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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Authors | 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 |
Journal | Internal medicine (Tokyo, Japan)
(Intern Med)
Vol. 48
Issue 23
Pg. 2019-23
( 2009)
ISSN: 1349-7235 [Electronic] Japan |
PMID | 19952485
(Publication Type: Case Reports, Journal Article)
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Topics |
- Female
- Hepatitis, Autoimmune
(complications, diagnosis)
- Humans
- Liver Cirrhosis, Biliary
(complications, diagnosis)
- Middle Aged
- Purpura, Thrombocytopenic, Idiopathic
(complications, diagnosis)
- Scleroderma, Systemic
(complications, diagnosis)
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