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[Non-cirrhotic portal hypertension associated with autoimmune disease]
1Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing 100730, China.
Objective:
To summarize the cases of non-cirrhotic portal hypertension associated with autoimmune disease, review the literature and introduce the clinical and pathological features of the syndrome.
Methods:
Six female patients treated, between 1983 and 1998 had no history of hepatitis. Variceal bleeding occurred in 5 patients. One patient received sclerotherapy and operation was performed in 3 patients for variceal bleeding. Four patients were subjected to liver biopsy.
Results:
Liver dysfunction of these patients remained normal and variceal bleeding didn't reccur after therapy. Liver biopsy showed nodular regenerative hyperplasia without fibrous septa.
Conclusions:
Non-cirrhotic portal hypertension associated with autoimmune disease is a rare syndrome, characterized by significant portal hypertension as well as histological evidence that cirrhosis is absent. Prevention and treatment of variceal bleeding is very important.