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Predictors of progression in systemic sclerosis patients with interstitial lung disease.

  • Distler, Oliver1
  • Assassi, Shervin2
  • Cottin, Vincent3
  • Cutolo, Maurizio4
  • Danoff, Sonye K5
  • Denton, Christopher P6
  • Distler, Jörg H W7
  • Hoffmann-Vold, Anna-Maria8
  • Johnson, Sindhu R9
  • Müller Ladner, Ulf10
  • Smith, Vanessa11, 12
  • Volkmann, Elizabeth R13
  • Maher, Toby M14, 15
  • 1 Dept of Rheumatology, University Hospital Zurich, Zurich, Switzerland. , (Switzerland)
  • 2 Dept of Rheumatology and Clinical Immunogenetics, McGovern Medical School, University of Texas, Houston, TX, USA.
  • 3 National Reference Center for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon, Claude Bernard University Lyon 1, UMR754, Lyon, France. , (France)
  • 4 Research Laboratory, Clinical Division of Rheumatology, Dept of Internal Medicine DIMI, University of Genoa, IRCSS Polyclinic Hospital San Martino, Genoa, Italy. , (Italy)
  • 5 Division of Pulmonary and Critical Care Medicine, Johns Hopkins Medicine, Baltimore, MD, USA.
  • 6 UCL Centre for Rheumatology and Connective Tissue Diseases, Royal Free Hospital, London, UK.
  • 7 Dept of Internal Medicine 3, University of Erlangen-Nuremberg, Erlangen, Germany [email protected] , (Germany)
  • 8 Dept of Rheumatology, Oslo University Hospital, Oslo, Norway. , (Norway)
  • 9 Toronto Scleroderma Program, Dept of Medicine, Toronto Western and Mount Sinai Hospitals, University of Toronto, Toronto, ON, Canada. , (Canada)
  • 10 Dept of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen, Campus Kerckhoff, Bad Nauheim, Germany. , (Germany)
  • 11 Dept of Rheumatology, Ghent University Hospital, Ghent, Belgium. , (Belgium)
  • 12 Dept of Internal Medicine, Ghent University, Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium. , (Belgium)
  • 13 Dept of Medicine, Division of Rheumatology, University of California, David Geffen School of Medicine, Los Angeles, CA, USA.
  • 14 National Heart and Lung Institute, Imperial College London, London, UK.
  • 15 NIHR Respiratory Clinical Research Facility, Royal Brompton Hospital, London, UK.
Published Article
European Respiratory Journal
European Respiratory Society
Publication Date
May 01, 2020
DOI: 10.1183/13993003.02026-2019
PMID: 32079645


Systemic sclerosis (SSc) is a systemic autoimmune disease affecting multiple organ systems, including the lungs. Interstitial lung disease (ILD) is the leading cause of death in SSc.There are no valid biomarkers to predict the occurrence of SSc-ILD, although auto-antibodies against anti-topoisomerase I and several inflammatory markers are candidate biomarkers that need further evaluation. Chest auscultation, presence of shortness of breath and pulmonary function testing are important diagnostic tools, but lack sensitivity to detect early ILD. Baseline screening with high-resolution computed tomography (HRCT) is therefore necessary to confirm an SSc-ILD diagnosis. Once diagnosed with SSc-ILD, patients' clinical courses are variable and difficult to predict, although certain patient characteristics and biomarkers are associated with disease progression. It is important to monitor patients with SSc-ILD for signs of disease progression, although there is no consensus about which diagnostic tools to use or how often monitoring should occur. In this article, we review methods used to define and predict disease progression in SSc-ILD.There is no valid definition of SSc-ILD disease progression, but we suggest that either a decline in forced vital capacity (FVC) from baseline of ≥10%, or a decline in FVC of 5-9% in association with a decline in diffusing capacity of the lung for carbon monoxide of ≥15% represents progression. An increase in the radiographic extent of ILD on HRCT imaging would also signify progression. A time period of 1-2 years is generally used for this definition, but a decline over a longer time period may also reflect clinically relevant disease progression. Copyright ©ERS 2020.

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