Interstitial Lung Disease in Systemic Sclerosis

To construct a readily applicable prognostic algorithm in SSc-ILD, integrating PFTs and HRCT.

The prognostic value of baseline PFT and HRCT variables was quantified in patients with SSc-ILD (n = 215) against survival and serial PFT data. MEASUREMENTS AND MAIN RESULTS: Increasingly extensive disease on HRCT was a powerful predictor of mortality (P or= 70%) or extensive disease (severe disease on HRCT or, in indeterminate cases, FVC 20%, respectively, and the use of an FVC threshold of 70% in indeterminate cases. The staging system was predictive of mortality for all scorers, with prognostic separation higher for practitioners (HR, 3.39-3.82) than trainees (HR, 1.87-2.60).