Association of limited scleroderma and pulmonary hypertension in a patient with primary biliary cirrhosis

Abstract

We report a male patient with primary biliary cirrhosis (PBC) who developed limited scleroderma (l-SSc) and pulmonary hypertension (PHT). He had noticed shortness of breath seven months earlier, which slowly progressed before admission. Sclerodactyly and telagiectasia of the fingers and chest wall were found. Chest X-ray and Doppler echocardiography suggested the presence of PHT. Histologic examination of the liver (needle biopsy) revealed stage two PBC, and histologic findings of the skin (obtained from the dorsum of right finger IV) were compatible with l-SSc. Direct measurement of pulmonary arterial pressure revealed PHT with normal capillary wedge pressure during right heart catheterization. A striking increment of plasma thromboxane B 2 across the lungs was found, which suggested that thromboxane A 2 (precursor of thromboxane B 2) contributed considerably to a rise in pulmonary vascular resistance leading to PHT.