Analysis of a case of systemic sclerosis complicated by noncirrhotic portal hypertension and literature review / 中华风湿病学杂志

ABSTRACT

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.