Autoimmune-type chronic active hepatitis in children. A report of 23 cases at a Hospital in Northwestern Mexico

Abstract

Introduction: Autoimmune type chronic active hepatitis (AI-CAH) is a rare pediatric disorder whose principal characteristics include hepatocellular dysfunction and active tissue damage with evolution to cirrhosis in 25-30% of cases. Objective: Our aim was to ascertain the evolution of 23 children with AI-CAH treated between 1978 and 2004 at the Hospital Infantil del Estado de Sonora in the Sonora State capital of Hermosillo in northern Mexico. Materials and methods: We conducted a retrospective review that included the following variables: age; sex; personal antecedents; signs and symptoms; laboratory and medical office studies; histologic tissue pattern obtained by biopsy; treatment; evolution, and mortality. Results: Thirteen males and 10 females participated in the study; predominant signs were hyporexia, fatigue-hepatomegalia, and icterus. Ten patients presented moderate anemia and six patients, leukopenia and thrombocytopenia. Eighteen patients presented hypergammaglobulinemia. Twenty three patients had percutaneous (p.c.) liver biopsy, and we observed the following histologic pattern: rupture of the limiting plate; necrotic foci; cholangiolar proliferation, and fibrotic bridges; in addition, four patients demonstrated precirrhotic changes. Twenty one children received prednisone for periods of between 1 and 12 years; in seven cases, children were administered azatioprine. Six children presented histologic, biochemical, and clinical remission, nine experienced biochemical remission but presented inflammatory activity, five abandoned treatment, and three died due to cirrhosis, liver insufficiency, and other complications. Discussion: To date, AI-CAH etiology continues to be unknown, while treatment has not been importantly modified in 50 years and continues to consist of prednisone alone or in association with azathioprine.