Clinicopathological features of patients with autoimmune hepatitis type Ⅰ / 中华消化杂志

ABSTRACT

Objective We screened autoimmune hepatitis (AIH) from patients who had abnormal liver function without any viral marker in serum, and investigated the clinical and pathological features of AIH type Ⅰ. Methods According to the revised descriptive criteria and scoring system for diagnosis of autoimmune hepatitis proposed by the International Autoimmune Hepatitis Group, we examined the clinical and pathological characteristics of the patients who were diagnosed as AIH type Ⅰ. Results All patients (32 cases) diagnosed as AIH type Ⅰ were female. The age distribution showed a single peak in the decades of the fifties and sixties. Liver function test of these patients showed hepatic profile. Twenty eight patients (87.5%) had hypergammaglobulinemia, 26(81.3%) and elevated serum IgG level. Twenty six cases were positive for antinuclear antibody (ANA). Twelve patients (37.5%) had concurrent autoimmune diseases. Among fifteen patients who underwent liver biopsy, interface hepatitis and lobular hepatitis were observed in 11(73.3%) and 5(33.3%) patients, respectively. Rosetting of liver cells and liver fibrosis or cirrhosis were also detected in 3(20%) and 6(40%) patients respectively. Twenty one patients (65.5%) were considered to have a complete response to immunosuppressive therapy; 9(28.1%) relapsed; and 12(37.5%) sustained remission. Conclusions Autoimmune hepatitis type Ⅰ is mainly diagnosed in middle aged women, characterized by interface hepatitis on histologic examination, the presence of circulating autoantibody, hypergammaglobulinemia and response to immunosupressive treatment.