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. 2019 Mar 4;5(1):e000826.
doi: 10.1136/rmdopen-2018-000826. eCollection 2019.

Setting the international standard for longitudinal follow-up of patients with systemic sclerosis: a Delphi-based expert consensus on core clinical features

Anna-Maria Hoffmann-Vold  1 , Oliver Distler  2 , Baron Murray  3 , Otylia Kowal-Bielecka  4 , Dinesh Khanna  5 , Yannick Allanore  6   7 ; EUSTAR and SCTC collaborators
Affiliations

Setting the international standard for longitudinal follow-up of patients with systemic sclerosis: a Delphi-based expert consensus on core clinical features

Anna-Maria Hoffmann-Vold et al. RMD Open. .

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.

Keywords: autoimmune diseases; multidisciplinary team-care; qualitative research; systemic sclerosis.

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Conflict of interest statement

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.

Figures

Figure 1
Figure 1
Delphi method applied to achieve expert consensus on standardised assessment of organ involvement in SSc. SSc, systemic sclerosis.
Figure 2
Figure 2
Results from the second Delphi step showing the percentage of rated domains that the experts agreed on as domains for the annual systemic assessment of organ involvement in systemic sclerosis. DU, digital ulcers; GI, gastrointestinal; RP, Raynaud’s phenomenon.
See this image and copyright information in PMC

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