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The European Scleroderma Trials and Research group (EUSTAR) task force for the development of revised activity criteria for systemic sclerosis: derivation and validation of a preliminarily revised EUSTAR activity index
  1. Gabriele Valentini1,
  2. Michele Iudici1,
  3. Ulrich A Walker2,
  4. Veronika K Jaeger2,
  5. Murray Baron3,
  6. Patricia Carreira4,
  7. László Czirják5,
  8. Christopher P Denton6,
  9. Oliver Distler7,
  10. Eric Hachulla8,
  11. Ariane L Herrick9,
  12. Otylia Kowal-Bielecka10,
  13. Janet Pope11,
  14. Ulf Müller-Ladner12,
  15. Gabriela Riemekasten13,
  16. Jerome Avouac14,
  17. Marc Frerix12,
  18. Suzana Jordan7,
  19. Tünde Minier5,
  20. Elise Siegert14,
  21. Voon H Ong6,
  22. Serena Vettori1,
  23. Yannick Allanore15
  1. 1Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples, Naples, Italy
  2. 2Department of Rheumatology, Basel University, Basel, Switzerland
  3. 3Division of Rheumatology, Jewish General Hospital, McGill University, Montreal, Quebec, Canada
  4. 4Department of Rheumatology, 12 de Octubre University Hospital, Madrid, Spain
  5. 5Department of Rheumatology and Immunology, University of Pécs, Medical Centre, Pécs, Hungary
  6. 6Division of Medicine, Centre for Rheumatology and Connective Tissue Disease, Royal Free Campus, University College London, London, UK
  7. 7Department of Rheumatology, University Hospital Zurich, Zurich, Switzerland
  8. 8Internal Medicine Department, Claude Huriez Hospital, Lille University, Lille, France
  9. 9NIHR Manchester Musculoskeletal Biomedical Research Unit, Centre for Musculoskeletal Research, Manchester Academic Health Science Centre, The University of Manchester, Manchester, UK
  10. 10Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland
  11. 11Department of Medicine, St. Joseph's Health Care, University of Western Ontario, London, Ontario, Canada
  12. 12Department of Rheumatology and Clinical Immunology, Justus-Liebig University Giessen, Bad Neuheim, Germany
  13. 13Clinic for Rheumatology, University of Lübeck, Lübeck, Germany
  14. 14Department of Rheumatology and Clinical Immunology, University Hospital Charité, Berlin, Germany
  15. 15Rheumatology A Department, INSERM U1016 UMR8104, Cochin Hospital, Paris Descartes University, Paris, France
  1. Correspondence to Professor Gabriele Valentini, Rheumatology Section, Department of Clinical and Experimental Medicine, Second University of Naples, II Policlinico, Edificio 3, Via Pansini 5, Naples 80131, Italy; gabriele.valentini{at}unina2.it

Abstract

Background Validity of European Scleroderma Study Group (EScSG) activity indexes currently used to assess disease activity in systemic sclerosis (SSc) has been criticised.

Methods Three investigators assigned an activity score on a 0–10 scale for 97 clinical charts. The median score served as gold standard. Two other investigators labelled the disease as inactive/moderately active or active/very active. Univariate–multivariate linear regression analyses were used to define variables predicting the ‘gold standard’, their weight and derive an activity index. The cut-off point of the index best separating active/very active from inactive/moderately active disease was identified by a receiver-operating curve analysis. The index was validated on a second set of 60 charts assessed by three different investigators on a 0–10 scale and defined as inactive/moderately active or active/very active by other two investigators. One hundred and twenty-three were investigated for changes over time in the index and their relationships with those in the summed Medsger severity score (MSS).

Results A weighted 10-point activity index was identified and validated: Δ-skin=1.5 (Δ=patient assessed worsening during the previous month), modified Rodnan skin score (mRss) >18=1.5, digital ulcers=1.5, tendon friction rubs=2.25, C-reactive protein >1 mg/dL=2.25 and diffusing capacity of the lung for CO (DLCO) % predicted <70%=1.0. A cut-off ≥2.5 was found to identify patients with active disease. Changes in the index paralleled those of MSS (p=0.0001).

Conclusions A preliminarily revised SSc activity index has been developed and validated, providing a valuable tool for clinical practice and observational studies.

  • Systemic Sclerosis
  • Disease Activity
  • Autoimmune Diseases

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Footnotes

  • Handling editor Tore K Kvien

  • Contributors Design of the study: GV and YA. Acquisition of data: GV, MI, UAW, VKJ, PC, LC, CPD, OD, EH, AH, OK-B, JP, UM-L, GR, JA, MF, SJ, TM, ES, VHO, SV and YA. Data interpretation and analysis: GV, MI, UAW, VKJ, MB, PC, LC, CPD, OD, EH, AH, OK-B, JP, UM-L, GR and YA. Drafting and revisiting the manuscript: GV, MI, UAW, VKJ, PC, LC, CPD, OD, EH, AH, OK-B, JP, UM-L, GR and YA. Final approval of the manuscript: GV, MI, UAW, VKJ, MB, PC, LC, CPD, OD, EH, AH, OK-B, JP, UM-L, GR, JA, MF, SJ, TM, ES, VHO, SV and YA.

  • Competing interests GV has received research funding in the area of systemic sclerosis from Abbvie, Actelion, Bayer, BMS, Merck SD, Pfizer and Roche. CPD has been a consultant to Roche, GSK, Actelion, Inventiva, CSL Behring, Takeda, Merck-Serono, MedImmune and Biogen. He has received research grants from Actelion, GSK, Novartis and CSL Behring. AH has undertaken consultancy work and received speaker's fees and research funding from Actelion. She has undertaken consultancy work for Apricus. OD has/had a consultancy relationship and/or has received research funding in the area of systemic sclerosis and related conditions from 4 D Science, Actelion, Active Biotec, BMS, Boehringer Ingelheim, EpiPharm, BiogenIdec, Genentech/Roche, GSK, Inventiva, Lilly, Medac, Pfizer, Serodapharm, Sinoxa, Ergonex, Pharmacyclics and Sanofi. In addition, OD has a patent mir-29 for the treatment of systemic sclerosis licensed. YA has/had consultancy relationship and/or has received research funding in relationship with the treatment of systemic sclerosis from Actelion, Bayer, Biogen Idec, Bristol-Myers Squibb, Genentech/Roche, Inventiva, Medac, Pfizer, Sanofi/Genzyme, Servier and UCB. JP has consulted for Actelion, Bayer, BMS, Merck, Pfizer and Roche.

  • Patient consent Obtained.

  • Ethics approval IRB approval was obtained previously as EULAR/EUSTAR data were used that were collected previously on patients who had signed informed consent.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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