Overlap syndrome of primary biliary cirrhosis and autoimmune hepatitis: a retrospective study of 115 cases of autoimmune liver disease

The aim of this retrospective study was to compare clinical, biological, and histological features and treatment response in 115 patients with overlap syndrome (OS), autoimmune hepatitis (AIH) or primary biliary cirrhosis (PBC). Consecutive patients with AIH, PBC or OS followed between 1984 and 2005 in five different centers were included . All data were re-evaluated using current diagnostic criteria of each disease. Fifteen patients had OS (13 females), 48 AIH (40 females) and 52 PBC (49 females). Patients with OS were significantly younger than patients with PBC (median age: 44 vs 59 years). Jaundice (20%) and pruritus (20%) were the main initial symptoms in OS. Patients with OS had serum transaminase and γ globulin levels significantly higher than patients with PBC; serum alkaline phosphatase, gamma-glutamyl-transpeptidase and IgM levels were significantly higher in OS than in patients with AIH. Histological analysis showed moderate or severe piecemeal necrosis in 86% and destructive cholangitis in 93% in OS group. Among 11 patients with OS treated with ursodeoxycholic acid (UDCA) or immunosuppressors alone, only 6 had a complete biochemical response. In contrast, all patients with OS receiving combined therapy, as first or second line, responded, 5 patients to the combination corticoste-roids-azathioprine-UDCA and 2 to the combination cyclosporine-UDCA. OS is not rare and accounts for 13.9% of patients with autoimmune liver disease in our series. Combination of immunosuppressors and UDCA appears the most efficient treatment in these patients. L’objectif de cette étude rétrospective était de comparer les caractéristiques cliniques, biologiques et histologiques ainsi que la réponse aux traitements de 115 malades atteints d’un syndrome de chevauchement (SC), d’une hépatite auto-immune (HAI) ou d’une cirrhose biliaire primitive (CBP). Ont été inclus les malades atteints d’HAI, de CBP ou de SC pris en charge entre 1984 et 2005 dans cinq centres de Champagne-Ardenne. Tous les dossiers ont été réévalués selon les critères diagnostiques actuels de chaque maladie. Quinze malades étaient atteints d’un SC (13 femmes), 48 d’une HAI (40femmes) et 52 d’une CBP (49 femmes). Le groupe SC était significativement plus jeune que le groupe CBP (âge médian : 44 vs 59 ans). L’ictère (20 %) et le prurit (20 %) étaient les principaux signes cliniques inauguraux du SC. Les malades ayant un SC avaient une activité sérique des transaminases et des taux de γ-globulines significat