Title :

French recommendations for the management of Behçet’s disease

Author(s) :

Kone-Paut, Isabelle [Auteur]
Barete, Stéphane [Auteur]
Bodaghi, Bahram [Auteur]
Deiva, Kumaran [Auteur]
Desbois, Anne-Claire [Auteur]
Galeotti, Caroline [Auteur]
Gaudric, Julien [Auteur]
Kaplanski, Gilles [Auteur]
Mahr, Alfred [Auteur]
Noel, Nicolas [Auteur]
Piram, Maryam [Auteur]
Tran, Tu-Anh [Auteur]
Wechsler, Bertrand [Auteur]
Saadoun, David [Auteur]
Lambert, Marc [Auteur]
Facteurs de Risque et Déterminants Moléculaires des Maladies liées au Vieillissement - U 1167 [RID-AGE]

Journal title :

Orphanet journal of rare diseases

Pages :

352

Publisher :

BioMed Central

Publication date :

2021-02-24

ISSN :

1750-1172

HAL domain(s) :

Sciences du Vivant [q-bio]

English abstract : [en]

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. ...
Show more >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.Show less >

Language :

Anglais

Peer reviewed article :

Oui

Audience :

Internationale

Popular science :

Non

Collections :

• Facteurs de risque et déterminants moléculaires des maladies liées au vieillissement (RID-AGE) - U1167

Source :

Harvested from HAL