Pulmonary Arterial Hypertension in Connective Tissue Diseases

Research output: Contribution to journalReview articlepeer-review

34 Scopus citations


Pulmonary arterial hypertension (PAH) may complicate diverse connective tissue diseases (CTDs). Approximately 10% of patients with systemic sclerosis develop PAH, the prevalence being much lower in other CTDs. However, PAH is an important contributor to morbidity and mortality in all forms of CTD. Despite similarities in presentation, hemodynamic perturbations, and pathogenesis, patients with CTD-associated PAH (CTD-PAH) usually have a poorer response to PAH-specific medications and poorer prognosis than patients with idiopathic PAH (IPAH). Select patients with CTD-PAH may be candidates for lung transplantation, but results are less favorable than for IPAH because of comorbidities and complications specifically associated with CTD.

Original languageEnglish (US)
Pages (from-to)413-425
Number of pages13
JournalHeart Failure Clinics
Issue number3
StatePublished - Jul 2012


  • Connective tissue disease
  • Mixed connective tissue disease
  • Pulmonary hypertension
  • Scleroderma
  • Systemic lupus erythematosus
  • Therapy

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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