Journal of Hepatology zyxwvutsrqponmlkjihgfedcbaZYXWVUTSRQPONMLKJIHGFEDCBA 1991; 21: 5X-582 Printed in Denmark AN rights reserved M unksgaard Copenhagen Copyright 0 European Association /or the Studs of the zyxwvutsrqponml Liver 1997 Journal of Hepatology ISSN 0168-8278 Case Report Subfulminant hepatic failure in autoimmune hepatitis type 1: an unusual form of presentation Denise Herzog’, Andree-Marie Rasquin-Weber’, Dominique Debray and Fernando Alvarez’ ‘Division of Gastroenterology-Nutrition, Department of Pediatrics, Sainte-Justine Hospital, University of Montreal, Canada and 2Division of Pediatric Hepatology, Kremlin-Bic&tre Hospital, Paris, France Autoimmune hepatitis type 1 is known to progress in- sidiously and in many cases cirrhosis is already estah- lished at the first presentation of symptoms. It affects mostly females, with peaks of incidence around 10 and 50 years of age. Suhfulminant hepatic failure is an unusual initial form of presentation of AIH type 1 and it was observed in three post-pubertal female patients. Rapid disease evolution or no response to immunosup- pressive therapy led to liver transplantation in all pa- ?” REE different subtypes of autoimmune hepatitis (AIH) have been described and classified by the presence of several non-organ-specific autoantibodies. AIH type 1 is characterized by the presence of smooth muscle (SMA) antibodies and anti-nuclear antibodies (ANA), AIH type 2 is characterized by the presence of liver kidney microsome antibodies (LKMl) and liver cytosol (LCl) antibodies, and AIH type 3 by anti- bodies against soluble liver antigens (SLA). Common features of the different subtypes are: predominance of female patients, hypergammaglobulinemia, and re- sponsiveness to immunosuppressive therapy (14). A genetic predisposition seems to exist, as AIH type 1 and 2 affect predominantly women expressing HLA class I Al,B8 and HLA class II DR3 antigens. AIH type 1 is the most frequent form of AIH, with inci- dence peaks around the age of 10 years and after the age of 50 years (3,5). The mean age of disease onset is 9.8 years (5). The youngest patient described within the pediatric age group, however, was 18 months old (6). In children, AIH presentation is usually insidious with Received 14 October 1996; revised 3 February; accepted 24 April 1997 Correspondence: E Alvarez, Division of Gastroenterology and Nutrition, Department of Pediatrics, University of Montreal, Sainte-Justine Hospital, 3175 CBte Sainte Catherine, Montreal, QC H3T lC5, Canada. Tel: 514-345-4626. Fax: 514-345-4999 578 tients. Two did not have cirrhosis, and the third had focal cirrhosis. The occurrence of the unusual suhful- minant form of autoimmune hepatitis in three late- pubertal girls (Tanner V) suggests that estrogen may play a role in the severity of the disease. Key words: Autoimmune hepatitis type 1; Female ado- lescent; Suhfulminant hepatic failure. progressive fatigue and jaundice (50-70%) (7,8), while 15% remain asymptomatic (9) and 20-30% present in a form indistinguishable from acute hepatitis (7,8). At presentation, 80% are jaundiced, nearly all patients have hepatomegaly, 50% splenomegaly, and 30-80% al- ready have cirrhosis (8). Other autoimmune diseases are frequently associated with AIH (2). Most AIH type 1 cases show a more favorable response to immunosup- pressive treatment (2,10), than other AIH subtypes, both in acute and chronic presentation (1 l), even if treatment does not prevent the progression of the dis- ease (12). Onset with fulminant hepatic failure (FHF), defined as acute liver failure and encephalopathy within 2 weeks after onset of jaundice (13), has seldom been reported in children with AIH type 2 (3,14,15) and rarely in children with AIH type 1 (16). AIH type 1 evolving to hepatic failure was believed to be mostly associated with a previously known symptomatic dis- ease (17). In this report we describe three cases of AIH type 1 presenting with subfulminant hepatic failure (SFHF), defined as acute liver failure and encepha- lopathy 2 weeks to 3 months after onset of jaundice (13) unusual in that each case occurred in the postpu- bertal period. Pertinent Clinical Data Patient 1 was a 16 10/12-year-old white female referred to Sainte-Justine Hospital in May 1993 for subfulmin-