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; Teruo Takano

doi:10.1272/jnms.72.230

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, Teruo Takano

Author information

Yasumi Katsuta
First Department of Internal Medicine, Nippon Medical School
Haruka Higashi
First Department of Internal Medicine, Nippon Medical School
Xue-Jun Zhang
First Department of Internal Medicine, Nippon Medical School
Yoshihito Kato
First Department of Internal Medicine, Nippon Medical School
Shuji Shimizu
First Department of Internal Medicine, Nippon Medical School
Hirokazu Komeichi
First Department of Internal Medicine, Nippon Medical School
Masaru Ohsuga
First Department of Internal Medicine, Nippon Medical School
Katsuaki Satomura
First Department of Internal Medicine, Nippon Medical School
Teruo Takano
First Department of Internal Medicine, Nippon Medical School

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

JOURNAL FREE ACCESS

2005 Volume 72 Issue 4 Pages 230-235

DOI https://doi.org/10.1272/jnms.72.230

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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 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.

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