Is pulmonary arterial hypertension really a late complication of systemic sclerosis?

Chest. 2009 Nov;136(5):1211-1219. doi: 10.1378/chest.08-3042. Epub 2009 May 8.


Background: Pulmonary arterial hypertension (PAH) is a frequent cause of morbidity and mortality in patients with systemic sclerosis (SSc). PAH is generally considered to be a late complication of limited cutaneous SSc. This study identified and investigated a subset of SSc patients with early-onset PAH.

Methods: Clinical and hemodynamic data at the time of diagnosis were collected retrospectively for 78 consecutive patients with PAH associated with SSc. PAH diagnosed within 5 years of the first non-Raynaud phenomenon symptom of SSc was considered to be an early-onset complication. PAH diagnosed > 5 years following SSc diagnosis was considered to be a late complication.

Results: PAH occurred a mean (+/- SD) duration of 6.3 +/- 6.6 years after the diagnosis of SSc (median delay, 4.0 years; 95% CI, 2.88 to 6.0 years). Early-onset PAH was diagnosed in 43 patients (55.1%), and late-onset PAH was diagnosed in 35 patients (44.9%). Patients with early-onset PAH were older at SSc diagnosis than patients with late-onset PAH (mean age, 58.0 +/- 12.5 vs 46.6 +/- 12.9 years, respectively; p = 0.0002). No differences in age at the time of PAH diagnosis, or in SSc subtype (limited vs diffuse; anticentromere vs anti-Scl70 antibodies), were observed between onset subgroups. At diagnosis, early-onset PAH was more severe than late-onset PAH, with a lower cardiac index (2.4 +/- 0.6 vs 2.8 +/- 0.6 L/min/m(2), respectively; p = 0.005) and greater total pulmonary resistance (1,708 +/- 777 vs 1,341 +/- 530 dyne x s x cm(-5)/m(2), respectively; p = 0.02). Mortality at 3 and 5 years was comparable between subgroups.

Conclusions: In contrast to the expected scenario, early-onset PAH occurred in approximately half of SSc patients. Early-onset PAH was as frequent among patients with diffuse SSc as those with limited SSc. Annual screening for PAH should be implemented immediately after SSc diagnosis for all patients.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Age of Onset
  • Aged
  • Body Mass Index
  • Female
  • Follow-Up Studies
  • Hemodynamics
  • Humans
  • Hypertension, Pulmonary / classification
  • Hypertension, Pulmonary / epidemiology*
  • Male
  • Middle Aged
  • Prognosis
  • Pulmonary Fibrosis / epidemiology
  • Retrospective Studies
  • Scleroderma, Systemic / classification
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / mortality
  • Survival Rate
  • Survivors
  • Time Factors