title: Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome concomitant with systemic sclerosis, immune thrombocytopenic purpura.
abstract: A 58-year-old Japanese woman presented with chronic fluctuating liver dysfunction with purpura. Raynaud's phenomenon had been diagnosed 4 years previously. At the initial examination, skin biopsy showed limited cutaneous systemic sclerosis (SSc). Laboratory investigations revealed liver dysfunction. Anti-nuclear antibodies, anti-mitochondria M2 antibody, anti-thyroglobulin antibody, and platelet-associated IgG were positive. Primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) were diagnosed serologically, clinically and histologically. Immune thrombocytopenic purpura (ITP) was diagnosed by bone marrow puncture, clinical and laboratory findings, and Helicobacter pylori IgG was positive. She was treated with prednisolone 30 mg/day, ursodeoxycholic acid 600 mg/day, and a 7-day course of lansoprazole plus amoxicillin and clarithromycin. Thrombocytes increased rapidly and transaminase improved at day 7. We report a rare case of PBC-AIH overlap syndrome with concurrent ITP and SSc which suggest the presence of shared genetic susceptibility factors in multiple autoimmune conditions including PBC, AIH, ITP and SSc.
authors: Division of Gastroenterology, Kitasato University, Kitasato Medical Center Hospital, Kitamoto.
authors: Megumi
authors: M
authors: Toyoda
authors: Hiroaki
authors: H
authors: Yokomori
authors: Fumihiko
authors: F
authors: Kaneko
authors: Hide
authors: H
authors: Yoshida
authors: Kenta
authors: K
authors: Hoshi
authors: Hajime
authors: H
authors: Takeuchi
authors: Kumiko
authors: K
authors: Tahara
authors: Akihiko
authors: A
authors: Takahashi
authors: Takeo
authors: T
authors: Kudo
authors: Tadashi
authors: T
authors: Motoori
authors: Makoto
authors: M
authors: Ohbu
authors: Hirobumi
authors: H
authors: Kondo
authors: Toshifumi
authors: T
authors: Hibi
mesh_terms: D005260:Female; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000150:complications / Q000175:diagnosis*; D008875:Middle Aged; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000150:complications / Q000175:diagnosis*; D012595:Scleroderma, Systemic / Q000150:complications / Q000175:diagnosis*