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Prognostic factors for lung function in systemic sclerosis: Prospective study of 105 cases
European Respiratory Journal , 06/24/09

Gilson M et al. – Study demonstrates that only diffuse systemic sclerosis (SSc) was predictive of a decrease in pulmonary function in this early-SSc cohort, however does not support a causative role of oesophageal involvement.

• Main objectives of this study were:
  • To identify prognostic factors for SSc- interstitial lung disease (ILD), and
  • To clarify the possible causative role of manometric esophageal involvement
• Consecutive SSc-pts underwent pulmonary function tests (PFT) and esophageal manometry
• Inclusion criteria: PFT were repeated >12 mo after baseline
• Primary endpoint: a decrease of ≥10% in forced vital capacity (FVC)
• Secondary endpoints: a decrease of ≥15% in carbon monoxide diffusing capacity (DLCO) and a decrease of ≥20% in FVC

• 105 pts: 45 diffuse SSc, median disease duration 2.0 yrs:
  • 23% had a FVC below 80%
  • 59% had a DLCO below 80%, and
  • 54% had severe esophageal hypomotility at baseline
• Over a period of 72±46 months:
  • 29 pts (28%) displayed a decrease of ≥10% in FVC
  • 39 of 98 pts (40%) displayed DLCO decline, and
  • 19 pts (18%) displayed a decrease of ≥20% in FVC
• Multivariate analysis: diffuse SSc was a significant predictor for a decrease of ≥10% in FVC
• No other predictor of a decrease in pulmonary function was identified

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