis is divided into three types, type I, type II have mutually exclusive autoantibodies and different clinical features, type III AIH is uncertain type. The response to immunosuppressive therapy is one of the hallmarks of AIH, especially in cases of relapse after complete response, supporting the diagnosis of AIH. IAIHG recommends immunosuppressive therapy for patients with definite or probable AIH to improve patient quality of life and prognosis, and those who do not respond well to immunosuppressive therapy should not rule out the diagnosis of AIH but those who do not respond to corticosteroids should undergo retrograde pancreatic Bile duct angiography or magnetic resonance cholangiopancreatography (MRCP) to rule out the possibility of primary sclerosing cholangitis, AIH-induced end-stage liver disease is one of the best indications for liver transplantation.