A Case of Diffuse Scleroderma Associated with Primary Biliary Cirrhosis and Isolated Pulmonary Hypertension.
- Author:
Hyun Kyu CHANG
;
Sang Sig CHUNG
;
Haing SUB
;
Sung Hee LEE
;
Hwan HERR
- Publication Type:Case Report
- MeSH:
Arthritis, Rheumatoid;
Esophageal Motility Disorders;
Humans;
Hypertension, Pulmonary*;
Liver Cirrhosis, Biliary*;
Lupus Erythematosus, Systemic;
Scleroderma, Diffuse*;
Sjogren's Syndrome;
Thyroiditis, Autoimmune
- From:The Journal of the Korean Rheumatism Association
1997;4(2):168-172
- CountryRepublic of Korea
- Language:Korean
-
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.
