Inflammatory muscle diseases: a critical review on pathogenesis and therapies

Angiotensin-converting enzyme insertion/deletion gene polymorphism is associated with dermatomyositis

“…10,14 In contrast to these diseases, there is no study thus far that has analyzed the ACE polymorphism in DM, a disease that involves vascular pathogenesis mediated by humoral processes with secondary ischemic changes of muscle fibers. 1,2,31,32 In these conditions, abnormalities have been found in the endothelium of capillaries, arterioles, and veins, which have been suggested to represent primary disease-related alterations. 31,32 DM is characterized by perifascicular atrophy, perivascular inflammation, microvascular deposits of the C5b-9 membrane attack complex on endothelial cells, and endomysial capillaries that are enlarged and reduced in number, with partial endothelial swelling.…”

“…1 The cornerstone of DM pathogenesis involves vascular disturbances that lead to hypoxia, capillary necrosis and muscle perifascicular atrophy. 1,2 The insertion/deletion (I/D) polymorphism of the angiotensin-converting enzyme (ACE) gene in an intron of the ACE gene, is in strong linkage disequilibrium with genetic factors that influence serum ACE concentrations. 3 ACE is expressed in a wide range of tissues including the lung, vascular endothelium, kidney, and heart.…”

Case Report of Necrotizing Autoimmune Myositis With Slightly High Creatinine Phosphokinase

“…They express CD68 and MHC class 1 and MHC class 2 antigens. CD3 T cells, mainly CD8, forming perivascular cuffs are frequently found [1][2][3][4][5]. CPK are usually found in higher ranges, i.e.…”

“…Based on distinct clinical, immunopathological, histological and prognostic criteria, as well as different degrees of response to therapies, the IM seen in practice can be separated into four distinct subsets: polymyositis (PM), dermatomyositis (DM), necrotizing autoimmune myositis (NAM) and sporadic inclusion body myositis (sIBM) [4,5]. The disorders have primarily an autoimmune pathogenesis, mediated either by cytotoxic T cells, as in PM and sIBM, by a complement-mediated microangiopathy as in DM, or by macrophages and possibly autoantibodies as in NAM [1][2][3][4][5].…”

Diagnostic des myopathies inflammatoires au CHU de Bordeaux de 2012 à 2014

“…La découverte de nouveaux auto-anticorps (AAC) et l'amélioration des techniques histologiques participent à une meilleure connaissance des MI. Une nouvelle classification a été proposée [1] avec un intérêt diagnostique mais aussi pronostique et thérapeutique [2]. Cliniquement les MI se présentent par un déficit moteur proximal subaigu avec myalgies.…”