Patterns and predictors of skin score change in early diffuse systemic sclerosis from the European Scleroderma Observational Study.
Ariane L. Herrick
(1)
,
Sebastien Peytrignet
(1)
,
Mark Lunt
(1)
,
Xiaoyan Pan
(1)
,
Roger Hesselstrand
(2)
,
Luc Mouthon
(3)
,
Alan Silman
(4)
,
Graham Dinsdale
(1)
,
Edith Brown
(1)
,
Laszlo Czirjak
(5)
,
Jorg Distler
(6)
,
Oliver Distler
(7)
,
Kim Fligelstone
(8)
,
Rachel Ochiel
(8)
,
Madelon Vonk
(9)
,
Codrina Ancuta
(10)
,
Voon H. Ong
,
Dominique Farge
(11)
,
Marie Hudson
(12)
,
Marco Matucci-Cerinic
(13)
,
Alexandra Balbir-Gurman
(14)
,
Oyvind Midtvedt
(15)
,
Paresh Jobanputra
,
Alison C. Jordan
,
Wendy Stevens
(16)
,
Pia Moinzadeh
(17)
,
Frances C. Hall
,
Christian Agard
(18)
,
Marina E. Anderson
(19)
,
Elisabeth Diot
(20)
,
Rajan Madhok
(21)
,
Mohammed Akil
,
Maya H. Buch
(22)
,
Lorinda Chung
,
Nemanja S. Damjanov
(23)
,
Harsha Gunawardena
(24)
,
Peter Lanyon
,
Yasmeen Ahmad
,
Kuntal Chakravarty
,
Soren Jacobsen
(25)
,
Alexander J. Macgregor
(26)
,
Neil Mchugh
(27)
,
Ulf Muller-Ladner
(28)
,
Gabriela Riemekasten
(29)
,
Michael Becker
(30)
,
Janet Roddy
(31)
,
Patricia Carreira
(32)
,
Anne-Laure Fauchais
(33)
,
Eric Hachulla
(34)
,
Jennifer Hamilton
,
Murat Inanc
(35)
,
John S. Mclaren
,
J. M. van Laar
(36)
,
Sanjay Pathare
,
Susanna Proudman
(37)
,
Anna Rudin
(38)
,
Joanne Sahhar
(39)
,
Brigitte Coppere
(40)
,
Christine Serratrice
(41)
,
Tom Sheeran
,
Douglas J. Veale
(16)
,
Claire Grange
(42)
,
Georges-Selim Trad
(43)
,
Christopher Denton
(44)
1
University of Manchester [Manchester]
2 Skane University Hospital [Lund]
3 Hôpital Cochin [AP-HP]
4 University of Oxford
5 University of Pecs
6 FAU - Friedrich-Alexander Universität Erlangen-Nürnberg = University of Erlangen-Nuremberg
7 University hospital of Zurich [Zurich]
8 NHS Foundation Trust [London]
9 Radboud University Medical Center [Nijmegen]
10 UMF lasi - University of Medicine and Pharmacy "Grigore T.Popa" Iasi
11 AP-HP - Hopital Saint-Louis [AP-HP]
12 McGill University = Université McGill [Montréal, Canada]
13 UniFI - Università degli Studi di Firenze = University of Florence = Université de Florence
14 Rappaport faculty of Medicine
15 Oslo University Hospital [Oslo]
16 St Vincent's University Hospital
17 University Hospital of Cologne [Cologne]
18 CHU Nantes - Centre Hospitalier Universitaire de Nantes = Nantes University Hospital
19 University of Liverpool
20 Hôpital Bretonneau
21 Glasgow Royal Infirmary
22 Leeds Teaching Hospitals NHS Trust
23 University of Belgrade [Belgrade]
24 North Bristol NHS Trust [Bristol, UK]
25 Rigshospitalet [Copenhagen]
26 UEA - University of East Anglia [Norwich]
27 University of Bath [Bath]
28 JLU - Justus-Liebig-Universität Gießen = Justus Liebig University
29 Universität zu Lübeck = University of Lübeck [Lübeck]
30 Charité - UniversitätsMedizin = Berlin University Medicine
31 Royal Perth Hospital
32 Hospital Universitario 12 de Octubre [Madrid]
33 Service de Médecine interne A et polyclinique médicale [CHU Limoges]
34 LIRIC - Lille Inflammation Research International Center - U 995
35 Istanbul University
36 Universiteit Utrecht / Utrecht University [Utrecht]
