A case of systemic sclerosis complicated by idiopathic portal hypertension: case report and literature review.
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.
Takagi
Kenji
K
Division of Rheumatology, Department of Internal Medicine (Omori), Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo, 143-8541, Japan. kjtakagi@med.toho-u.ac.jp
Nishio
Shinichiro
S
Akimoto
Kimiko
K
Yoshino
Takumi
T
Kawai
Shinichi
S
D001201:Ascites / Q000209:etiology / Q000628:therapy; D004932:Esophageal and Gastric Varices / Q000209:etiology / Q000601:surgery; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications* / Q000209:etiology*; D008026:Ligation; D008875:Middle Aged; D010996:Pleural Effusion / Q000209:etiology / Q000628:therapy; D011239:Prednisolone / Q000008:administration & dosage*; D011379:Prognosis; D011928:Raynaud Disease / Q000150:complications; D012595:Scleroderma, Systemic / Q000150:complications*
D005938:Glucocorticoids; D011239:Prednisolone