RT Journal Article
SR Electronic
T1 Patterns and predictors of skin score change in early diffuse systemic sclerosis from the European Scleroderma Observational Study
JF Annals of the Rheumatic Diseases
JO Ann Rheum Dis
FD BMJ Publishing Group Ltd and European League Against Rheumatism
SP 563
OP 570
DO 10.1136/annrheumdis-2017-211912
VO 77
IS 4
A1 Herrick, Ariane L
A1 Peytrignet, Sebastien
A1 Lunt, Mark
A1 Pan, Xiaoyan
A1 Hesselstrand, Roger
A1 Mouthon, Luc
A1 Silman, Alan J
A1 Dinsdale, Graham
A1 Brown, Edith
A1 Czirják, László
A1 Distler, Jörg H W
A1 Distler, Oliver
A1 Fligelstone, Kim
A1 Gregory, William J
A1 Ochiel, Rachel
A1 Vonk, Madelon C
A1 Ancuţa, Codrina
A1 Ong, Voon H
A1 Farge, Dominique
A1 Hudson, Marie
A1 Matucci-Cerinic, Marco
A1 Balbir-Gurman, Alexandra
A1 Midtvedt, Øyvind
A1 Jobanputra, Paresh
A1 Jordan, Alison C
A1 Stevens, Wendy
A1 Moinzadeh, Pia
A1 Hall, Frances C
A1 Agard, Christian
A1 Anderson, Marina E
A1 Diot, Elisabeth
A1 Madhok, Rajan
A1 Akil, Mohammed
A1 Buch, Maya H
A1 Chung, Lorinda
A1 Damjanov, Nemanja S
A1 Gunawardena, Harsha
A1 Lanyon, Peter
A1 Ahmad, Yasmeen
A1 Chakravarty, Kuntal
A1 Jacobsen, Søren
A1 MacGregor, Alexander J
A1 McHugh, Neil
A1 Müller-Ladner, Ulf
A1 Riemekasten, Gabriela
A1 Becker, Michael
A1 Roddy, Janet
A1 Carreira, Patricia E
A1 Fauchais, Anne Laure
A1 Hachulla, Eric
A1 Hamilton, Jennifer
A1 İnanç, Murat
A1 McLaren, John S
A1 van Laar, Jacob M
A1 Pathare, Sanjay
A1 Proudman, Susanna M
A1 Rudin, Anna
A1 Sahhar, Joanne
A1 Coppere, Brigitte
A1 Serratrice, Christine
A1 Sheeran, Tom
A1 Veale, Douglas J
A1 Grange, Claire
A1 Trad, Georges-Selim
A1 Denton, Christopher P
YR 2018
UL http://ard.bmj.com/content/77/4/563.abstract
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.