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Abstract
Objectives: Pulmonary fibrosis is a leading cause of death in systemic sclerosis (SSc). This report examines the differences at baseline and over 12 months between patients with limited versus diffuse cutaneous SSc who participated in the Scleroderma Lung Study.
Methods: SSc patients (64 limited; 94 diffuse) exhibiting dyspnoea on exertion, restrictive pulmonary function and evidence of alveolitis on bronchoalveolar lavage and/or high-resolution computed tomography (HRCT) were randomised to receive cyclophosphamide (CYC) or placebo and serially evaluated over 12 months.
Results: Baseline measures of alveolitis, dyspnoea and pulmonary function were similar in limited and diffuse SSc. However, differences were noted with respect to HRCT-scored fibrosis (worse in limited SSc), and to functional activity, quality of life, skin and musculoskeletal manifestations (worse in diffuse SSc) (p<0.05). When adjusted for the baseline level of fibrosis, both groups responded similarly to CYC with regard to lung function and dyspnoea (p<0.05). Cyclophosphamide was also associated with more improvement in skin score in the diffuse disease group more than in the limited disease group (p<0.05).
Conclusions: After adjusting for the severity of fibrosis at baseline, CYC slowed the decline of lung volumes and improved dyspnoea equally in the limited and the diffuse SSc groups. On the other hand, diffuse SSc patients responded better than limited patients with respect to improvements in skin thickening.
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Footnotes
The Scleroderma Lung Study Research Group (listed by participating site or Committee) University of California Los Angeles, Los Angeles, CA *(UO1 HL 60587 & UO1 HL 60606 for DCC): Philip J. Clements, MD, MPH; Donald P. Tashkin, MD; Robert Elashoff, PhD; Jonathan Goldin, MD, PhD; Michael Roth, MD; Daniel Furst, MD; Ken Bulpitt, MD; Dinesh Khanna, MD; Wen-Ling Joanie Chung, MPH; Sherrie Viasco, RN; Mildred Sterz, RN, MPH; Lovlette Woolcock; Xiaohong Yan, MS; Judy Ho, Sarinnapha Vasunilashorn; Irene da Costa
University of Medicine & Dentistry of New Jersey, New Brunswick, NJ *(UO1 HL 60550): James R. Seibold, MD*; David J. Riley, MD; Judith K. Amorosa, MD; Vivien M. Hsu, MD; Deborah A. McCloskey, BSN; Julianne E. Wilson, RN; *Current address: University of Michigan Scleroderma Program, Ann Arbor, MI
University of Illinois Chicago, Chicago, Illinois *(UO1 HL 60895): John Varga, MD; Dean Schraugnagel, MD; Andrew Wilbur, MD; David Lapota, MD; Shiva Arami, MD; Patricia Cole-Saffold, MS
Boston University, Boston, MA *(UO1 HL 60682): Robert Simms, MD; Arthur Theodore, MD; Peter Clarke, MD; Joseph Korn, MD; Kimberley Tobin, Melynn Nuite BSN
Medical University of South Carolina, Charleston, SC *(UO1 HL 60750): Richard Silver, MD; Marcie Bolster, MD; Charlie Strange, MD; Steve Schabel, MD; Edwin Smith, MD; June Arnold; Katie Caldwell; Michael Bonner
Johns Hopkins School of Medicine, Baltimore, MD *(UO1 HL 60597): Robert Wise, MD; Fred Wigley, MD; Barbara White, MD; Laura Hummers, MD; Mark Bohlman, MD; Albert Polito, MD; Gwen Leatherman, MSN; Edrick Forbes, RN; Marie Daniel
Georgetown University, Washington, DC *(UO1 HL 60794): Virginia Steen, MD; Charles Read, MD; Cirrelda Cooper, MD; Sean Wheaton, MD; Anise Carey; Adriana Ortiz
University of Texas Houston, Houston, TX *(UO1 HL 60839): Maureen Mayes, MD, MPH; Ed Parsley, DO; Sandra Oldham, MD; Tan Filemon, MD; Samantha Jordan, RN; Marilyn Perry
University of California San Francisco, San Francisco, CA *(UO1 HL 60587): Kari Connolly, MD; Jeffrey Golden, MD; Paul Wolters, MD; Richard Webb, MD; John Davis, MD; Christine Antolos; Carla Maynetto
University of Connecticut Health Center, Farmington, CT *(UO1 HL 60587): Naomi Rothfield, MD; Mark Metersky, MD; Richard Cobb, MD; Macha Aberles, MD; Fran Ingenito, RN; Elena Breen
Wayne State University, Detroit, MI (UO1 HL 60839): Maureen Mayes, MD, MPH*; Kamal Mubarak, MD; Jose L Granda, MD; Joseph Silva, MD; Zora Injic, RN, MS; Ronika Alexander, RN. *Present address: University of Texas Houston, Houston, TX
Virginia Mason Research Center, Seattle, WA *(UO1 HL 60823): Daniel Furst, MD *; Steven Springmeyer, MD; Steven Kirkland, MD; Jerry Molitor, MD; Richard Hinke, MD; Amanda Mondt, RN *Present address: University of California at Los Angeles, CA
University of Alabama, Birmingham, AL *(UO1 HL 60748): Mitchell Olman, MD; Barri Fessler, MD; Colleen Sanders, MD; Louis Heck, MD; Tina Parkhill (all centers are supported by a grant from the NHLBI)
Data Safety and Monitoring Board members: Taylor Thompson, MD (Harvard Medical School, Boston, MA); Sharon Rounds, MD (VA Medical Center, Brown University, Providence, RI); Michael Weisman, MD (Cedars Sinai/UCLA, Los Angeles, CA); Bruce Thompson, PhD (Clinical Trials Surveys, Baltimore, MD)
Mortality and Morbidity Review Committee members: Harold Paulus, MD (UCLA); Steven Levy, MD (UCLA); Donald Martin, MD (Johns Hopkins University).
Competing interests: None of the investigators has any financial relationship with Bristol-Myers Squibb.
- Abbreviations:
- BAL
- bronchoalveolar lavage
- CYC
- cyclophosphamide
- dcSSC
- diffuse cutaneous systemic sclerosis
- DLCO
- single-breath diffusing capacity of the lung for carbon monoxide
- FEV1
- forced expiratory volume in the first second
- FVC
- forced vital capacity
- GEE
- generalised estimating equation
- GGO
- ground glass opacification
- HAQ-DI
- Health Assessment Questionnaire disability index
- HRCT
- high-resolution computed tomography
- HRQoL
- health-related quality of life
- lcSSC
- limited cutaneous systemic sclerosis
- PGA
- patient global assessment
- RV
- residual volume
- SD
- standard deviation
- SF-36
- 36-Item Short Form Health
- SHAQ
- Scleroderma Health Assessment Questionnaire
- SLS
- Scleroderma Lung Study
- SSc
- systemic sclerosis
- TDI
- transitional dyspnoea index
- TLC
- total lung capacity
- VAS
- visual analogue scales