J Korean Rheum Assoc.  1997 Jul;4(2):168-172.

A Case of Diffuse Scleroderma Associated with Primary Biliary Cirrhosis and Isolated Pulmonary Hypertension

• Chang HK
• Chung SS
• Sub H
• Lee SH
• Herr H

Abstract

Primary biliary cirrhosis is frequently associated with a variety of disorders presumed to be autoimmune in nature, such as Sjogrens syndrome, scleroderma, rheumatoid arthritis, systemic lupus erythematosus, and autoimmune thyroiditis. Scleroderma has been recognized in association with primary biliary cirrhosis. Most cases present as the CREST (calcinosis cutis, Raynauds phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome. Isolated pulmonary hypertension develops in a small proportion of patients, nearly all of whom have limited cutaneous involvement. We report a case who has diffuse scleroderma associated with primary biliary cirrhosis and isolated pulmonary hypertension.

MeSH Terms

Arthritis, Rheumatoid
Esophageal Motility Disorders
Humans
Hypertension, Pulmonary*
Liver Cirrhosis, Biliary*
Lupus Erythematosus, Systemic
Scleroderma, Diffuse*
Sjogren's Syndrome
Thyroiditis, Autoimmune