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Original article
Setting the international standard for longitudinal follow-up of patients with systemic sclerosis: a Delphi-based expert consensus on core clinical features
  1. Anna-Maria Hoffmann-Vold1,
  2. Oliver Distler2,
  3. Baron Murray3,
  4. Otylia Kowal-Bielecka4,
  5. Dinesh Khanna5 and
  6. Yannick Allanore6,7
  7. EUSTAR and SCTC collaborators
  1. 1 Department of Rheumatology, Oslo University Hospital, Oslo, Norway
  2. 2 Department of Rheumatology, University of Zurich, Zurich, Switzerland
  3. 3 Rheumatology, Jewish General Hospital, Montreal, Quebec, Canada
  4. 4 Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland
  5. 5 Scleroderma Program, University of Michigan, Ann Arbor, Michigan, USA
  6. 6 Rheumatology A Department, Universite Paris Descartes, Paris, France
  7. 7 INSERM U1016, Immunology, Cochin Institute, Paris, France
  1. Correspondence to Dr Anna-Maria Hoffmann-Vold; a.m.hoffmann-vold{at}medisin.uio.no

Abstract

Background Systemic sclerosis (SSc) is a severe, progressive multiorgan disease but to date, there are no established standardised international guidelines for follow-up of patients with SSc. The goal of this project was to develop an expert consensus for annual systematic investigations in patients with SSc to enhance their standard-of-care.

Material and methods The Delphi method was applied. All SSc experts from the European Scleroderma Trials and Research group network and the Scleroderma Clinical Trial Consortium were invited to participate. All experts were asked to answer questionnaires in five Delphi steps to determine the domains of interest and tools for each domain for an annual systematic assessment of patients with SSc. Each item was rated on a scale between 0% and 100% (not and very important), and parameters rated >80% by more than 75% of the experts were regarded as acceptable.

Results In total, 157 experts worldwide participated with 71.3% experts seeing >50 patients with SSc annually. In the first round, 23 domains and 204 tools were suggested. After five Delphi steps, experts agreed on 10 domains including (1) Raynaud’s phenomenon; (2) Digital ulcers; (3) Skin and mucosa; (4) Lung; (5); Heart; (6) GI domain, (7) Renal; (8) Musculoskeletal; (9) Laboratory and (10) Treatment. Overall, 55 tools were identified including clinical assessments, laboratory measurements and imaging or functional investigations.

Conclusion Through five Delphi steps with world leading experts, a consensus was established on strongly suggested tools for a minimum annual systemic assessment of organ involvement in SSc. This work should enhance the standardisation and homogenisation of the practices.

  • systemic sclerosis
  • autoimmune diseases
  • multidisciplinary team-care
  • qualitative research

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Footnotes

  • Collaborators EUSTAR and SCTC collaborators.

  • Contributors Substantial contributions to the conception or design of the work; or the acquisition, analysis or interpretation of data for the work: All authors. Drafting the work or revising it critically for important intellectual content: All authors. Final approval of the version to be published: All authors. Agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved: All authors.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests A-MH-V has obtained research support from Boehringer Ingelheim. She is a scientific consultant for Actelion and Boehringer Ingelheim in the field of systemic sclerosis and related diseases. She has received travel expenses from GSK, Actelion and Boehringer Ingelheim. OD has obtained research support from Bayer, Sanofi, Ergonex, Boehringer Ingelheim, Actelion and Pfizer. He is a scientific consultant for 4 D Science, Actelion, Active Biotec, Bayer, BiogenIdec, BMS, Boehringer Ingelheim, ChemoAb, EpiPharm, Ergonex, espeRare foundation, Genentech/Roche, GSK, Inventiva, Lilly, medac, MedImmune, Pharmacyclics, Pfizer, Serodapharm and Sinoxa in the field of systemic sclerosis and related diseases and has a patent licensed on mir-29 for the treatment of systemic sclerosis. OK-B received consultancies, honoraria and/or speaker fees from Abbvie, Actelion, Bayer, Roche. DK has received consulting fees from Actelion, Bayer, Bristol-Myers Squibb, Cytori, CSL Behring, Corbus, Genentech/Roche, GlaxoSmithKline, Inventiva, Regeneron, Sanofi-Aventis and UCB and has stock options with Eicos Sciences, Inc. 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.

  • Patient consent for publication Not required.

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

  • Data sharing statement All data collected for this manuscript are included and there are no additional data for sharing.

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