Autoimmune Pemphigus: Latest Advances and Emerging Therapies.
advance in pemphigus
autoimmune bullous diseases
autoimmunity
pemphigus
pemphigus treatment
Journal
Frontiers in molecular biosciences
ISSN: 2296-889X
Titre abrégé: Front Mol Biosci
Pays: Switzerland
ID NLM: 101653173
Informations de publication
Date de publication:
2021
2021
Historique:
received:
03
11
2021
accepted:
20
12
2021
entrez:
21
2
2022
pubmed:
22
2
2022
medline:
22
2
2022
Statut:
epublish
Résumé
Pemphigus represents a group of rare and severe autoimmune intra-epidermal blistering diseases affecting the skin and mucous membranes. These painful and debilitating diseases are driven by the production of autoantibodies that are mainly directed against the desmosomal adhesion proteins, desmoglein 3 (Dsg3) and desmoglein 1 (Dsg1). The search to define underlying triggers for anti-Dsg-antibody production has revealed genetic, environmental, and possible vaccine-driven factors, but our knowledge of the processes underlying disease initiation and pathology remains incomplete. Recent studies point to an important role of T cells in supporting auto-antibody production; yet the involvement of the myeloid compartment remains unexplored. Clinical management of pemphigus is beginning to move away from broad-spectrum immunosuppression and towards B-cell-targeted therapies, which reduce many patients' symptoms but can have significant side effects. Here, we review the latest developments in our understanding of the predisposing factors/conditions of pemphigus, the underlying pathogenic mechanisms, and new and emerging therapies to treat these devastating diseases.
Identifiants
pubmed: 35187073
doi: 10.3389/fmolb.2021.808536
pii: 808536
pmc: PMC8855930
doi:
Types de publication
Journal Article
Review
Langues
eng
Pagination
808536Informations de copyright
Copyright © 2022 Lim, Bohelay, Hanakawa, Musette and Janela.
Déclaration de conflit d'intérêts
BJ receives research funding from Evonik. PM is a consultant for Servier. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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