[Non-cirrhotic portal hypertension associated with autoimmune disease].
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.
Li
X
X
Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing 100730, China.
Gao
W
W
Chen
J
J
Tang
W
W
D000328:Adult; D001327:Autoimmune Diseases / Q000150:complications*; D005260:Female; D006470:Hemorrhage / Q000209:etiology / Q000517:prevention & control; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications*; D008107:Liver Diseases / Q000150:complications* / Q000503:physiopathology; D008111:Liver Function Tests; D008875:Middle Aged