Pulmonary hypertension associated with connective tissue disease

Prog Cardiovasc Dis. Nov-Dec 2002;45(3):225-34. doi: 10.1053/pcad.2002.129975.

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.

Publication types

  • Review

MeSH terms

  • Adjuvants, Immunologic / therapeutic use
  • Arthritis, Rheumatoid / complications
  • CREST Syndrome / complications
  • Connective Tissue Diseases / complications*
  • Dermatomyositis / complications
  • Endothelin-1 / antagonists & inhibitors
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / drug therapy*
  • Hypertension, Pulmonary / etiology*
  • Lung Transplantation
  • Lupus Erythematosus, Systemic / complications
  • Phosphodiesterase Inhibitors / therapeutic use
  • Polymyositis / complications
  • Prostaglandins / therapeutic use
  • Scleroderma, Localized / complications
  • Scleroderma, Systemic / complications
  • Serotonin Antagonists / therapeutic use
  • Vasodilator Agents / therapeutic use

Substances

  • Adjuvants, Immunologic
  • Endothelin-1
  • Phosphodiesterase Inhibitors
  • Prostaglandins
  • Serotonin Antagonists
  • Vasodilator Agents