Objectives: This study aims to update the European Alliance of Associations for Rheumatology (EULAR) recommendations for the management of Behet's syndrome according to the updated EULAR standard operating procedures.Methods: The task force comprised 29 members from 11 countries, including 19 rheumatologists, 2 ophthalmologists, 1 dermatologist, 1 gastroenterologist, 1 neurologist, 1 health professional, 2 patient research partners, and 2 Emerging EUlar NETwork members.Research questions were proposed by the task force through a Delphi survey and formulated into patients, interventions, comparison, and outcomes (PICO) questions for the systematic literature review.The results of the systematic literature review were discussed among the task force members.Previous recommendations and overarching principles were modified, and new recommendations were developed as needed.The updated recommendations were voted, and the levels of evidence and levels of agreement were determined.Results: The updated recommendations consist of 5 overarching principles and 12 recommendations that were tabulated according to organ involvement.Among the 12 recommendations, 1 was a new recommendation, 7 recommendations were modified, and only the wording was changed in 4 recommendations.The overarching principles focus on the importance of recognising the relapsing and remitting disease course and individualising treatment according to disease activity and prognostic risk factors, and emphasise the importance of a multidisciplinary approach, patient education, and shared decision making for optimal care.For mucocutaneous and joint involvement, colchicine is recommended as the first-line treatment modality.Apremilast and immunosuppressives such as tumour necrosis factor alpha (TNF) inhibitors are recommended for refractory patients.For patients with organ involvement, more aggressive treatment with glucocorticoids and immunosuppressives is recommended for rapid induction of remission.Early use of monoclonal antibodies against TNF is encouraged in patients with organ or lifethreatening manifestations.Conclusions: These recommendations, which were updated based on new evidence and expert opinion, provide guidance for all stakeholders involved in the management of patients with Behet's syndrome to improve the quality of care of these patients.
Hatemi et al. (Sun,) studied this question.