Idiopathic portal hypertension associated with progressive systemic sclerosis

Herein we describe the clinical course of a case of idiopathic portal hypertension accompanied by splenomegaly, portal hypertension, and pancytopenia in addition to progressive systemic sclerosis. Immunological studies revealed positive antinuclear antibodies, hypergammaglobulinemia with increases in IgG and IgA, a decrease in peripheral T lymphocytes, and a decrease in the rate of lymphocyte blast transformation induced with phytohemagglutinin. The spleen weighed 1070 g, and the portal pressure was 270 nm H2O. Esophageal varices were also noted. These findings suggest the possibility of association of some immunological abnormalities with the etiology of idiopathic portal hypertension.