OBJECTIVES To evaluate serum levels of 25(OH) vitamin D in patients with idiopathic inflammatory myopathies (IIM) (polymyositis (PM), dermatomyosistis (DM), inclusion body myositis (IBM) and juvenile DM (JDM)) and to compare these with healthy controls. METHODS Serum samples from 149 patients with IIM and 290 healthy controls matched for gender and the month of blood sampling were analysed fo...

Idiopathic inflammatory myopathies are a group of rare muscular diseases that are characterized by acute, subacute or chronic proximal and symmetric muscle weakness, muscle fiber necrosis and infiltration of inflammatory cells, particularly activated CD8+ cytotoxic T cells and phagocytes. 3-n-butylphthalide (NBP) protects mitochondria and reduces the inflammatory response in multiple disease mo...

The review provides an update on the diagnosis of the main subtypes of inflammatory myopathies including dermatomyositis (DM), polymyositis (PM), necrotizing autoimmune myositis (NAM) and sporadic inclusion body myositis (sIBM). The fundamental aspects on muscle pathology and the unique pathomechanisms of each subset are outlined and the diagnostic dilemmas concerning the distinction of PM from...

Background: Dermatomyositis (DM) is a rare autoimmune muscle disease characterized by an atrophy and type-I interferon signature in perifascicular fibers. We recently showed that mitochondrial dysfunction also characteristic of this participates to both decrease exercise capacity maintenance inflammation (1). Pathophysiological mechanisms underlying these characteristics are unknown. Objectives...

OBJECTIVE Serum cytokines play an important role in the pathogenesis of myositis by initiating and perpetuating various cellular and humoral autoimmune processes. The aim of the present study was to describe a broad spectrum of T- and B-cell cytokines, growth factors and chemokines in patients with idiopathic inflammatory myopathies (IIMs) and healthy individuals. METHODS A protein array syst...

clostridial myositis, considered to be rare in pet animals, is an acutely fatal toxaemic condition. some species of clostridia are responsible for necrotic myositis. a 2-year-old male german shepherd dog was admitted with non-weight bearing lameness and massive swelling of the left hind limb. clostridium perfringens type a with alpha toxin was diagnosed as a pathogenic agent. based on the histo...

Abrogation of ICOS/ICOS ligand (ICOSL) costimulation prevents the onset of diabetes in the non-obese diabetic (NOD) mouse but, remarkably, yields to the development of a spontaneous autoimmune neuromyopathy. At the pathological level, ICOSL-/- NOD mice show stronger protection from insulitis than their ICOS-/- counterparts. Also, the ICOSL-/- NOD model carries a limited C57BL/6 region containin...

INTRODUCTION We developed an experimental autoimmune myositis (EAM) mouse model of polymyositis where we outlined the role of regulatory T (Treg) cells. Rapamycin, this immunosuppressant drug used to prevent rejection in organ transplantation, is known to spare Treg. Our aim was to test the efficacy of rapamycin in vivo in this EAM model and to investigate the effects of the drug on different i...

The juvenile idiopathic inflammatory myopathies (JIIM) are systemic autoimmune diseases characterized by skeletal muscle weakness, characteristic rashes, and other systemic features. Although juvenile dermatomyositis (JDM), the most common form of JIIM, has been well studied, the other major clinical subgroups of JIIM, including juvenile polymyositis (JPM) and juvenile myositis overlapping with...

BACKGROUND Antibodies against Ro-52 have been described in patients with a broad spectrum of autoimmune disease, most commonly in association with anti-Ro-60 in systemic lupus erythematosus and Sjogrens syndrome. However, in inflammatory myositis anti-Ro-52 is frequently present without anti-Ro-60 and is closely linked to the presence of aminoacyl-tRNA synthetase (aats) antibodies. To date ther...