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Changes in estimated right ventricular systolic pressure predict mortality and pulmonary hypertension in a cohort of scleroderma patients
  1. Ami A Shah1,
  2. Shang-En Chung2,
  3. Fredrick M Wigley1,
  4. Robert A Wise3,
  5. Laura K Hummers1
  1. 1Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
  2. 2Bayview Biostatistics, Epidemiology and Data Management Core, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
  3. 3Division of Pulmonary and Critical Care Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
  1. Correspondence to Dr Ami A Shah, Division of Rheumatology, Johns Hopkins University School of Medicine, 5501 Hopkins Bayview Circle, Room 1B7, Baltimore, MD 21224, USA; ashah32{at}jhmi.edu

Abstract

Objectives 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.

  • Systemic Sclerosis
  • Cardiovascular Disease
  • Epidemiology

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