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Scleroderma lung study (SLS): differences in the presentation and course of patients with limited versus diffuse systemic sclerosis
  1. Philip J Clements1,
  2. Michael D Roth2,
  3. Robert Elashoff3,
  4. Donald P Tashkin2,
  5. Jonathan Goldin4,
  6. Richard M Silver5,
  7. Mildred Sterz3,
  8. James R Seibold6,
  9. Dean Schraufnagel7,
  10. Robert W Simms8,
  11. Marcy Bolster9,
  12. Robert A Wise10,
  13. Virginia Steen11,12,
  14. M D Mayes,
  15. Kari Connelly13,
  16. Mark Metersky14,
  17. Daniel E Furst1
  1. 1
    Divisions of Rheumatology, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA
  2. 2
    Pulmonary and Critical Care Medicine, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA
  3. 3
    Biomathematics, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA
  4. 4
    Radiology, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA
  5. 5
    Division of Rheumatology, Medical University of South Carolina, Charleston, SC, USA
  6. 6
    Univeristy of Michigan Scleroderma Program, Ann Arbor, MI, USA
  7. 7
    Division of Pulmonology and Critical Care Medicine, University of Illinois, Chicago, IL, USA
  8. 8
    Division of Rheumatology, Boston University, Boston, MA, USA
  9. 9
    Division of Rheumatology and Immunology, Medical University of South Carolina, Charleston, SC, USA
  10. 10
    Division of Pulmonology, The Johns Hopkins University, Baltimore, MD, USA
  11. 11
    Division of Rheumatology, Georgetown University Medical Center, Washington, DC, USA
  12. 12
    Division of Rheumatology and Clinical Immunogenetics, University of Texas Houston Health Science Center, Houston, TX, USA
  13. 13
    Division of Dermatology, UCSF, San Francisco, CA, USA
  14. 14
    Division of Pulmonology, University of Connecticut Health Center, Farmington, CT, USA
  1. P J Clements, Rm 32–59, 1000 Veteran Ave, Los Angeles, CA 90095-1670, USA; pclements{at}mednet.ucla.edu

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

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