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Pulmonary hypertension associated with connective tissue disease
Karen A Fagan1, David B Badesch
1Pulmonary Hypertension Center, University of Colorado Health Sciences Center, Denver, CO, USA.
Progress in Cardiovascular Diseases
|January 15, 2003
Abstract:
Pulmonary arterial hypertension is a life threatening complication of several connective tissue diseases including scleroderma (both diffuse and limited scleroderma, or the CREST syndrome--calcinosis cutis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangectasia), systemic lupus erythomatosis (SLE), mixed connective tissue disease (MCTD), and less commonly, rheumatoid arthritis (RA) and dermatomyositis/polymyositis. This report reviews the occurrence of this complication, potential etiologies, clinical presentation, and treatment options.