title: Age and risk of pulmonary arterial hypertension in scleroderma.
abstract: OBJECTIVE
  To investigate whether age at disease onset is a risk factor for pulmonary arterial hypertension (PAH) in scleroderma.
  
  
  METHODS
  Scleroderma center.
  
  
  METHODS
  Seven hundred nine consecutive scleroderma patients who underwent echocardiography.
  
  
  METHODS
  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 > or =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 > or =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.
authors.affiliation: Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
authors.forename: Lionel
authors.initials: L
authors.lastname: Schachna
authors.forename: Fredrick M
authors.initials: FM
authors.lastname: Wigley
authors.forename: Betty
authors.initials: B
authors.lastname: Chang
authors.forename: Barbara
authors.initials: B
authors.lastname: White
authors.forename: Robert A
authors.initials: RA
authors.lastname: Wise
authors.forename: Allan C
authors.initials: AC
authors.lastname: Gelber
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000375:Aging; D002648:Child; D016001:Confidence Intervals; D004452:Echocardiography; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000145:classification / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications*; D012720:Severity of Illness Index