Changes in Pulmonary Exercise Haemodynamics in Scleroderma: A 4-year Prospective Study

Eur Respir J. 2017 Jul 13;50(1):1601708. doi: 10.1183/13993003.01708-2016. Print 2017 Jul.


Pulmonary arterial hypertension (PAH) is a feared complication of systemic sclerosis. In this prospective cohort study, we monitored the changes in resting and exercise pulmonary haemodynamics of scleroderma patients without initial PAH over a mean follow-up period of ∼4 years.All patients underwent exercise echocardiography and cardiopulmonary exercise testing at baseline and follow-up. A subgroup underwent exercise right heart catheter (RHC) investigations. The primary end-point was the echocardiographic systolic pulmonary arterial pressure at 50 W exercise (sPAP50).We included 99 patients, of whom 58 had a complete dataset. Three out of 99 patients developed RHC-confirmed PAH (0.75 cases per 100 patient-years). sPAP50 increased (p<0.001) and peak oxygen uptake (secondary end-point) decreased significantly (p=0.001) during follow-up, but there was no significant change in resting sPAP (p=0.38). In the RHC subgroup (n=28), mean (m)PAP and pulmonary vascular resistance at 50 W increased significantly (p=0.02 and p=0.002, respectively), but resting mPAP was unchanged.Scleroderma patients without PAH develop a mild but significant deterioration of pulmonary exercise haemodynamics and exercise capacity over a 4-year follow-up period, indicating a progression of pulmonary vascular disease. The manifestation rate of RHC-confirmed PAH was 0.75 cases per 100 patient-years.

Trial registration: NCT01468792.

Publication types

  • Observational Study

MeSH terms

  • Adult
  • Austria
  • Blood Pressure
  • Cardiac Output
  • Echocardiography
  • Exercise Test
  • Exercise*
  • Female
  • Humans
  • Hypertension, Pulmonary / physiopathology*
  • Male
  • Middle Aged
  • Prospective Studies
  • Pulmonary Artery / physiopathology*
  • Rest
  • Scleroderma, Systemic / complications*
  • Vascular Resistance*

Associated data