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.