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

Herrick, Ariane L. ; Peytrignet, Sebastien ; Lunt, Mark ; Pan, Xiaoyan ; Hesselstrand, Roger LU ; Mouthon, Luc ; Silman, Alan J. ; DInsdale, Graham ; Brown, Edith and Czirják, László , et al. (2018) In Annals of the Rheumatic Diseases 77(4). p.563-570
Abstract

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... (More)

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.

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organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
autoantibodies, outcomes research, systemic sclerosis
in
Annals of the Rheumatic Diseases
volume
77
issue
4
pages
8 pages
publisher
BMJ Publishing Group
external identifiers
  • pmid:29306872
  • scopus:85044451196
ISSN
0003-4967
DOI
10.1136/annrheumdis-2017-211912
language
English
LU publication?
yes
id
b5af0956-5f33-464b-a969-bead190d3d1b
date added to LUP
2018-05-22 08:40:10
date last changed
2025-01-08 09:54:49
@article{b5af0956-5f33-464b-a969-bead190d3d1b,
  abstract     = {{<p>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.</p>}},
  author       = {{Herrick, Ariane L. and Peytrignet, Sebastien and Lunt, Mark and Pan, Xiaoyan and Hesselstrand, Roger and Mouthon, Luc and Silman, Alan J. and DInsdale, Graham and Brown, Edith and Czirják, László and DIstler, Jörg H.W. and DIstler, Oliver and Fligelstone, Kim and Gregory, William J. and Ochiel, Rachel and Vonk, Madelon C. and Ancu, Codrina and Ong, Voon H. and Farge, Dominique and Hudson, Marie and Matucci-Cerinic, Marco and Balbir-Gurman, Alexandra and Midtvedt, Øyvind and Jobanputra, Paresh and Jordan, Alison C. and Stevens, Wendy and Moinzadeh, Pia and Hall, Frances C. and Agard, Christian and Anderson, Marina E. and DIot, Elisabeth and Madhok, Rajan and Akil, Mohammed and Buch, Maya H. and Chung, Lorinda and Damjanov, Nemanja S. and Gunawardena, Harsha and Lanyon, Peter and Ahmad, Yasmeen and Chakravarty, Kuntal and Jacobsen, Søren and MacGregor, Alexander J. and McHugh, Neil and Müller-Ladner, Ulf and Riemekasten, Gabriela and Becker, Michael and Roddy, Janet and Carreira, Patricia E. and Fauchais, Anne Laure and Hachulla, Eric and Hamilton, Jennifer and Inanç, Murat and McLaren, John S. and Van Laar, Jacob M. and Pathare, Sanjay and Proudman, Susanna M. and Rudin, Anna and Sahhar, Joanne and Coppere, Brigitte and Serratrice, Christine and Sheeran, Tom and Veale, Douglas J. and Grange, Claire and Trad, Georges Selim and Denton, Christopher P.}},
  issn         = {{0003-4967}},
  keywords     = {{autoantibodies; outcomes research; systemic sclerosis}},
  language     = {{eng}},
  month        = {{04}},
  number       = {{4}},
  pages        = {{563--570}},
  publisher    = {{BMJ Publishing Group}},
  series       = {{Annals of the Rheumatic Diseases}},
  title        = {{Patterns and predictors of skin score change in early diffuse systemic sclerosis from the European Scleroderma Observational Study}},
  url          = {{http://dx.doi.org/10.1136/annrheumdis-2017-211912}},
  doi          = {{10.1136/annrheumdis-2017-211912}},
  volume       = {{77}},
  year         = {{2018}},
}