一例系统性硬化症合并特发性门静脉高压症：病例报告及文献复习

A case of systemic sclerosis complicated by idiopathic portal hypertension: case report and literature review.

作者信息

Takagi Kenji, Nishio Shinichiro, Akimoto Kimiko, Yoshino Takumi, Kawai Shinichi

机构信息

Division of Rheumatology, Department of Internal Medicine (Omori), Toho University School of Medicine, 6-11-1 Omori-Nishi, Ota-ku, Tokyo, 143-8541, Japan.

出版信息

Mod Rheumatol. 2006;16(3):183-7. doi: 10.1007/s10165-006-0479-1.

DOI:10.1007/s10165-006-0479-1

PMID:16767559

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.

摘要

我们遇到一位62岁患有系统性硬化症（SSc）并伴有特发性门静脉高压（IPH）的女性。她有10年的硬皮病和雷诺现象病史。她还伴有全血细胞减少、脾肿大和食管静脉曲张。泼尼松龙治疗和内镜下静脉曲张结扎术使她的症状得到改善。根据我们的文献综述，SSc合并IPH患者的预后相对较差。然而，预测这些患者预后的因素尚未阐明。