Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, 200 First Street SW, Rochester, MN 55905, USA.
Best Pract Res Clin Gastroenterol. 2011 Dec;25(6):689-700. doi: 10.1016/j.bpg.2011.09.011.
Autoimmune hepatitis has diverse clinical phenotypes that challenge conventional diagnostic criteria and treatment strategies. The goals of this review are to characterize these special populations and provide guidelines for their management. Patients with acute or acute severe (fulminant) presentations may have centrilobular zone 3 hepatic necrosis, but they can respond to conventional corticosteroid therapy. Asymptomatic mild disease warrants corticosteroid treatment because spontaneous resolution is uncertain and 10-year survival is less than expected. Male gender or the absence of conventional autoantibodies does not preclude the diagnosis or need for treatment, and patients with cholestatic changes warrant cholangiography and possible combination therapy with ursodeoxycholic acid. Different ethnic groups commonly have advanced hepatic fibrosis, rapidly progressive disease, or cholestatic features, and elderly patients typically respond well to corticosteroid therapy. Pregnancy is usually well-tolerated by mother and foetus but requires protection against postpartum exacerbation. Special populations must be recognized and treated with tailored regimens.
自身免疫性肝炎具有多种临床表型,这给传统的诊断标准和治疗策略带来了挑战。本综述的目的是描述这些特殊人群,并为其治疗提供指导。急性或急性重症(暴发性)表现的患者可能存在中央静脉区 3 区肝坏死,但他们可能对常规皮质类固醇治疗有反应。无症状的轻度疾病需要皮质类固醇治疗,因为自发性缓解不确定,10 年生存率低于预期。男性性别或缺乏常规自身抗体并不排除诊断或治疗的需要,伴有胆汁淤积改变的患者需要胆管造影,并可能需要熊去氧胆酸联合治疗。不同的种族群体通常有进展性肝纤维化、快速进展的疾病或胆汁淤积的特征,老年患者通常对皮质类固醇治疗反应良好。妊娠通常对母亲和胎儿耐受良好,但需要预防产后恶化。特殊人群必须被识别,并采用量身定制的治疗方案进行治疗。