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(Chest. 2003;124:2098-2104.)
© 2003 American College of Chest Physicians

Age and Risk of Pulmonary Arterial Hypertension in Scleroderma*

Lionel Schachna, MBBS, FRACP, PhD; Fredrick M. Wigley, MD; Betty Chang, MD, CM, PhD; Barbara White, MD; Robert A. Wise, MD, FCCP and Allan C. Gelber, MD, MPH, PhD

From the Divisions of Rheumatology (Drs. Schachna, Wigley, and Gelber) and Pulmonary and Critical Care Medicine (Drs. Chang and Wise), Johns Hopkins University School of Medicine; and Division of Rheumatology, University of Maryland School of Medicine (Dr. White), Baltimore, MD.

Correspondence to: Lionel Schachna, MBBS, FRACP, PhD, Department of Rheumatology, Austin & Repatriation Medical Centre, Locked Bag 25, Heidelberg, VIC 3084, Australia

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.

Key Words: age • echocardiography • late onset • pulmonary arterial hypertension • scleroderma






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