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.