Patterns and predictors of skin score change in early diffuse systemic sclerosis from the European Scleroderma Observational Study

Ariane L. Herrick, Sebastien Peytrignet, Mark Lunt, Xiaoyan Pan, Roger Hesselstrand, Luc Mouthon, Alan J. Silman, Graham DInsdale, Edith Brown, László Czirják, Jörg H.W. DIstler, Oliver DIstler, Kim Fligelstone, William J. Gregory, Rachel Ochiel, Madelon C. Vonk, Codrina Ancu, Voon H. Ong, Dominique Farge, Marie HudsonMarco Matucci-Cerinic, Alexandra Balbir-Gurman, Øyvind Midtvedt, Paresh Jobanputra, Alison C. Jordan, Wendy Stevens, Pia Moinzadeh, Frances C. Hall, Christian Agard, Marina E. Anderson, Elisabeth DIot, Rajan Madhok, Mohammed Akil, Maya H. Buch, Lorinda Chung, Nemanja S. Damjanov, Harsha Gunawardena, Peter Lanyon, Yasmeen Ahmad, Kuntal Chakravarty, Søren Jacobsen, Alexander J. MacGregor, Neil McHugh, Ulf Müller-Ladner, Gabriela Riemekasten, Michael Becker, Janet Roddy, Patricia E. Carreira, Anne Laure Fauchais, Eric Hachulla, Jennifer Hamilton, Murat Inanç, John S. McLaren, Jacob M. Van Laar, Sanjay Pathare, Susanna M. Proudman, Anna Rudin, Joanne Sahhar, Brigitte Coppere, Christine Serratrice, Tom Sheeran, Douglas J. Veale, Claire Grange, Georges Selim Trad, Christopher P. Denton

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17 Citations (Scopus)

Abstract

Objectives Our aim was to use the opportunity provided by the European Scleroderma Observational Study to (1) identify and describe those patients with early diffuse cutaneous systemic sclerosis (dcSSc) with progressive skin thickness, and (2) derive prediction models for progression over 12 months, to inform future randomised controlled trials (RCTs). Methods The modified Rodnan skin score (mRSS) was recorded every 3 months in 326 patients. 'Progressors' were defined as those experiencing a 5-unit and 25% increase in mRSS score over 12 months (±3 months). Logistic models were fitted to predict progression and, using receiver operating characteristic (ROC) curves, were compared on the basis of the area under curve (AUC), accuracy and positive predictive value (PPV). Results 66 patients (22.5%) progressed, 227 (77.5%) did not (33 could not have their status assessed due to insufficient data). Progressors had shorter disease duration (median 8.1 vs 12.6 months, P=0.001) and lower mRSS (median 19 vs 21 units, P=0.030) than non-progressors. Skin score was highest, and peaked earliest, in the anti-RNA polymerase III (Pol3+) subgroup (n=50). A first predictive model (including mRSS, duration of skin thickening and their interaction) had an accuracy of 60.9%, AUC of 0.666 and PPV of 33.8%. By adding a variable for Pol3 positivity, the model reached an accuracy of 71%, AUC of 0.711 and PPV of 41%. Conclusions Two prediction models for progressive skin thickening were derived, for use both in clinical practice and for cohort enrichment in RCTs. These models will inform recruitment into the many clinical trials of dcSSc projected for the coming years. Trial registration number NCT02339441.

Original languageEnglish
Pages (from-to)563-570
Number of pages8
JournalAnnals of the Rheumatic Diseases
Volume77
Issue number4
DOIs
Publication statusPublished - Apr 2018

Keywords

  • autoantibodies
  • outcomes research
  • systemic sclerosis

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