Pulmonary hypertension associated with connective tissue disease.
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.
Fagan
Karen A
KA
Pulmonary Hypertension Center, University of Colorado Health Sciences Center, Denver, CO, USA.
Badesch
David B
DB
D000276:Adjuvants, Immunologic / Q000627:therapeutic use; D001172:Arthritis, Rheumatoid / Q000150:complications; D017675:CREST Syndrome / Q000150:complications; D003240:Connective Tissue Diseases / Q000150:complications*; D003882:Dermatomyositis / Q000150:complications; D019332:Endothelin-1 / Q000037:antagonists & inhibitors; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology*; D016040:Lung Transplantation; D008180:Lupus Erythematosus, Systemic / Q000150:complications; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use; D017285:Polymyositis / Q000150:complications; D011453:Prostaglandins / Q000627:therapeutic use; D012594:Scleroderma, Localized / Q000150:complications; D012595:Scleroderma, Systemic / Q000150:complications; D012702:Serotonin Antagonists / Q000627:therapeutic use; D014665:Vasodilator Agents / Q000627:therapeutic use
D000276:Adjuvants, Immunologic; D019332:Endothelin-1; D010726:Phosphodiesterase Inhibitors; D011453:Prostaglandins; D012702:Serotonin Antagonists; D014665:Vasodilator Agents