CASE REPORT
A case of systemic sclerosis complicated by idiopathic portal hypertension: case report and literature review
Kenji Takagi1, Shinichiro Nishio1, Kimiko Akimoto1, Takumi Yoshino1 and Shinichi Kawai1
| (1) |
Division of Rheumatology, Department of Internal Medicine (Omori), Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo 143-8541, Japan |
Received: 06 February 2006 Accepted: 28 March 2006
Abstract We encountered a 62-year-old woman who had systemic sclerosis (SSc) complicated by idiopathic portal hypertension (IPH). She had a 10-year history of scleroderma and Raynaud's phenomenon. She also had pancytopenia, splenomegaly, and esophageal varices. Treatment with prednisolone and endoscopic variceal ligation resulted in improvement of her symptoms. According to our literature review, the prognosis of patients with SSc complicated by IPH is relatively poor. However, the factors that predict outcome of these patients have not been elucidated.
Key words Esophageal varices - Idiopathic portal hypertension (IPH) - Pulmonary hypertension - Raynaud's phenomenon - Systemic sclerosis (SSc)
