title: Clinical course of lung physiology in patients with scleroderma and interstitial lung disease: analysis of the Scleroderma Lung Study Placebo Group.
abstract: OBJECTIVE
  Patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) are thought to have the greatest decline in lung function (forced vital capacity [FVC]% predicted) in the early years after disease onset. The aim of this study was to assess the natural history of the decline in FVC% predicted in patients receiving placebo in the Scleroderma Lung Study and to evaluate possible factors for cohort enrichment in future therapeutic trials.
  
  
  METHODS
  Patients randomized to receive placebo (n=79) were divided into 3 groups based on the duration of SSc (0-2 years, 2-4 years, and >4 years). Descriptive statistics and a mixed-effects model were used to analyze the rate of decline in the FVC% predicted over a 1-year period. Additional analyses stratified according to the severity of fibrosis on high-resolution computed tomography (HRCT) were performed, and interactions between disease severity and disease duration were explored.
  
  
  RESULTS
  The mean±SD decline in the unadjusted FVC% predicted during the 1-year period was 4.2±12.8%. At baseline, 28.5%, 43.0%, and 28.5% of patients were in the groups with disease durations of 0-2 years, 2-4 years, and >4 years, respectively. The rate of decline in the FVC% predicted was not significantly different across the 3 disease groups (P=0.85). When stratified by baseline fibrosis on HRCT, the rate of decline in the FVC% predicted was statistically significantly greater in the group with severe fibrosis (mean annualized decline in the FVC% predicted 7.2% versus 2.7% in the groups with no or moderate fibrosis; P=0.008). The decline observed in the group with severe fibrosis was most pronounced in those with a relatively short disease duration (0-2 years; annualized decline 7.0%).
  
  
  CONCLUSIONS
  Among patients with SSc-ILD in the Scleroderma Lung Study, the rates of progression of lung disease were similar irrespective of disease duration. The baseline HRCT fibrosis score is a predictor of a future decline in the FVC% predicted in the absence of effective treatment.
authors: University of California, Los Angeles, CA, USA. khannad@med.umich.edu
authors: Dinesh
authors: D
authors: Khanna
authors: Chi-Hong
authors: CH
authors: Tseng
authors: Niloofar
authors: N
authors: Farmani
authors: Virginia
authors: V
authors: Steen
authors: Daniel E
authors: DE
authors: Furst
authors: Philip J
authors: PJ
authors: Clements
authors: Michael D
authors: MD
authors: Roth
authors: Jonathan
authors: J
authors: Goldin
authors: Robert
authors: R
authors: Elashoff
authors: James R
authors: JR
authors: Seibold
authors: Rajeev
authors: R
authors: Saggar
authors: Donald P
authors: DP
authors: Tashkin
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