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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.

Methods

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

Results

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