Primary Biliary Cirrhosis-Autoimmune Hepatitis Overlap Syndrome Concomitant with Systemic Sclerosis, Immune Thrombocytopenic Purpura

Megumi Toyoda; Hiroaki Yokomori; Fumihiko Kaneko; Hide Yoshida; Kenta Hoshi; Hajime Takeuchi; Kumiko Tahara; Akihiko Takahashi; Takeo Kudo; Tadashi Motoori; Makoto Ohbu; Hirobumi Kondo; Toshifumi Hibi

doi:10.2169/internalmedicine.48.2687

CASE REPORTS

Primary Biliary Cirrhosis-Autoimmune Hepatitis Overlap Syndrome Concomitant with Systemic Sclerosis, Immune Thrombocytopenic Purpura

Megumi Toyoda, Hiroaki Yokomori, Fumihiko Kaneko, Hide Yoshida, Kenta Hoshi, Hajime Takeuchi, Kumiko Tahara, Akihiko Takahashi, Takeo Kudo, Tadashi Motoori, Makoto Ohbu, Hirobumi Kondo, Toshifumi Hibi

Author information

Megumi Toyoda
Division of Gastroenterology, Kitasato University, Kitasato Medical Center Hospital
Hiroaki Yokomori
Division of Gastroenterology, Kitasato University, Kitasato Medical Center Hospital
Fumihiko Kaneko
Division of Gastroenterology, Kitasato University, Kitasato Medical Center Hospital
Hide Yoshida
Division of Rheumatology, Department of Internal Medicine, Kitasato University, Kitasato Medical Center Hospital
Kenta Hoshi
Division of Rheumatology, Department of Internal Medicine, Kitasato University, Kitasato Medical Center Hospital
Hajime Takeuchi
Division of Gastroenterology, Kitasato University, Kitasato Medical Center Hospital
Kumiko Tahara
Division of Gastroenterology, Kitasato University, Kitasato Medical Center Hospital
Akihiko Takahashi
Division of Gastroenterology, Kitasato University, Kitasato Medical Center Hospital
Takeo Kudo
Division of Rheumatology, Department of Internal Medicine, Kitasato University, Kitasato Medical Center Hospital
Tadashi Motoori
Division of Pathology, Kitasato University, Kitasato Medical Center Hospital
Makoto Ohbu
Department of Pathology, School of Allied Health Sciences, Kitasato University
Hirobumi Kondo
Division of Rheumatology, Department of Internal Medicine, Kitasato University, Kitasato Medical Center Hospital
Toshifumi Hibi
Division of Gastroenterology, Department of Internal Medicine, School of Medicine, Keio University

Keywords: primary biliary cirrhosis (PBC), autoimmune, hepatitis (AIH), systemic sclerosis (SSc), immune thrombocytopenic purpura (ITP)

JOURNAL OPEN ACCESS

2009 Volume 48 Issue 23 Pages 2019-2023

DOI https://doi.org/10.2169/internalmedicine.48.2687

Details

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.

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