P0320 AUTOIMMUNE HEPATITIS: MULTICENTRE DATA AT DIAGNOSIS IN 245 CHILDREN

Introduction: Autoimmune hepatitis (AIH) is a progressive inflammatory disease of the liver of unknown etiology. AIH spontaneously progresses toward cirrhosis and liver failure but often shows a good response to immunossupressive treatment. AIH is the most common etiology of chronic hepatitis in children in Argentina. Aim: to assess demographic, clinical, laboratory and histological data of patients with AIH at diagnosis. Methods: Medical records of 245 patients with definite AIH according to the scoring system described by the “International Autoimmune Hepatitis Group” seen from 1–1–93 to 31–8–03 were retrospectively reviewed. Results: 187/245 patients (76.3%) were females. Type 1 was found in 227/245 (92.6%) cases (SMA: 72/227 (31.7%), FAN: 7/227 (3%), SMA/FAN: 139/227 (61.2%)) and type 2 (LKM1) in 18/245 (7.3%). Mean age at onset: 9.7±3.7 years. Personal and familial antecedents of autoimmune disease were observed in: 25/229 (11%) and in 90/230 (39%) cases respectively. Antecedents of a previous episode of jaundice was found in 85/245 (34.6%) patients, gastrointestinal bleeding in 16/245 (6.5%), ascitis in 10/245 (4%), peritonitis in 1/245 (0.4%). Acute hepatitis like syndrome was the most frequent form of onset. Presenting features included: nonspecific symptoms such as fatigue, anorexia: 129/245 (52.6%), spider nevi: 120/245 (49%), palmar erythema: 112/245 (45.7%), jaundice: 154/245 (62.8%), hepatomegaly: 195/245 (79.5%), splenomegaly: 105/245 (42.6%) and extrahepatic autoimmune disease: 59/245 (24%). Laboratory: bilirubin was elevated in 72.2% of patients (median: 3 ×normal values (NV)), ALT/AST (median) 12/15 ×NV, albumin (mean) 38 g/l, high levels of gammaglobulins in 79.6% (median: 3.3 ×NV), prothrombin time <60%: 60/245 (24.4%). Histological features: cirrhosis 60/189 (31.7%). Conclusion: Type 1, female gender and presentation as acute hepatitis were the most frequent observations in our experience. Extrahepatic autoimmune disease and complications of end-stage liver disease were not frequently found at diagnosis.