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