Association of limited scleroderma and pulmonary hypertension in a patient with primary biliary cirrhosis.
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.
Katsuta
Yasumi
Y
First Department of Internal Medicine, Nippon Medical School, Tokyo, Japan. ykatsuta@nms.ac.jp
Higashi
Haruka
H
Zhang
Xue-Jun
XJ
Kato
Yoshihito
Y
Shimizu
Shuji
S
Komeichi
Hirokazu
H
Ohsuga
Masaru
M
Satomura
Katsuaki
K
Takano
Teruo
T
D000368:Aged; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D008105:Liver Cirrhosis, Biliary / Q000150:complications*; D008297:Male; D045745:Scleroderma, Limited / Q000209:etiology*