title: Autoimmune hepatitis: effect of symptoms and cirrhosis on natural history and outcome.
abstract: Although the natural history of autoimmune hepatitis (AIH) has been characterized, little is known about patients who present asymptomatically. Consequently, whether they require immunosuppressive therapy with its associated complications is unclear. To compare the natural history of asymptomatic AIH with symptomatic AIH, a large cohort of patients from a single center was examined. All patients with a clinical diagnosis of AIH were reassessed using the revised criteria of the International Autoimmune Hepatitis Group. Liver histology, response to therapy, and survival were assessed. Patients asymptomatic at presentation (n = 31) had lower serum aminotransferase, bilirubin, and immunoglobulin G (IgG) values at baseline. Half of the asymptomatic patients received no therapy, and their survival was no different from that of the total cohort. Ten-year survival was 80.0% (62.5%-97.5%) in the asymptomatic group and 83.8% (75.1%-92.6%) in the symptomatic patients (P = NS). Survival to liver-related endpoints at 10 years was similar in both groups: 89.5% (75.7%-100%) asymptomatic and 83.8% (75.1%-92.6%) symptomatic patients (P = NS). Patients with cirrhosis at baseline had poorer 10-year survival (61.9% [CI 44.9%-78.9%]) than those without cirrhosis at presentation (94.0% [CI 87.4%-100%]) (P = .003) regardless of whether they presented with symptoms or whether they received immunosuppressive therapy. In conclusion, patients with AIH who are asymptomatic at presentation have a good prognosis and may not require immunosuppressive therapy. Cirrhosis on initial liver biopsy portends a poor prognosis in all patients with AIH.
authors.affiliation: Department of Medicine and Pathology, University Health Network, University of Toronto, Canada. feldj@niddk.nih.gov
authors.forename: Jordan J
authors.initials: JJ
authors.lastname: Feld
authors.forename: Huong
authors.initials: H
authors.lastname: Dinh
authors.forename: Tamara
authors.initials: T
authors.lastname: Arenovich
authors.forename: Victoria A
authors.initials: VA
authors.lastname: Marcus
authors.forename: Ian R
authors.initials: IR
authors.lastname: Wanless
authors.forename: E Jenny
authors.initials: EJ
authors.lastname: Heathcote
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D015331:Cohort Studies; D018450:Disease Progression; D005260:Female; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008103:Liver Cirrhosis / Q000188:drug therapy / Q000209:etiology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D017063:Outcome Assessment, Health Care; D011379:Prognosis; D016019:Survival Analysis
chemical_list: D007166:Immunosuppressive Agents