Étude qualitative par entretiens individuels des conséquences des myopathies inflammatoires sur la qualité de vie

Introduction La survenue d’une myopathie inflammatoire engendre un bouleversement majeur de la vie quotidienne des patients, notamment du fait de la diminution des capacites fonctionnelles liees a l’atteinte musculaire. Les consequences psychologiques et socioprofessionnelles des myopathies inflammatoires sont importantes mais restent actuellement sous-evaluees. Les outils d’evaluation de la qualite de vie utilisables au cours des myopathies inflammatoires sont rares, generiques mais non specifiques et ne permettent pas d’approcher exhaustivement les problematiques des patients. Cette etude qualitative a donc ete menee dans le but d’identifier les determinants du retentissement des myopathi…

Nuclear actin aggregation is a hallmark of anti-synthetase syndrome-induced dysimmune myopathy

Objective: To analyze antisynthetase syndrome–associated myositis by modern myopathologic methods and to define its place in the spectrum of idiopathic inflammatory myopathies (IIMs). Methods: Skeletal muscle biopsies from antisynthetase syndrome–associated myositis and other IIMs from different institutions worldwide were analyzed by histopathology, quantitative PCR, and electron microscopy. Results: Myonuclear actin filament inclusions were identified as a unique morphologic hallmark of antisynthetase syndrome–associated myositis. Nuclear actin inclusions were never found in dermatomyositis, polymyositis, sporadic inclusion body myositis, autoimmune necrotizing myopathy associated with si…

205th ENMC International Workshop: Pathology diagnosis of idiopathic inflammatory myopathies Part II 28-30 March 2014, Naarden, The Netherlands.

The idiopathic inflammatory myopathies (IM) are a heterogeneous group of diseases and diagnosis often necessitates a muscle biopsy. Five main entities are recognized: (1) dermatomyositis (DM); (2) polymyositis (PM); (3) necrotizing autoimmune myopathy (NAM); (4) sporadic inclusion body myositis (IBM); and (5) non-specific myositis. Other entities include granulomatous myopathy, macrophagic myofasciitis, and eosinophilic fasciitis (Shulman's syndrome). The pathological classification and subsequent identification of disease subgroups are extremely important for assessing treatment options and prognosis in the individual patient, yet classification criteria have not been standardized and vali…

Moving from Dermatomyositis Associated with Anti-Melanoma Differentiation-Associated Gene 5 Antibody to Anti-Melanoma Differentiation-Associated Gene 5 Syndrome

International audience

Systematic retrospective study of 64 patients with anti-Mi2 dermatomyositis: A classic skin rash with a necrotizing myositis and high risk of malignancy