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与系统性红斑狼疮相关的特发性门静脉高压症。

Idiopathic portal hypertension associated with systemic lupus erythematosus.

作者信息

Inagaki H, Nonami T, Kawagoe T, Miwa T, Hosono J, Kurokawa T, Harada A, Nakao A, Takagi H, Suzuki H, Sakamoto J

机构信息

Department of Surgery, Aichi Prefectural Hospital, Kakemachi, Okazaki, Japan.

出版信息

J Gastroenterol. 2000;35(3):235-9. doi: 10.1007/s005350050336.

DOI:10.1007/s005350050336
PMID:10755694
Abstract

A case of idiopathic portal hypertension (IPH) associated with systemic lupus erythematosus (SLE) is reported in a 38-year-old man who had been diagnosed with SLE and treated for 18 years. Esophageal varices. found in 1994 on endoscopic examination, had been followed up for 2 years. On July 16, 1996, he was admitted to Nagoya University Hospital because there was a high risk of bleeding from the esophageal varices due to severe thrombocytopenia. As partial splenic embolization had temporarily controlled the thrombocytopenia, splenectomy and devascularization of the stomach vessels were performed after endoscopic ligation of the esophageal varices. Histological specimens of wedge biopsied liver showed chronic inactive hepatitis without cirrhosis. The presence of anticardiolipin antibody, indicated by positivity for lupus anticoagulant, was suggestive of the presence of a common immunological mechanism in the etiology of SLE and IPH.

摘要

报告了一例与系统性红斑狼疮(SLE)相关的特发性门静脉高压(IPH)病例,患者为一名38岁男性,已被诊断为SLE并接受治疗18年。1994年内镜检查发现食管静脉曲张,已随访2年。1996年7月16日,他因严重血小板减少导致食管静脉曲张出血风险高而入住名古屋大学医院。由于部分脾栓塞术曾暂时控制血小板减少,在食管静脉曲张内镜结扎术后进行了脾切除术和胃血管离断术。楔形活检肝组织标本显示为慢性非活动性肝炎,无肝硬化。狼疮抗凝物阳性提示抗心磷脂抗体的存在,这表明SLE和IPH的病因中存在共同的免疫机制。

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