Analysis of clinical features and liver histopathology of autoimmune liver diseases: an analysis of 109cases / 中华消化杂志

ABSTRACT

Objective To analyze the clinical characteristics of autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and AIH/PSC or/PBC overlap syndrome in order to further understand the autoimmune liver diseases (AILD). Methods One hundred and nine patients with AILDs confirmed pathologically were collected between Jan. 2004 and June 2006. Of 109 patients, AIH was found in 27 eases, PBC in 67 cases, PSC in 4 eases, AIH-PSC overlap syndrome in 1 case and AIH-PBC overlap syndrome in 10 cases. The clinical and laboratory data of all patients were assessed retrospectively. Results The AILD was predominantly found in middle-aged women (73.3% ,80/109), and the main clinical manifestations were jaundice, malaise, anorexia and pruritus. The age distribution of patients with AIH showed a single peak at 50 years. Elevated serum gamma globulin and IgG were found in patients with AIH, of whom 62.9% (17/27) were positive for anti-nuclear antibody (ANA) and 3 were positive for liver-kidney microsomes type 1 antibody. The main histological changes in severe AIH cases included interface hepatitis (77.7 %) and bridging necrosis. Most of the PBC patients were presented with elevated serum alkaline phosphatase, glutamyl transpeptidase and IgM. Fifty patients (74.6%) were positive for anti-mitochondrial antibody (AMA) and AMA-M2. The pathological examination showed that 28. 3% of the cases were in Ⅰ or Ⅱ stage and 71.7% in Ⅲ or Ⅳ stage in patients with PBC who received liver biopsy. The pathologic change of reduction or even disappearing of bile ducts was found in 62. 6% patients with PBC. The clinical and pathological manifestations in patients with AIH-PBC overlap syndrome had both characteristics of PBC and AIH. Three out of 10 patients with AIH-PBC overlap syndrome were positive for ANA and AMA/AMA-M2. Conclusion Since AILD is not rare in Chinese, its diagnosis should be based on the clinical presentation, biochemical, immunological and histologic changes.