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Age and Risk of Pulmonary Arterial Hypertension in Scleroderma*

      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

      Abbreviations:

      ACA (anticentromere antibody), ACR (American College of Rheumatology), CI (confidence interval), PAH (pulmonary arterial hypertension), OR (odds ratio), PASP (pulmonary artery systolic pressure), RVSP (right ventricular systolic pressure)
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