Age and Risk of Pulmonary Arterial Hypertension in Scleroderma

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

Research output: Contribution to journalArticle

Abstract

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
JournalChest
Volume124
Issue number6
DOIs
StatePublished - Dec 2003

Fingerprint

Pulmonary Hypertension
Age of Onset
Ventricular Pressure
Blood Pressure
Echocardiography
Odds Ratio
Confidence Intervals
Doppler Echocardiography
Vascular Diseases
Lung Diseases
Smoking
Antibodies

Keywords

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

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Age and Risk of Pulmonary Arterial Hypertension in Scleroderma. / Schachna, Lionel; Wigley, Fredrick; Chang, Betty; White, Barbara; Wise, Robert A; Gelber, Allan.

In: Chest, Vol. 124, No. 6, 12.2003, p. 2098-2104.

Research output: Contribution to journalArticle

Schachna, Lionel ; Wigley, Fredrick ; Chang, Betty ; White, Barbara ; Wise, Robert A ; Gelber, Allan. / Age and Risk of Pulmonary Arterial Hypertension in Scleroderma. In: Chest. 2003 ; Vol. 124, No. 6. pp. 2098-2104.
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