PT  - JOURNAL ARTICLE
AU  - Herrick, Ariane L
AU  - Peytrignet, Sebastien
AU  - Lunt, Mark
AU  - Pan, Xiaoyan
AU  - Hesselstrand, Roger
AU  - Mouthon, Luc
AU  - Silman, Alan J
AU  - Dinsdale, Graham
AU  - Brown, Edith
AU  - Czirják, László
AU  - Distler, Jörg H W
AU  - Distler, Oliver
AU  - Fligelstone, Kim
AU  - Gregory, William J
AU  - Ochiel, Rachel
AU  - Vonk, Madelon C
AU  - Ancuţa, Codrina
AU  - Ong, Voon H
AU  - Farge, Dominique
AU  - Hudson, Marie
AU  - Matucci-Cerinic, Marco
AU  - Balbir-Gurman, Alexandra
AU  - Midtvedt, Øyvind
AU  - Jobanputra, Paresh
AU  - Jordan, Alison C
AU  - Stevens, Wendy
AU  - Moinzadeh, Pia
AU  - Hall, Frances C
AU  - Agard, Christian
AU  - Anderson, Marina E
AU  - Diot, Elisabeth
AU  - Madhok, Rajan
AU  - Akil, Mohammed
AU  - Buch, Maya H
AU  - Chung, Lorinda
AU  - Damjanov, Nemanja S
AU  - Gunawardena, Harsha
AU  - Lanyon, Peter
AU  - Ahmad, Yasmeen
AU  - Chakravarty, Kuntal
AU  - Jacobsen, Søren
AU  - MacGregor, Alexander J
AU  - McHugh, Neil
AU  - Müller-Ladner, Ulf
AU  - Riemekasten, Gabriela
AU  - Becker, Michael
AU  - Roddy, Janet
AU  - Carreira, Patricia E
AU  - Fauchais, Anne Laure
AU  - Hachulla, Eric
AU  - Hamilton, Jennifer
AU  - İnanç, Murat
AU  - McLaren, John S
AU  - van Laar, Jacob M
AU  - Pathare, Sanjay
AU  - Proudman, Susanna M
AU  - Rudin, Anna
AU  - Sahhar, Joanne
AU  - Coppere, Brigitte
AU  - Serratrice, Christine
AU  - Sheeran, Tom
AU  - Veale, Douglas J
AU  - Grange, Claire
AU  - Trad, Georges-Selim
AU  - Denton, Christopher P
TI  - Patterns and predictors of skin score change in early diffuse systemic sclerosis from the European Scleroderma Observational Study
AID  - 10.1136/annrheumdis-2017-211912
DP  - 2018 Apr 01
TA  - Annals of the Rheumatic Diseases
PG  - 563--570
VI  - 77
IP  - 4
4099  - http://ard.bmj.com/content/77/4/563.short
4100  - http://ard.bmj.com/content/77/4/563.full
SO  - Ann Rheum Dis2018 Apr 01; 77
AB  - 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.