Seo Suk, Toutounjian Raphael, Conrad Andrew, Blatt Larry, Tong Myron J
Medicine and Surgery, David Geffen School of Medicine at UCLA, The Pfleger Liver Institute, Los Angeles, California 90095-7302, USA.
J Gastroenterol Hepatol. 2008 Sep;23(9):1410-4. doi: 10.1111/j.1440-1746.2008.05365.x. Epub 2008 Mar 27.
Autoimmune hepatitis (AIH) is an idiopathic disease with diverse clinical manifestations. The aims of the present study were: (i) to describe the clinical characteristics of AIH patients in a community clinic setting; and (ii) to determine factors which were associated with poor clinical outcomes.
A retrospective review was performed on 72 AIH patients who: (i) had pretreatment sera: (ii) were treatment-naïve at presentation; and (iii) had a minimum of 24 months of follow up.
On initial presentation, 22 (30%) had an acute onset of symptoms simulating acute viral hepatitis, 34 (47%) had chronic symptoms of greater than 6 months duration, and the remaining 16 (22%) were asymptomatic. Twenty-six (36%) had coexisting autoimmune diseases. Anti-nuclear antibody (ANA) was positive in 73% of the patients, and antismooth muscle antibody was positive in 15% of ANA-negative patients. Those few patients who tested positive for soluble liver antigen, anti-liver-kidney, microsomal antibody type-1, and anti-mitochondrial antibody were all also ANA positive. The median (range) duration of follow up was 98 (24-331) months. After immunosuppressive therapy, 26 of 72 (36%) remained in remission without further treatment. However, 46 (64%) required maintenance immunosuppression. Three patients who presented under the age of 20 years progressed to liver failure while on therapy and died while waiting for liver transplantation. Two other patients developed hepatocellular carcinoma (HCC) while on therapy and died.
A majority of AIH patients have an excellent prognosis. However, presentation at a younger age is a predictor of poor disease outcome and, although uncommon, HCC may develop during the late stages of cirrhosis.
自身免疫性肝炎(AIH)是一种临床表现多样的特发性疾病。本研究的目的是:(i)描述社区诊所环境中AIH患者的临床特征;(ii)确定与不良临床结局相关的因素。
对72例AIH患者进行回顾性研究,这些患者:(i)有治疗前血清样本;(ii)就诊时未接受过治疗;(iii)至少随访24个月。
初次就诊时,22例(30%)有类似急性病毒性肝炎的急性症状发作,34例(47%)有持续超过6个月的慢性症状,其余16例(22%)无症状。26例(36%)合并自身免疫性疾病。73%的患者抗核抗体(ANA)呈阳性,15%的ANA阴性患者抗平滑肌抗体呈阳性。少数可溶性肝抗原、抗肝肾微粒体抗体1型和抗线粒体抗体检测呈阳性的患者ANA也呈阳性。随访时间中位数(范围)为98(24 - 331)个月。免疫抑制治疗后,72例中有26例(36%)在无需进一步治疗的情况下仍处于缓解期。然而,46例(64%)需要维持免疫抑制治疗。3例20岁以下就诊的患者在治疗期间进展为肝衰竭,在等待肝移植时死亡。另外2例患者在治疗期间发生肝细胞癌(HCC)并死亡。
大多数AIH患者预后良好。然而,年轻时就诊是疾病不良结局的一个预测因素,并且尽管不常见,但HCC可能在肝硬化晚期发生。