title: The clinical phenotypes of autoimmune hepatitis: A comprehensive review.
abstract: Autoimmune hepatitis (AIH) fulfills the generally accepted contemporary criteria of an autoimmune liver disease: the presence of autoantibodies and autoreactive T cells, a female gender bias, association with other autoimmune diseases, response to immunosuppressive therapy and strong associations with the major histocompatibility complex HLA loci. It occurs worldwide in both children and adults and is marked by both etiopathogenic and clinical heterogeneity, differing from the other putative autoimmune liver diseases, primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC), albeit occasionally presenting with overlapping features of PBC or PSC. Although diagnostic criteria have been established and validated, there are still major issues to be clarified due to its variability, such as autoantibody-negative AIH, drug-induced AIH, AIH sharing features with PBC or PSC, and post-transplant de novo AIH. In view of the diverse presentations and courses, including classical chronic onset, acute and acute severe onset, cirrhosis and decompensated cirrhosis, individualized management of patients is indicated. Each patient should receive a personalized analysis of the benefits and side effect risks of drugs. Herein we describe a comprehensive review of the clinical phenotypes of AIH underscoring its clinical heterogeneity.
authors.affiliation: State Key Laboratory of Oncogenes and Related Genes, Key Laboratory of Gastroenterology & Hepatology, Ministry of Health, Division of Gastroenterology and Hepatology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai Cancer Institute, Shanghai Institute of Digestive Disease, 145 Shandong Road, Shanghai 200001, China.
authors.forename: Qixia
authors.initials: Q
authors.lastname: Wang
authors.affiliation: State Key Laboratory of Oncogenes and Related Genes, Key Laboratory of Gastroenterology & Hepatology, Ministry of Health, Division of Gastroenterology and Hepatology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai Cancer Institute, Shanghai Institute of Digestive Disease, 145 Shandong Road, Shanghai 200001, China.
authors.forename: Fan
authors.initials: F
authors.lastname: Yang
authors.affiliation: State Key Laboratory of Oncogenes and Related Genes, Key Laboratory of Gastroenterology & Hepatology, Ministry of Health, Division of Gastroenterology and Hepatology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai Cancer Institute, Shanghai Institute of Digestive Disease, 145 Shandong Road, Shanghai 200001, China.
authors.forename: Qi
authors.initials: Q
authors.lastname: Miao
authors.affiliation: Department of Medicine, Dartmouth College, Hanover, NH, USA.
authors.forename: Edward L
authors.initials: EL
authors.lastname: Krawitt
authors.affiliation: Division of Rheumatology, Allergy, and Clinical Immunology, University of California at Davis, Davis, CA, USA.
authors.forename: M Eric
authors.initials: ME
authors.lastname: Gershwin
authors.affiliation: State Key Laboratory of Oncogenes and Related Genes, Key Laboratory of Gastroenterology & Hepatology, Ministry of Health, Division of Gastroenterology and Hepatology, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai Cancer Institute, Shanghai Institute of Digestive Disease, 145 Shandong Road, Shanghai 200001, China. Electronic address: maxiongmd@163.com.
authors.forename: Xiong
authors.initials: X
authors.lastname: Ma
mesh_terms: D000328:Adult; D000893:Anti-Inflammatory Agents / Q000009:adverse effects / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D002648:Child; D005260:Female; D019693:Hepatitis, Autoimmune / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology*; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D008099:Liver / Q000276:immunology / Q000473:pathology; D016031:Liver Transplantation / Q000009:adverse effects; D010641:Phenotype; D012737:Sex Factors
chemical_list: D000893:Anti-Inflammatory Agents; D001323:Autoantibodies; D007166:Immunosuppressive Agents
keywords: Acute hepatitis; Autoantibodies; Autoimmune hepatitis; Chronic hepatitis; Drug-induced liver injury; Jaundice; Liver failure; Liver transplantation