Titre :
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Rapamycin for inclusion body myositis: targeting non-inflammatory mechanisms
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Revue :
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Rheumatology
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Auteurs :
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Lilleker JB, Auteur ;
M Bukhari ;
Chinoy H
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Type de document :
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Article
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Année de publication :
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02/2018
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Langues:
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Anglais
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Mots-clés :
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arimoclomol
;
essai clinique
;
essai clinique de phase 2
;
évaluation de la force musculaire
;
examen respiratoire
;
immunosuppression
;
IRM
;
malade
;
mécanisme d'action
;
mTOR
;
muscle
;
muscle quadriceps fémoral
;
myopathie inflammatoire idiopathique
;
myosite à inclusions
;
sirolimus
;
test de marche de 6 minutes
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Résumé :
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Inclusion body myositis (IBM) is an acquired myopathy usually occurring in those aged >50 years and with a prevalence of 33 cases per million. While conventionally grouped with the idiopathic inflammatory myopathies, IBM has several unique clinical and pathological characteristics. Muscle inflammation is a prominent feature, but the disease is resistant to treatment with routine immunosuppressive therapies, none of which have demonstrated sustained therapeutic benefits. Disease progression is characterised by an accumulation of degenerative change in skeletal muscle, including misfolded protein aggregates and rimmed vacuoles. The exact sequence of events that culminate in severely disabling muscle atrophy is the subject of intense debate and the lack of effective treatment represents a significant unmet need for sufferers...
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Lien associé :
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accès au document numérique
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Pubmed / DOI :
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DOI : 10.1093/rheumatology/key043
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Voir aussi :
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