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

Journal article

Publication Details

Author(s): Herrick AL, Peytrignet S, Lunt M, Pan X, Hesselstrand R, Mouthon L, Silman AJ, Dinsdale G, Brown E, Czirjak L, Distler J, Distler O, Fligelstone K, Gregory WJ, Ochiel R, Vonk MC, Ancuta C, Ong VH, Farge D, Hudson M, Matucci-Cerinic M, Balbir-Gurman A, Midtvedt O, Jobanputra P, Jordan AC, Stevens W, Moinzadeh P, Hall FC, Agard C, Anderson ME, Diot E, Madhok R, Akil M, Buch MH, Chung L, Damjanov NS, Gunawardena H, Lanyon P, Ahmad Y, Chakravarty K, Jacobsen S, Macgregor AJ, Mchugh N, Mueller-Ladner U, Riemekasten G, Becker M, Roddy J, Carreira PE, Fauchais AL, Hachulla E, Hamilton J, Inanc M, Mclaren JS, Van Laar JM, Pathare S, Proudman SM, Rudin A, Sahhar J, Coppere B, Serratrice C, Sheeran T, Veale DJ, Grange C, Trad GS, Denton CP
Journal: Annals of the Rheumatic Diseases
Publication year: 2018
ISSN: 0003-4967


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).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).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%.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.NCT02339441.

FAU Authors / FAU Editors

Distler, Jörg PD Dr.
Heisenberg-Professur für Molekulare Mechanismen der Organfibrose

External institutions with authors

Assistance Publique-Hôpitaux de Paris (AP-HP)
Cambridge University Hospital
Cannock Chase Hospital
Centre Hospitalier Régional Universitaire de TOURS
Charité - Universitätsmedizin Berlin
Gateshead Health NHS Foundation Trust
Glasgow Royal Infirmary (GRI)
Grigore T. Popa University of Medicine and Pharmacy / Universitatea de Medicină și Farmacie "Grigore T. Popa" (UMF Iași)
Hôpital Edouard Herriot
Hôpital Saint Joseph
Hospices Civils de Lyon (CHU)
Hospital Center University De Limoges Dupuytren
Hospital Universitario 12 de Octubre
Istanbul University / İstanbul Üniversitesi
James Cook University Hospital
Justus-Liebig-Universität Gießen
LILLE 1 University - Science and Technology
Lund University / Lunds universitet
McGill University
Monash University
North Bristol NHS Trust
Nottingham University Hospitals
Oslo University Hospital / Oslo Universitetssykehus
Peter Maddison Rheumatology Centre
Queen's Hospital
Radboud University Nijmegen
Rambam Health Care Campus
Royal National Hospital for Rheumatic Diseases
Royal Perth Hospital
Salford Royal NHS Foundation Trust
Sheffield Teaching Hospitals NHS Foundation Trust
Stanford University
St Vincent's Hospital
St Vincent's University Hospital
The University of Liverpool
Università degli Studi di Firenze / University of Florence
Universität Köln
Universität zu Lübeck
Universität Zürich (UZH)
Université de Nantes
University College London (UCL)
University Hospitals Birmingham NHS Foundation Trust
University Medical Centre Utrecht (UMC Utrecht)
University of Adelaide
University of Belgrade / Универзитет у Београду
University of Copenhagen
University of East Anglia
University of Gothenburg / Göteborgs universitet
University of Leeds
University of Manchester
University of Oxford
University of Paris 5 - René Descartes / Université Paris V René Descartes
University of Paris 7 - Denis Diderot / Université Paris VII Denis Diderot
University of Pécs / Pécsi Tudományegyetem
Whytemans Brae Hospital

How to cite

Herrick, A.L., Peytrignet, S., Lunt, M., Pan, X., Hesselstrand, R., Mouthon, L.,... Denton, C.P. (2018). Patterns and predictors of skin score change in early diffuse systemic sclerosis from the European Scleroderma Observational Study. Annals of the Rheumatic Diseases.

Herrick, Ariane L., et al. "Patterns and predictors of skin score change in early diffuse systemic sclerosis from the European Scleroderma Observational Study." Annals of the Rheumatic Diseases (2018).


Last updated on 2018-20-11 at 20:08