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Review
. 2021 Feb 24;16(Suppl 1):352.
doi: 10.1186/s13023-020-01620-4.

French recommendations for the management of Behçet's disease

Isabelle Kone-Paut  1 , Stéphane Barete  2 , Bahram Bodaghi  3 , Kumaran Deiva  4   5 , Anne-Claire Desbois  6   7   8 , Caroline Galeotti  9 , Julien Gaudric  10 , Gilles Kaplanski  11 , Alfred Mahr  12 , Nicolas Noel  13   14   15 , Maryam Piram  9   16 , Tu-Anh Tran  17 , Bertrand Wechsler  6   7   8 , David Saadoun  18   19   20 ; Collaborators
Collaborators, Affiliations
Review

French recommendations for the management of Behçet's disease

Isabelle Kone-Paut et al. Orphanet J Rare Dis. .

Abstract

Behçet's disease (BD) is a systemic variable vessel vasculitis that involves the skin, mucosa, joints, eyes, arteries, veins, nervous system and gastrointestinal system, presenting with remissions and exacerbations. It is a multifactorial disease, and several triggering factors including oral cavity infections and viruses may induce inflammatory attacks in genetically susceptible individuals. BD vasculitis involves different vessel types and sizes of the vascular tree with mixed-cellular perivascular infiltrates and is often complicated by recurrent thrombosis, particularly in the venous compartment. Several new therapeutic modalities with different mechanisms of action have been studied in patients with BD. A substantial amount of new data have been published on the management of BD, especially with biologics, over the last years. These important therapeutic advances in BD have led us to propose French recommendations for the management of Behçet's disease [Protocole National de Diagnostic et de Soins de la maladie de Behçet (PNDS)]. These recommendations are divided into two parts: (1) the diagnostic process and initial assessment; (2) the therapeutic management. Thirty key points summarize the essence of the recommendations. We highlighted the main differential diagnosis of BD according to the type of clinical involvement; the role of genetics is also discussed, and we indicate the clinical presentations that must lead to the search for a genetic cause.

Keywords: Behçet’s disease; Management; Recommendation.

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Conflict of interest statement

IKP reports consulting fees/meeting fees from Novartis, Abbvie, SOBI, LFB; speaking fees from Novartis, SOBI, Pfizer, Zeste Research, LFB; and research funding from Novartis. SB, ACD, CG, JG, GK, NN and DS reports no conflicts of interest. BB reports consulting fees/meeting fees from Allergan, Santen, Abbvie, Alimera; speaking fees from Abbvie, Alimera. AM reports consulting fees/meeting fees from Celgène and speaking fees from Roche-Chugaï. MP reports consulting fees/meeting fees from Patent Valor and research funding from Novartis. TAT reports speaking fees from Abbvie and Roche-Chugaï.

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