French recommendations for the management of Behcet's disease

被引：47
作者：
Kone-Paut, Isabelle [1 ,2 ]
Barete, Stephane [3 ,4 ]
Bodaghi, Bahram [5 ]
Deiva, Kumaran [6 ,7 ]
Desbois, Anne-Claire [8 ,9 ,10 ,11 ]
Galeotti, Caroline [1 ]
Gaudric, Julien [12 ]
Kaplanski, Gilles [13 ]
Mahr, Alfred [14 ]
Noel, Nicolas [15 ,16 ,17 ]
Piram, Maryam [1 ,18 ]
Tran, Tu-Anh [19 ]
Wechsler, Bertrand [8 ,9 ,10 ,11 ]
Saadoun, David [8 ,9 ,10 ,11 ]
机构：
[1] Univ Paris Sud Saclay, Bicetre Hosp, AP HP, Pediat Rheumatol, Le Kremlin Bicetre, France
[2] Univ Paris Sud Saclay, Bicetre Hosp, AP HP, CEREMAIA, Le Kremlin Bicetre, France
[3] Sorbonne Univ, DMU3ID, Grp Hosp Pitie Salpetriere, AP HP,Unit Dermatol, Paris, France
[4] Sorbonne Univ, Inflammat Immunopathol Biotherapy Dept DHU i2B, INSERM, UMRS 959, Paris, France
[5] Sorbonne Univ, Pitie Salpetriere Hosp, AP HP, Dept Ophthalmol,CRMR OPHTARA,IHU FOReSIGHT, Paris, France
[6] Bicetre Hosp, Natl Referral Ctr Rare Inflammatory Brain & Spina, Univ Hosp Paris Saclay, AP HP,Dept Pediat Neurol, Paris, France
[7] Univ Paris Saclay, INSERM, Immunol Viral Infect & Autoimmune Dis, UMR1184, Le Kremlin Bicetre, France
[8] Sorbonne Univ, Immunol Immunopathol Immunotherapy I3, UPMC Univ Paris 06, Inserm,UMR S 959, F-75005 Paris, France
[9] Hop La Pitie Salpetriere, AP HP, Biotherapy CIC BTi, F-75651 Paris, France
[10] Hop La Pitie Salpetriere, AP HP, Inflammat Immunopathol Biotherapy Dept DHU i2B, F-75651 Paris, France
[11] Grp Hosp Pitie Salpetriere, Ctr Natl References Malad Autoimmunes Syst Rares, Ctr Natl Reference Malad Autoinflammatoires & Amy, Dept Internal Med & Clin Immunol,AP HP, F-75013 Paris, France
[12] Sorbonne Univ, Pitie Salpetriere Hosp, AP HP, Dept Vasc Surg, Paris, France
[13] Aix Marseille Univ, Hop Concept, Internal Med & Clin Immunol Dept, Marseille, France
[14] Kantonsspital St Gallen, Clin Rheumatol, St Gallen, Switzerland
[15] CHU Bicetre, Serv Med Interne & Immunol Clin, AP HP, Le Kremlin Bicetre, France
[16] Univ Paris Saclay, Immunol Malad Virales & Autoimmunes, INSERM, UMR 1184, Le Kremlin Bicetre, France
[17] IDMIT, Div Immunovirol, CEA, DSV iMETI, Fontenay Aux Roses, France
[18] Univ Montreal, CHU Sainte Justine, CHU Sainte Justine Res Ctr, Pediat Dermatol, Montreal, PQ, Canada
[19] Montpellier Nimes Univ, Nimes Univ Hosp, INSERM, Dept Pediat,U1183, Nimes, France
来源：
ORPHANET JOURNAL OF RARE DISEASES | 2021年 / 16卷 / SUPPL 1期
关键词：
Behç et’ s disease; Recommendation; Management;
D O I：
10.1186/s13023-020-01620-4
中图分类号：
Q3 [遗传学];
学科分类号：
071007 ; 090102 ;
摘要：
Behcet'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 Behcet's disease [Protocole National de Diagnostic et de Soins de la maladie de Behcet (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.
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页数：28
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