Simpliﬁed Criteria for the Diagnosis of Autoimmune Hepatitis
´ Elke M. Hennes,1 Mikio Zeniya,2 Albert. J. Czaja,3 Albert Pares,4 George N. Dalekos,5 Edward L. Krawitt,6 7 Gilda Porta,8 Kirsten M. Boberg,9 Harald Hofer,10 Francesco B. Bianchi,11 Minoru Shibata,12 Paulo L. Bittencourt, Christoph Schramm,1 Barbara Eisenmann de Torres,13 Peter R.Galle,13 Ian McFarlane,14 Hans-Peter Dienes,15 Ansgar W. Lohse,1 and the International Autoimmune Hepatitis Group
Diagnosis of autoimmune hepatitis (AIH) may be challenging. However, early diagnosis is important because immunosuppression is life-saving. Diagnostic criteria of the International Autoimmune Hepatitis Group (IAIHG) were complex and purely meant for scientiﬁc purposes. This study of theIAIHG aims to deﬁne simpliﬁed diagnostic criteria for routine clinical practice. Candidate criteria included sex, age, autoantibodies, immunoglobulins, absence of viral hepatitis, and histology. The training set included 250 AIH patients and 193 controls from 11 centers worldwide. Scores were built from variables showing predictive ability in univariate analysis. Diagnostic value of each score wasassessed by the area under the receiver operating characteristic (ROC) curve. The best score was validated using data of an additional 109 AIH patients and 284 controls. This score included autoantibodies, immunoglobulin G, histology, and exclusion of viral hepatitis. The area under the curve for prediction of AIH was 0.946 in the training set and 0.91 in the validation set. Based on the ROC curves,two cutoff points were chosen. The score was found to have 88% sensitivity and 97% speciﬁcity (cutoff >6) and 81% sensitivity and 99% speciﬁcity (cutoff >7) in the validation set. Conclusion: A reliable diagnosis of AIH can be made using a very simple diagnostic score. We propose the diagnosis of probable AIH at a cutoff point greater than 6 points and deﬁnite AIH 7 points or higher. (HEPATOLOGY2008;48:169-176.) ferases. Timely diagnosis and immunosuppressive therapy contain disease activity in almost all affected patients, and various case series have reported near normal or normal life expectancy in patients diagnosed and treated adequately.2-4 Untreated AIH, however, has a 5-year mortality above 50%.3,5 Early diagnosis may be difﬁcult because the clinical picture is heterogeneous andthere is
See Editorial on Page 10
utoimmune hepatitis (AIH) is an inﬂammatory condition of the liver that can affect patients of all ages, sexes, and races.1 The diagnosis needs to be considered in any patient with elevated aminotrans-
Abbreviations: AIH, autoimmune hepatitis; AMA, antimitochondrial antibodies; ANA, antinuclear antibodies; AUC, area under the curve; ELISA,enzyme-linked immunosorbent assay; HCV, hepatitis C virus; IAIHG, International Autoimmune Hepatitis Group; IgG, immunoglobulin G; IgM, immunoglobulin M; LKM, liver-kidney microsomal antibodies; PBC, primary biliary cirrhosis; PSC, primary sclerosing cholangitis; ROC, receiver operating characteristic curve; SLA/LP, soluble liver/liver-pancreas antibodies; SMA, smooth muscle cell antibodies. From the1Department of Medicine, University Medical Centre Hamburg Eppendorf, Germany; 2Department of Gastroenterology, Jikei University Graduate School of Medicine, Tokyo, Japan; 3Division of Gastroenterology/Hepatology, Mayo Clinic, Rochester, MN; 4Liver Unit, Digestive Diseases Institute, Hospital Clınic, Ciberhed, ´ Barcelona, Spain; 5Department of Medicine, Medical School, University of Thessaly, Larissa,Greece; 6University of Vermont College of Medicine, Burlington, VT; 7Portuguese Hospital Salvador, University of Sao Paulo, Brazil; 8Children’s Institute Liver Unit, University of Sao Paulo School of Medicine, Sao Paulo, Brazil; 9Medical ˜ Department, Rikshospitalet, Oslo, Norway; 10Department of Internal Medicine IV, Medical University of Vienna, Vienna, Austria; 11Department of Internal...