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

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 (2018)

Publication Type: Journal article

Publication year: 2018

Journal

DOI: 10.1136/annrheumdis-2017-211912

Abstract

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.

Authors with CRIS profile

Involved external institutions

University College London (UCL) United Kingdom (GB) Hospices Civils de Lyon (CHU) France (FR) Cannock Chase Hospital United Kingdom (GB) Hôpital Saint Joseph France (FR) Hôpital Edouard Herriot France (FR) Monash University Australia (AU) University of Gothenburg / Göteborgs universitet Sweden (SE) University of Adelaide Australia (AU) James Cook University Hospital United Kingdom (GB) University Medical Centre Utrecht (UMC Utrecht) Netherlands (NL) Whytemans Brae Hospital United Kingdom (GB) Gateshead Health NHS Foundation Trust United Kingdom (GB) LILLE 1 University - Science and Technology France (FR) Hospital Center University De Limoges Dupuytren France (FR) Royal Perth Hospital Australia (AU) Charité - Universitätsmedizin Berlin Germany (DE) Universität zu Lübeck Germany (DE) Justus-Liebig-Universität Gießen Germany (DE) University of Belgrade / Универзитет у Београду Serbia (RS) Stanford University United States (USA) (US) University of Leeds United Kingdom (GB) Sheffield Teaching Hospitals NHS Foundation Trust United Kingdom (GB) Centre Hospitalier Régional Universitaire de Tours France (FR) Cambridge University Hospital United Kingdom (GB) Universität zu Köln Germany (DE) University Hospitals Birmingham NHS Foundation Trust United Kingdom (GB) Oslo University Hospital / Oslo Universitetssykehus Rikshospitalet Norway (NO) Università degli Studi di Firenze / University of Florence Italy (IT) University of Manchester United Kingdom (GB) University of Paris 5 - René Descartes / Université Paris V René Descartes France (FR) Lund University / Lunds universitet Sweden (SE) Assistance Publique-Hôpitaux de Paris (AP-HP) France (FR) St Vincent's University Hospital Ireland (IE) Istanbul University / İstanbul Üniversitesi Turkey (TR) Hospital Universitario 12 de Octubre Spain (ES) Nottingham University Hospitals United Kingdom (GB) North Bristol NHS Trust United Kingdom (GB) Glasgow Royal Infirmary (GRI) United Kingdom (GB) The University of Liverpool United Kingdom (GB) Université de Nantes France (FR) St Vincent's Hospital Australia (AU) Rambam Health Care Campus Israel (IL) McGill University Canada (CA) University of Paris 7 - Denis Diderot / Université Paris VII Denis Diderot France (FR) Grigore T. Popa University of Medicine and Pharmacy / Universitatea de Medicină și Farmacie "Grigore T. Popa" (UMF Iași) Romania (RO) Radboud University Nijmegen Netherlands (NL) Salford Royal NHS Foundation Trust United Kingdom (GB) University of Zurich / Universität Zürich (UZH) Switzerland (CH) University of Pécs / Pécsi Tudományegyetem Hungary (HU) University of Oxford United Kingdom (GB) Royal National Hospital for Rheumatic Diseases United Kingdom (GB) University of East Anglia United Kingdom (GB) University of Copenhagen Denmark (DK) Queen's Hospital United Kingdom (GB) Peter Maddison Rheumatology Centre United Kingdom (GB)

How to cite

APA:

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. https://doi.org/10.1136/annrheumdis-2017-211912

MLA:

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).

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