Age and Risk of Pulmonary Arterial Hypertension in Scleroderma

Lionel Schachna, Fredrick M. Wigley, Betty Chang, Barbara White, Robert A. Wise, Allan C. Gelber

Research output: Contribution to journalArticlepeer-review

98 Scopus citations


Study objectives: To investigate whether age at disease onset is a risk factor for pulmonary arterial hypertension (PAH) in scleroderma. Setting: Scleroderma center. Patients: Seven hundred nine consecutive scleroderma patients who underwent echocardiography. Measurements: The risk of PAH associated with age at disease onset was modeled as both a continuous and categorical variable. Risk estimates were adjusted for sex, race, scleroderma subtype, disease duration, smoking status, FVC, anticentromere and antitopoisomerase I antibody status. Results: Overall, 274 patients (38.6%), 272 patients by Doppler echocardiography and 2 patients by M-mode echocardiography, had PAH at baseline or during follow-up. There were 114 patients with mild PAH (right ventricular systolic pressure [RVSP], 36 to 45 mm Hg), 66 patients with moderate PAH (RVSP, 46 to 55 mm Hg), and 92 patients with severe PAH (RVSP ≥ 56 mm Hg). A 52% increase in risk of PAH was demonstrated for every 10 years of age at disease onset (odds ratio [OR], 1.52; 95% confidence interval [CI], 1.31 to 1.76). In addition, there was a twofold greater risk of PAH (OR, 2.30; 95% CI, 1.32 to 3.99) for late-onset (age ≥ 60 years) vs earlier-onset (< 60 years) disease. These associations remained evident and were somewhat strengthened when the analyses were restricted to patients with moderate and severe PAH. Conclusions: We identified increasing age at scleroderma onset as a risk factor for PAH. Vigilance among these high-risk patients may provide an opportunity to intervene prior to development of irreversible pulmonary vascular disease.

Original languageEnglish (US)
Pages (from-to)2098-2104
Number of pages7
Issue number6
StatePublished - Dec 2003


  • Age
  • Echocardiography
  • Late onset
  • Pulmonary arterial hypertension
  • Scleroderma

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine
  • Cardiology and Cardiovascular Medicine


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