-Report on Experiments and Clinical Cases-

Association of Limited Scleroderma and Pulmonary Hypertension in a Patient with Primary Biliary Cirrhosis

Yasumi Katsuta, Haruka Higashi, Xue-Jun Zhang, Yoshihito Kato, Shuji Shimizu, Hirokazu Komeichi, Masaru Ohsuga, Katsuaki Satomura and Teruo Takano

First Department of Internal Medicine, Nippon Medical School

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 B₂ across the lungs was found, which suggested that thromboxane A₂ (precursor of thromboxane B₂) contributed considerably to a rise in pulmonary vascular resistance leading to PHT.

J Nippon Med Sch 2005; 72: 230-235

Keywords
hepatopulmonary syndrome, intrapulmonary vasodilatation, thromboxane B₂, endothelin-1, nitric oxide

Correspondence to
Yasumi Katsuta, MD, First Department of Internal Medicine, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan
ykatsuta@nms.ac.jp

Received, February 10, 2005
Accepted, February 25, 2005