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CONCISE REPORT
Transitions to different patterns of interstitial lung disease in scleroderma with and without treatment
  1. Hyun J Kim1,2,
  2. Donald P Tashkin3,
  3. David W Gjertson1,2,
  4. Matthew S Brown1,
  5. Eric Kleerup3,
  6. Semin Chong4,
  7. John A Belperio3,
  8. Michael D Roth3,
  9. Fereidoun Abtin1,
  10. Robert Elashoff2,5,
  11. Chi-Hong Tseng5,
  12. Dinesh Khanna6,
  13. Jonathan G Goldin1
  1. 1Department of Radiological Science, Center for Computer Vision Imaging Biomarker, David Geffen School of Medicine at UCLA, Los Angeles, California, USA
  2. 2Department of Biostatistics, Fielding School of Public Health at UCLA, Los Angeles, California, USA
  3. 3Division of Pulmonary and Critical Care Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California, USA
  4. 4Department of Radiology, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul, Korea
  5. 5Department of Biomathematics, David Geffen School of Medicine at UCLA, Los Angeles, California, USA
  6. 6Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, USA
  1. Correspondence to Professor Hyun J Kim, Department of Radiological Science, Center for Computer Vision Imaging Biomarker, David Geffen School of Medicine at UCLA, Los Angeles CA 90024, USA; gracekim{at}mednet.ucla.edu

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.

  • Systemic Sclerosis
  • Pulmonary Fibrosis
  • Outcomes research
  • Treatment

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