Idiopathic portal hypertension associated with systemic sclerosis and Sjogren's syndrome

We report a patient with idiopathic portal hypertension (IPH) associated with systemic sclerosis (SSc) and Sjogren's syndrome. A 72-year-old Japanese woman was admitted to our hospital because of Raynaud's phenomenon, sclerodactyly, and dyspnea. The patient had splenomegaly, esophageal varices in the absence of extrahepatic portal obstruction, and cirrhosis of the liver. Immunological studies revealed positive anti-nuclear antibodies and high titers of anti-Scl-70, anti-SS-A, anti-centromere, and anti-mitochondrial M2 antibodies. Histological examinations of the liver biopsy specimen revealed stenosis and loss of small portal veins without findings of primary biliary cirrhosis. The patient was diagnosed as having IPH associated with SSc and Sjogren's syndrome. These observations suggest an immunological role in the pathogenesis of IPH.