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 annrheumdis-2017-211912
DO 10.1136/annrheumdis-2017-211912
A1 Ariane L Herrick
A1 Sebastien Peytrignet
A1 Mark Lunt
A1 Xiaoyan Pan
A1 Roger Hesselstrand
A1 Luc Mouthon
A1 Alan J Silman
A1 Graham Dinsdale
A1 Edith Brown
A1 László Czirják
A1 Jörg H W Distler
A1 Oliver Distler
A1 Kim Fligelstone
A1 William J Gregory
A1 Rachel Ochiel
A1 Madelon C Vonk
A1 Codrina Ancuţa
A1 Voon H Ong
A1 Dominique Farge
A1 Marie Hudson
A1 Marco Matucci-Cerinic
A1 Alexandra Balbir-Gurman
A1 Øyvind Midtvedt
A1 Paresh Jobanputra
A1 Alison C Jordan
A1 Wendy Stevens
A1 Pia Moinzadeh
A1 Frances C Hall
A1 Christian Agard
A1 Marina E Anderson
A1 Elisabeth Diot
A1 Rajan Madhok
A1 Mohammed Akil
A1 Maya H Buch
A1 Lorinda Chung
A1 Nemanja S Damjanov
A1 Harsha Gunawardena
A1 Peter Lanyon
A1 Yasmeen Ahmad
A1 Kuntal Chakravarty
A1 Søren Jacobsen
A1 Alexander J MacGregor
A1 Neil McHugh
A1 Ulf Müller-Ladner
A1 Gabriela Riemekasten
A1 Michael Becker
A1 Janet Roddy
A1 Patricia E Carreira
A1 Anne Laure Fauchais
A1 Eric Hachulla
A1 Jennifer Hamilton
A1 Murat İnanç
A1 John S McLaren
A1 Jacob M van Laar
A1 Sanjay Pathare
A1 Susanna M Proudman
A1 Anna Rudin
A1 Joanne Sahhar
A1 Brigitte Coppere
A1 Christine Serratrice
A1 Tom Sheeran
A1 Douglas J Veale
A1 Claire Grange
A1 Georges-Selim Trad
A1 Christopher P Denton
YR 2018
UL http://ard.bmj.com/content/early/2018/01/06/annrheumdis-2017-211912.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.