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Defining skin ulcers in systemic sclerosis: systematic literature review and proposed World Scleroderma Foundation (WSF) definition

Defining skin ulcers in systemic sclerosis: systematic literature review and proposed World Scleroderma Foundation (WSF) definition

J scleroderma relat disord 2017; 2(2): 115 - 120

Article Type: ORIGINAL RESEARCH ARTICLE

DOI:10.5301/jsrd.5000236

Authors

Yossra Atef Suliman, Cosimo Bruni, Sindhu R. Johnson, Emanuela Praino, Mohamed Alemam, Nabeel Borazan, Laura Cometi, Bethany Myers, Dinesh Khanna, Yannick Allanore, Murray Baron, Thomas Krieg, Ariane Herrick, Ana Afonso, Oliver Distler, Suzanne Kafaja, Christopher P. Denton, Marco Matucci-Cerinic, Daniel E. Furst

Abstract

Purpose

There is a lack of a valid definition for skin ulcers in systemic sclerosis (SSc) to be used in clinical trials. Our aim was to develop a consensus definition for SSc skin ulcers based on the results of a systematic literature review (SLR) for skin ulcer definitions and expert opinion; and to evaluate its face validity, reliability and feasibility.

Methods

SLR for skin ulcer definitions was conducted using PubMed, Web of Science, and Cochrane library for articles published from inception to January 1st, 2016. SSc experts were to discuss the definition categories and vote for the relevant terms. Reliability of the definition was tested in a second expert meeting, seven SSc experts evaluated 7 SSc patients with skin lesions twice. Face validity and feasibility were evaluated by sending out case report forms (CRFs) to four SSc experts, who were each asked to use the definition in five patients.

Results

A total of 3464 abstracts and titles were screened, and 446 articles were fully evaluated. Of these, 66 met eligibility criteria and skin ulcer definitions were extracted. SSc experts discussed, refined, and voted on the consensus definition using nominal process. Kappa for inter-rater agreement was 0.51 and for intra-rater agreement was 0.90. The mean time to decide if the lesion is an ulcer was 7.4 seconds. All investigators endorsed the face validity of the new definition in the CRFs.

Conclusions

Using an SLR and a nominal technique, we developed a preliminary consensus-based definition of SSc skin ulcers. Face validity, feasibility and reliability were demonstrated for the developed definition.

Article History

Disclosures

Financial support: The study was supported by an unrestricted grant from the World Scleroderma Foundation. Y.A. Suliman was supported by Egyptian government scholarship program, Joint supervision. S.R. Johnson has been awarded a Canadian Institutes of Health Research New Investigator Award, and is supported by the Oscar and Eleanor Markovitz Scleroderma Research Fund. This work was funded in part by the NIH/NIAMS K24 AR06312 grant.
Conflict of interest: D. Khanna has consultancy relationships with Actelion, Bayer, Bristol-Myers Squibb, ChemomAb, Corbus, Cytori, Eicos, EMD Serono, Genentech/Roche, Gilead, GlaxoSmithKline, MedImmune, Medac, and Sanofi-Aventis. He has received research funding from Astra-Zeneca, Bayer, Bristol-Myers Squibb, and Pfizer. C. Denton: has received grants from CSL Behring, Glaxo-SmithKline and Inventiva (paid to his institution); consultancy fees from Bristol-Myers Squibb, Merck-Serono, GlaxoSmithKline, Inventiva, and Genentech/Roche; and speaker fees from Actelion and Bayer. M. Matucci-Cerinic: received grants from BMS, lectured Actelion, Pfizer, Lilly, Hospira and on the boards of Actelion, BMS, Lilly, Pfizer, Sandoz. Y. Allanore: has consultancy relationships 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. D. Furst: has consultancy relationships and/or has received research funding in relationship with the treatment of systemic sclerosis from Abbvie, Actelion, Amgen, BMS, Celgene, Genentech/Roche, Gilead, GSK, NIH, Novartis, UCB.

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Authors

Affiliations

  •  Division of Rheumatology, David Geffen School of Medicine, University of California Los Angeles, California - USA
  •  Rheumatology and Rehabilitation Department, Assiut University Hospital, Assiut - Egypt
  •  Department of Clinical and Experimental Medicine, University of Florence and Division of Rheumatology and Scleroderma Unit AOUC, University of Florence, Florence - Italy
  •  Toronto Scleroderma Program, Toronto Western Hospital, University of Toronto, Toronto, Ontario - Canada
  •  Rheumatology Unit, University of Bari, Bari - Italy
  •  Clinical Pathology Department, South Valley University, Qena - Egypt
  •  Louise M. Darling Biomedical Library, University of California, Los Angeles - USA
  •  University of Michigan, Ann Arbor, Michigan - USA
  •  Paris Descartes University and Cochin Hospital, AP-HP, Paris - France
  •  Jewish General Hospital and McGill University, Montreal, Quebec - Canada
  •  Department of Dermatology, University of Cologne, Cologne - Germany
  •  University of Manchester, Manchester Academic Health Science Centre, Manchester - UK
  •  University of Oporto, Oporto - Portugal
  •  Division of Rheumatology, University Hospital Zurich, Zurich - Switzerland
  •  Centre for Rheumatology, Royal Free Hospital, London - UK

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