title: Autoimmune hepatitis-primary biliary cirrhosis overlap syndrome concomitant with immune hemolytic anemia and immune thrombocytopenic purpura (Evans syndrome).
abstract: Autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) associated with Evans syndrome; combination of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenic purpura (ITP) has rarely been reported. We report the case of a 53-year-old patient who presented with weakness, myalgia, arthralgia, shortness of breath and purpura. Initial laboratory investigations revealed liver dysfunction, anemia and thrombocytopenia. Anti-nuclear (ANA) and antimitochondrial M2 (AMA M2) antibodies were positive. Diagnose of PBC-AIH overlap was made by clinical, serological and histological investigations. AIHA and ITP was identified with clinical-laboratory findings and bone marrow puncture. She was treated with IVIG followed by prednisolone and ursodeoxycholic acid. Hemoglobin-thrombocytes increased rapidly and transaminases improved at day 8. We have reported the first case in the literature with AIH-PBC overlap syndrome concurrent by ITP and AIHA which suggest the presence of shared genetic susceptibility factors in multiple autoimmune conditions including AIH, PBC, ITP and AIHA.
authors.affiliation: Selcuk University, Selcuklu medical faculty, department of internal medicine division of Gastroenterohepatology, Selcuklu, Konya, Turkey. husein68@hotmail.com
authors.forename: Huseyin
authors.initials: H
authors.lastname: Korkmaz
authors.forename: Mehmet Sait
authors.initials: MS
authors.lastname: Bugdaci
authors.forename: Tuncer
authors.initials: T
authors.lastname: Temel
authors.forename: Mehmet
authors.initials: M
authors.lastname: Dagli
authors.forename: Pinar
authors.initials: P
authors.lastname: Karabagli
mesh_terms: D000744:Anemia, Hemolytic, Autoimmune / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy; D000974:Antibodies, Antinuclear / Q000097:blood; D001323:Autoantibodies / Q000097:blood; D002756:Cholagogues and Choleretics / Q000627:therapeutic use; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D019693:Hepatitis, Autoimmune / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008105:Liver Cirrhosis, Biliary / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy; D008875:Middle Aged; D008930:Mitochondria, Liver / Q000276:immunology; D011239:Prednisolone / Q000627:therapeutic use; D013921:Thrombocytopenia / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
chemical_list: D000974:Antibodies, Antinuclear; D001323:Autoantibodies; D002756:Cholagogues and Choleretics; D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D014580:Ursodeoxycholic Acid; D011239:Prednisolone