title: Changes in estimated right ventricular systolic pressure predict mortality and pulmonary hypertension in a cohort of scleroderma patients.
abstract: OBJECTIVE
  Annual echocardiography screening is widely used in scleroderma, but the utility of longitudinal assessment is unknown. We evaluated whether change in right ventricular systolic pressure (RVSP) was a risk factor for mortality and development of pulmonary arterial hypertension (PAH) in a cohort of scleroderma patients.
  
  
  METHODS
  The study population consisted of scleroderma patients who had at least three echocardiograms and pulmonary function tests (PFTs) over ≥1 year as part of routine care. The annual rate of change in RVSP was determined for each subject. Cox proportional hazards regression was performed to assess the association between PAH and mortality and change in RVSP/year, adjusted for relevant covariates.
  
  
  RESULTS
  613 scleroderma patients with 3244 echocardiograms were studied. The adjusted relative hazards of PAH and mortality were 1.08 (95% CI 1.05-1.11) and 1.12 (95% CI 1.08-1.15) per 1 mm Hg increase in RVSP/year, respectively. Compared with patients with a stable RVSP, the relative hazards for the development of PAH were 1.90 (95% CI 0.91-3.96), 5.09 (95% CI 2.53-10.26) and 6.15 (95% CI 3.58-10.56) for subjects whose RVSP increased at rates of 1-1.99, 2-2.99 and 3+ mm Hg/year. Compared with the same reference group, the relative hazards for death were 0.92 (95% CI 0.48-1.73), 2.16 (95% CI 1.16-4.01) and 5.05 (95% CI 3.47-7.34) for subjects whose RVSP increased at rates of 1-1.99, 2-2.99 and 3+ mm Hg/year.
  
  
  CONCLUSIONS
  In a population of scleroderma patients, the rate of increase in RVSP is a risk factor for mortality and PAH even after adjustment for clinical characteristics and longitudinal PFT data.
authors.affiliation: Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD 21224, USA. ashah32@jhmi.edu
authors.forename: Ami A
authors.initials: AA
authors.lastname: Shah
authors.forename: Shang-En
authors.initials: SE
authors.lastname: Chung
authors.forename: Fredrick M
authors.initials: FM
authors.lastname: Wigley
authors.forename: Robert A
authors.initials: RA
authors.lastname: Wise
authors.forename: Laura K
authors.initials: LK
authors.lastname: Hummers
mesh_terms: D000328:Adult; D015331:Cohort Studies; D004452:Echocardiography; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000209:etiology*; D008297:Male; D008875:Middle Aged; D016016:Proportional Hazards Models; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications / Q000000981:diagnostic imaging / Q000401:mortality*; D013599:Systole / Q000502:physiology; D017725:Ventricular Pressure / Q000502:physiology*