Idiopathic portal hypertension associated with systemic lupus erythematosus

A case of idiopathic portal hypertension (IPH) associated with systemic lup us erythematosus (SLE) is reported in a 38-year-old man who had been diagno sed with SLE and treated for 18 years. Esophageal varices, found in 1994 on endoscopic examination, had been followed up for 2 years. On July 16, 1996 , he was admitted to Nagoya University Hospital because there was a high ri sk of bleeding from the esophageal varices due to severe thrombocytopenia. As partial splenic embolization had temporarily controlled the thrombocytop enia, splenectomy and devascularization of the stomach vessels were perform ed after endoscopic ligation of the esophageal varices. Histological specim ens of wedge biopsied liver showed chronic inactive hepatitis without cirrh osis. The presence of anticardiolipin antibody, indicated by positivity for lupus anticoagulant, was suggestive of the presence of a common immunologi cal mechanism in the etiology of SLE and IPH.