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Management of skin, mucosa and joint involvement of Behçet's syndrome: A systematic review for update of the EULAR recommendations for the management of Behçet's syndrome

Research output: Contribution to journalJournal articleResearchpeer-review

  • Pietro Leccese
  • Yesim Ozguler
  • Robin Christensen
  • Sinem Nihal Esatoglu
  • Dongsik Bang
  • Bahram Bodaghi
  • Aykut Ferhat Celik
  • Farida Fortune
  • Julien Gaudric
  • Ahmet Gül
  • Ina Kötter
  • Alfred Mahr
  • Robert J Moots
  • Jutta Richter
  • David Saadoun
  • Carlo Salvarani
  • Francesco Scuderi
  • Petros P Sfikakis
  • Aksel Siva
  • Miles Stanford
  • Ilknur Tugal-Tutkun
  • Richard West
  • Sebahattin Yurdakul
  • Ignazio Olivieri
  • Hasan Yazici
  • Gulen Hatemi
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OBJECTIVES: The aim of this systematic review was to inform the update of European League Against Rheumatism (EULAR) Recommendations for the management of Behçet's syndrome (BS), on the evidence for the treatment of skin, mucosa and joint involvement of BS.

METHODS: A systematic literature search, data extraction, statistical analyses and assessment of the quality of evidence were performed according to a pre-specified protocol using the PRISMA guidelines. Studies that assessed the efficacy of an intervention in comparison to an active comparator or placebo for oral ulcers, genital ulcers, papulopustular lesions, nodular lesions or arthritis were included. Where possible, risk ratios were calculated for binary outcomes and mean difference for continuous outcomes.

RESULTS: Among the 3927 references that were screened, 37 were included in the analyses. Twenty-seven of these assessed mucocutaneous and 17 assessed joint involvement. Twenty-one of these studies were randomised controlled trials (RCTs). RCTs with colchicine, azathioprine, interferon-alpha, thalidomide, etanercept and apremilast showed beneficial results with some differences according to lesion type and gender. These agents were generally well tolerated with few adverse events causing withdrawal from the study.

CONCLUSIONS: RCTs comprised more than a half (21/37, 57%) of the sources included in the evidence synthesis related to skin, mucosa and joint involvement applicable for the EULAR Recommendations for the management of BS. Differences in the outcome measures that were used across the included studies often made it difficult to combine and compare the results.

Original languageEnglish
JournalSeminars in Arthritis and Rheumatism
Issue number4
Pages (from-to)752-762
Number of pages11
Publication statusPublished - Feb 2019

    Research areas

  • Behcet Syndrome/drug therapy, Humans, Immunosuppressive Agents/therapeutic use, Joints/pathology, Mucous Membrane/pathology, Skin/pathology, Behçet's disease, Treatment, Anti-TNF, Systematic review

ID: 57027481