Diagnosis and management of pulmonary hypertension in systemic sclerosis

Nadera J. Sweiss, Linda Hushaw, Thenappan Thenappan, Ray Sawaqed, Roberto F. MacHado, Amit R. Patel, Mardi Gomberg-Maitland, Aliya N. Husain, Stephen L. Archer

Research output: Contribution to journalReview articlepeer-review

29 Scopus citations


Patients with systemic sclerosis (SSc) can develop pulmonary hypertension (PH; mean pulmonary artery pressure ≥ 25 mm Hg) caused by pulmonary arterial hypertension (PAH), left ventricular disease, or pulmonary fibrosis. PAH is a pulmonary vascular disease, the diagnosis of which requires pulmonary capillary wedge pressure less than 15 mm Hg, pulmonary vascular resistance greater than 3 Wood Units, and exclusion of thromboembolism and parenchymal lung disease. Molecular mechanisms underlying PAH-SSc include activation of inflammatory and fibrogenic pathways in the vasculature and right ventricle. Circulating autoantibodies trigger endothelial damage and fibroblast activation. PAH most commonly occurs as a late complication in patients with limited cutaneous disease and anticentromere antibodies. Although echocardiography is a useful screening tool, heart catheterization is required to diagnose PAH before initiating therapy. Prognosis and therapeutic response are worse in PAH-SSc than in other PAH categories (median survival, 1-3 y). Approved therapies include prostacyclins, endothelin antagonists, and phosphodiesterase type 5 inhibitors. Research is needed to define disease mechanisms and develop effective therapies.

Original languageEnglish (US)
Pages (from-to)8-18
Number of pages11
JournalCurrent rheumatology reports
Issue number1
StatePublished - Feb 2010


  • Circulating autoantibodies
  • Connective tissue disease
  • Endothelin receptor antagonists
  • Flolan (epoprostenol)
  • Phosphodiesterase 5 inhibitors
  • Pulmonary arterial hypertension


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