Behçet disease : From pathogenesis to novel therapeutic options

Copyright © 2021. Published by Elsevier Ltd..

Behçet disease (BD) is a complex, multi-systemic inflammatory condition mainly hallmarked by oral and genital ulcers which can also affect the vessels, gastrointestinal tract, central nervous system and even the axial skeleton. Without a clear classification among autoimmune or autoinflammatory conditions, BD has been recently classified as a MHC-I-opathy. BD aetiology is still obscure, but it is thought that certain microorganisms can elicit an aberrant adaptive immune response in the presence of a permissive genetic background. Altered T-cell homeostasis, mostly Th1/Th17 expansion and Treg impairment, could lead to an overactivation of the innate immunity, which underlies tissue damage and thus, signs and symptoms. Immunosuppression and/or immunomodulation are central to the BD management. A complex armamentarium ranging from classical synthetic disease-modifying antirrheumatic drugs to new-era biologic agents or small molecules is available in BD, with different therapeutic outcomes depending on disease manifestations. However, the precise disease mechanisms that underlie BD symptoms are not fully deciphered, which may limit their therapeutic potential and add a significant layer of complexity to the treatment decision-making process. The aim of the present review is to provide an exhaustive overview of the latest breakthroughs in BD pathogenesis and therapeutic options.

Medienart:

E-Artikel

Erscheinungsjahr:

2021

Erschienen:

2021

Enthalten in:

Zur Gesamtaufnahme - volume:167

Enthalten in:

Pharmacological research - 167(2021) vom: 16. Mai, Seite 105593

Sprache:

Englisch

Beteiligte Personen:

Rodríguez-Carrio, Javier [VerfasserIn]
Nucera, Valeria [VerfasserIn]
Masala, Ignazio Francesco [VerfasserIn]
Atzeni, Fabiola [VerfasserIn]

Links:

Volltext

Themen:

Antibodies, Monoclonal
Apremilast
Behçet disease
Biological Factors
IL-17
Journal Article
Pathogenesis
Research Support, Non-U.S. Gov't
Review
Therapy

Anmerkungen:

Date Completed 09.12.2021

Date Revised 14.12.2021

published: Print-Electronic

Citation Status MEDLINE

doi:

10.1016/j.phrs.2021.105593

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM323822274