系统性硬化症合并非肝硬化性门静脉高压症一例并文献复习

目的 提高风湿科医生对非肝硬化性门静脉高压症的认识.方法 报告1例系统性硬化症合并非肝硬化性门静脉高压症的认识并复习相关文献.结果 患者为51岁女性,病程较长,以门静脉高压为最突出的临床表现,有脾大,全血细胞减少,门静脉重度曲张.肝脏的生化、病毒学以及形态学指标均正常.腹部增强CT未见门静脉血栓形成.最后,诊断为系统性硬化症合并非肝硬化性门静脉高压症.给予小剂量激素及免疫抑制剂治疗,病情稳定.结论 非肝硬化性门静脉高压症是一种少见病,临床上最突出的表现是门静脉高压,该病与风湿性疾病尤其是系统性硬化症合并鲜有报道,风湿科医生应予以重视.

Analysis of a case of systemic sclerosis complicated by noncirrhotic portal hypertension and literature review

Objective To improve the rheumatologists' understanding of noncirrhotic portal hypertension.MethodsA case of systemic sclerosis complicated by noncirrhotic portal hypertension was reported and the related literatures were reviewed.Results A 51-year-old female who had been diagnosed as systemic sclerosis presented clinically with an chronic onset of portal hypertension.She also had pancytopenia,splenomegaly,and significant esophageal varices.Liver function tests were normal.Hepatitis viral serology was negative.Ultrasound scan of liver revealed no focal lesion.ACT scan confirmed the absence of portal vein thrombosis.Taking into account the above evaluation we concluded that the patient had systemic sclerosis and noncirrhotic portal hypertension.The patient was on prednisolone and immunosuppressant and the condition was well.Conclusion Noncirrhotic portal hypertension complicated by autoimmune disease,especially SSC,is very poor,characteriged by significant portal hypertension as well as histological evidence that cirrhosis is absent.Rheumatilogist should pay attention to it.