37 University of Adelaide
38 Sahlgrenska Academy at University of Gothenburg [Göteborg]
39 Monash University [Melbourne]
40 Hôpital Edouard Herriot [CHU - HCL]
41 Hôpital Saint-Joseph [Marseille]
42 CHLS - Centre Hospitalier Lyon Sud [CHU - HCL]
43 Hôpital Ambroise Paré [AP-HP]
44 UCL - University College of London [London]
2 Skane University Hospital [Lund]
3 Hôpital Cochin [AP-HP]
4 University of Oxford
5 University of Pecs
6 FAU - Friedrich-Alexander Universität Erlangen-Nürnberg = University of Erlangen-Nuremberg
7 University hospital of Zurich [Zurich]
8 NHS Foundation Trust [London]
9 Radboud University Medical Center [Nijmegen]
10 UMF lasi - University of Medicine and Pharmacy "Grigore T.Popa" Iasi
11 AP-HP - Hopital Saint-Louis [AP-HP]
12 McGill University = Université McGill [Montréal, Canada]
13 UniFI - Università degli Studi di Firenze = University of Florence = Université de Florence
14 Rappaport faculty of Medicine
15 Oslo University Hospital [Oslo]
16 St Vincent's University Hospital
17 University Hospital of Cologne [Cologne]
18 CHU Nantes - Centre Hospitalier Universitaire de Nantes = Nantes University Hospital
19 University of Liverpool
20 Hôpital Bretonneau
21 Glasgow Royal Infirmary
22 Leeds Teaching Hospitals NHS Trust
23 University of Belgrade [Belgrade]
24 North Bristol NHS Trust [Bristol, UK]
25 Rigshospitalet [Copenhagen]
26 UEA - University of East Anglia [Norwich]
27 University of Bath [Bath]
28 JLU - Justus-Liebig-Universität Gießen = Justus Liebig University
29 Universität zu Lübeck = University of Lübeck [Lübeck]
30 Charité - UniversitätsMedizin = Berlin University Medicine
31 Royal Perth Hospital
32 Hospital Universitario 12 de Octubre [Madrid]
33 Service de Médecine interne A et polyclinique médicale [CHU Limoges]
34 LIRIC - Lille Inflammation Research International Center - U 995
35 Istanbul University
36 Universiteit Utrecht / Utrecht University [Utrecht]
37 University of Adelaide
38 Sahlgrenska Academy at University of Gothenburg [Göteborg]
39 Monash University [Melbourne]
40 Hôpital Edouard Herriot [CHU - HCL]
41 Hôpital Saint-Joseph [Marseille]
42 CHLS - Centre Hospitalier Lyon Sud [CHU - HCL]
43 Hôpital Ambroise Paré [AP-HP]
44 UCL - University College of London [London]
Luc Mouthon
- Fonction : Auteur
- PersonId : 1330915
- ORCID : 0000-0002-9838-246X
- IdRef : 069140634
Voon H. Ong
- Fonction : Auteur
Paresh Jobanputra
- Fonction : Auteur
Alison C. Jordan
- Fonction : Auteur
Frances C. Hall
- Fonction : Auteur
Christian Agard
- Fonction : Auteur
- PersonId : 1192626
- ORCID : 0000-0002-1156-0607
- IdRef : 068256752
Mohammed Akil
- Fonction : Auteur
Lorinda Chung
- Fonction : Auteur
Peter Lanyon
- Fonction : Auteur
Yasmeen Ahmad
- Fonction : Auteur
Kuntal Chakravarty
- Fonction : Auteur
Eric Hachulla
- Fonction : Auteur
- PersonId : 1366882
- ORCID : 0000-0001-7432-847X
- IdRef : 031788866
Jennifer Hamilton
- Fonction : Auteur
John S. Mclaren
- Fonction : Auteur
Sanjay Pathare
- Fonction : Auteur
Tom Sheeran
- Fonction : Auteur
Résumé
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.
| Origine | Publication financée par une institution |
|---|---|
| Licence |
