Gregorio G V, Portmann B, Karani J, Harrison P, Donaldson P T, Vergani D, Mieli-Vergani G
Department of Child Health, Institute of Liver Studies, and Department of Radiology, King's College Hospital, London, England.
Hepatology. 2001 Mar;33(3):544-53. doi: 10.1053/jhep.2001.22131.
To investigate whether sclerosing cholangitis with an autoimmune serology characteristic of autoimmune hepatitis (AIH) and AIH are distinct entities, we studied 55 consecutive children with clinical and/or biochemical evidence of liver disease and circulating antinuclear (ANA), anti-smooth muscle (SMA), and/or liver-kidney-microsomal type 1 (LKM1) autoantibodies. They underwent liver biopsy, direct cholangiography, sigmoidoscopy, and rectal biopsy at presentation. Twenty-eight were diagnosed as AIH in the absence and 27 autoimmune sclerosing cholangitis (ASC) in the presence of radiological features of cholangiopathy. Twenty-six ASC and 20 AIH had ANA and/or SMA; 1 ASC and 8 AIH LKM1 autoantibody. Similarities between the 2 conditions included most clinical and biochemical parameters and a lower frequency of HLA DR4. Inflammatory bowel disease and histological biliary changes were more common in ASC; coagulopathy, hypoalbuminemia, lymphocytic periportal hepatitis, and HLA DR3 were more common in AIH. Histological biliary changes were observed in 65% of ASC and 31% of AIH patients. Eighty-nine percent responded to immunosuppression. Follow-up liver biopsies from 17 ASC and 18 AIH patients had similarly reduced inflammatory activity and no progression to cirrhosis. Sixteen follow-up cholangiograms from AIH patients and 9 from ASC patients were unchanged, while 8 ASC patients showed a progressive cholangiopathy. One child with AIH and ulcerative colitis developed sclerosing cholangitis 8 years after presentation. At 2 to 16 years (median, 7 years) from presentation, all patients are alive, including 4 ASC patients who underwent liver transplantation. In conclusion, ASC and AIH are similarly prevalent in childhood; cholangiography is often needed to distinguish between these 2 entities, which are likely to lie within the same disease process.
为了研究具有自身免疫性肝炎（AIH）自身免疫血清学特征的硬化性胆管炎与AIH是否为不同的疾病实体，我们对55例具有肝病临床和/或生化证据以及循环抗核抗体（ANA）、抗平滑肌抗体（SMA）和/或1型肝肾微粒体抗体（LKM1）的连续儿童进行了研究。他们在就诊时接受了肝活检、直接胆管造影、乙状结肠镜检查和直肠活检。28例在无胆管病变放射学特征的情况下被诊断为AIH，27例在有胆管病变放射学特征的情况下被诊断为自身免疫性硬化性胆管炎（ASC）。26例ASC和20例AIH有ANA和/或SMA；1例ASC和8例AIH有LKM1自身抗体。这两种疾病的相似之处包括大多数临床和生化参数以及较低的HLA DR4频率。炎症性肠病和组织学上的胆管改变在ASC中更常见；凝血病、低白蛋白血症、淋巴细胞性汇管区肝炎和HLA DR3在AIH中更常见。65%的ASC患者和31%的AIH患者观察到组织学上的胆管改变。89%的患者对免疫抑制有反应。17例ASC患者和18例AIH患者的随访肝活检显示炎症活动同样减轻，且无进展为肝硬化。16例AIH患者和9例ASC患者的随访胆管造影结果未改变，而8例ASC患者显示进行性胆管病变。1例患有AIH和溃疡性结肠炎的儿童在就诊8年后发生了硬化性胆管炎。从就诊起2至16年（中位数为7年），所有患者均存活，包括4例接受肝移植的ASC患者。总之，ASC和AIH在儿童中同样常见；通常需要胆管造影来区分这两种疾病实体，它们可能处于同一疾病过程中。
