Clinical characteristics and survival in systemic sclerosis-related pulmonary hypertension associated with interstitial lung disease

Chest. 2011 Oct;140(4):1016-1024. doi: 10.1378/chest.10-2473. Epub 2011 Apr 7.


Background: Pulmonary hypertension (PH) complicating systemic sclerosis (SSc)-related interstitial lung disease (ILD) is usually associated with a poor prognosis. However, data are either lacking or scarce on prognostic factors in this condition. The objectives of this study were to compare the survival of patients with ILD-associated PH (PH-ILD) or pulmonary arterial hypertension (PAH) and to determine whether the severity of PH has prognostic value in SSc-associated PH-ILD.

Methods: Consecutive patients with SSc and PH-ILD (n = 47) or PAH (n = 50) confirmed by right-sided heart catheterization were included in a cross-sectional analysis. PH was classified as mild (mean pulmonary arterial pressure [mPAP] ≤ 35 mm Hg) or moderate to severe (mPAP > 35 mm Hg).

Results: As compared with patients with PAH, subjects with PH-ILD were younger, were more frequently men with a history of smoking, had more frequently diffuse SSc, less frequently anticentromere antibodies, and a lower FVC/diffusing capacity of lung for carbon monoxide (DLCO) ratio. They had a worse prognosis than patients with PAH (3-year survival of 47% vs 71%, respectively; P = .07). Patients with mild PH-ILD had similar poor outcomes when compared with those with moderate to severe PH-ILD. Pericardial effusion (hazard ratio [HR], 2.44; P = .04) and lower DLCO (HR, 0.96; P = .01) were the only independent factors predictive of a poor survival in the PH-ILD group.

Conclusions: Patients with SSc with PH-ILD had a different phenotype and a worse prognosis than those with SSc and PAH. Lower DLCO and presence of pericardial effusion were predictive of a poor outcome in PH-ILD, whereas mPAP seemed to have no prognostic significance.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Antibodies, Antinuclear / blood
  • Blood Pressure / physiology
  • Carbon Monoxide / metabolism
  • Comorbidity
  • Cross-Sectional Studies
  • Familial Primary Pulmonary Hypertension
  • Female
  • Humans
  • Hypertension, Pulmonary / epidemiology
  • Hypertension, Pulmonary / mortality*
  • Hypertension, Pulmonary / physiopathology*
  • Lung / metabolism
  • Lung Diseases, Interstitial / epidemiology
  • Lung Diseases, Interstitial / mortality*
  • Lung Diseases, Interstitial / physiopathology*
  • Male
  • Middle Aged
  • Predictive Value of Tests
  • Prognosis
  • Scleroderma, Systemic / epidemiology
  • Scleroderma, Systemic / mortality*
  • Scleroderma, Systemic / physiopathology*
  • Severity of Illness Index
  • Survival Rate


  • Antibodies, Antinuclear
  • anticentromere antibody
  • Carbon Monoxide