Transitions to different patterns of interstitial lung disease in scleroderma with and without treatment

Ann Rheum Dis. 2016 Jul;75(7):1367-71. doi: 10.1136/annrheumdis-2015-208929. Epub 2016 Jan 12.

Abstract

Objectives: The aim is to investigate whether the 12-month quantitative changes in high-resolution CT (HRCT) measures of interstitial lung disease (ILD) are different, and to understand how they change, in patients with scleroderma-related ILD who receive drug therapy versus placebo.

Methods: HRCT images were acquired at baseline and at 12 months in 83 participants in Scleroderma Lung Study I, a clinical trial comparing treatment with oral cyclophosphamide versus placebo. A computer-aided model was used to quantify the extent of fibrotic reticulation, ground glass and honeycomb patterns and quantitative ILD (QILD: sum of these patterns) in the whole lung and the lung zone (upper, middle or lower) of maximal disease involvement.

Results: Mean QILD score decreased by 3.9% in the cyclophosphamide group while increasing by 4.2% in the placebo group in the most severe zone (p=0.01) and decreased by 3.2% in the cyclophosphamide group while increasing by 2.2% in the placebo group in the whole lung (p=0.03). Transitional probabilities demonstrated greater changes from a fibrotic to either a ground glass or normal pattern in the cyclophosphamide group and the reverse in the placebo group.

Conclusions: Changes in quantitative HRCT measures of ILD provide a sensitive indication of disease progression and response to treatment.

Trial registration number: NCT00004563; Post-results.

Keywords: Outcomes research; Pulmonary Fibrosis; Systemic Sclerosis; Treatment.

Publication types

  • Randomized Controlled Trial

MeSH terms

  • Adult
  • Antirheumatic Agents / therapeutic use*
  • Cyclophosphamide / therapeutic use*
  • Disease Progression
  • Female
  • Humans
  • Lung / pathology
  • Lung Diseases, Interstitial / etiology
  • Lung Diseases, Interstitial / pathology*
  • Male
  • Scleroderma, Systemic / complications
  • Scleroderma, Systemic / drug therapy*
  • Scleroderma, Systemic / pathology
  • Treatment Outcome

Substances

  • Antirheumatic Agents
  • Cyclophosphamide

Associated data

  • ClinicalTrials.gov/NCT00004563