Fiche publication
Date publication
septembre 2024
Journal
Autoimmunity reviews
Auteurs
Membres identifiés du Cancéropôle Est :
Pr MULLER Sylviane
Tous les auteurs :
Brady S, Poulton J, Muller S
Lien Pubmed
Résumé
Inclusion body myositis (IBM) is a late onset sporadic myopathy with a characteristic clinical presentation, but as yet unknown aetiology or effective treatment. Typical clinical features are early predominant asymmetric weakness of finger flexor and knee extensor muscles. Muscle biopsy shows endomysial inflammatory infiltrate, mitochondrial changes, and protein aggregation. Proteostasis (protein turnover) appears to be impaired, linked to potentially dysregulated chaperone-mediated autophagy and mitophagy (mitochondrial quality control). In this review, we bring together the most recent clinical and biological data describing IBM. We then address the question of diagnosing this pathology and the relevance of the current biological markers that characterize IBM. In these descriptions, we put a particular emphasis on data related to the deregulation of autophagic processes and to the mitochondria-lysosome crosstalk. Finally, after a short description of current treatments, an overview is provided pointing towards novel therapeutic targets and upcoming regulating molecules tested for treating IBM. Special attention is paid to autophagy inhibitors that may offer innovative breakthrough therapies for patients with IBM.
Mots clés
Autoimmunity, Autophagy, Idiopathic inflammatory myopathies, Inclusion body myositis, Mitochondrial disease, Novel treatment
Référence
Autoimmun Rev. 2024 09 19;:103644
