Abstract
Muscular dystrophies and inflammatory myopathies are heterogeneous muscular disorders characterized by progressive muscle weakness and mass loss. Despite the high variability of etiology, inflammation and involvement of both innate and adaptive immune response are shared features. The best understood immune mechanisms involved in these pathologies include complement cascade activation, auto-antibodies directed against muscular proteins or de-novo expressed antigens in myofibers, MHC-I overexpression in myofibers, and lymphocytes-mediated cytotoxicity. Intravenous immunoglobulins (IVIGs) administration could represent a suitable immunomodulator with this respect. Here we focus on mechanisms of action of immunoglobulins in muscular dystrophies and inflammatory myopathies highlighting results of IVIGs from pre-clinical and case reports evidences.
Highlights
Growing evidences support the role of the immune system in different pathological conditions of the skeletal muscle
Immune cell infiltrate following muscle injury contributes to the pathology of various muscular dystrophies (MDs), whereas autoimmune responses specific for defined or yet undefined muscle antigens are suggested as the cause of some idiopathic inflammatory myopathies (IIMs)
The most frequently occurring MDs involve damage to the muscle fiber membrane, which can lead to the release of Danger Associated Molecular Patterns (DAMPs) in Immunoglobulins in Muscular Diseases the extracellular environment which interact with toll-like receptors (TLR) of antigen-presenting cells (APCs) such as dendritic cells (DCs) and macrophages, triggering an innate immune response, with recruitment of inflammasomes and activation of the NF-kB signaling pathway
Summary
Stem Cell Laboratory, Department of Pathophysiology and Transplantation, University of Milan, Dino Ferrari Center, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milan, Italy. Reviewed by: Renato Mantegazza, Fondazione Istituto Neurologio Carlo Besta (IRCCS), Italy. Muscular dystrophies and inflammatory myopathies are heterogeneous muscular disorders characterized by progressive muscle weakness and mass loss. Despite the high variability of etiology, inflammation and involvement of both innate and adaptive immune response are shared features. The best understood immune mechanisms involved in these pathologies include complement cascade activation, auto-antibodies directed against muscular proteins or de-novo expressed antigens in myofibers, MHC-I overexpression in myofibers, and lymphocytes-mediated cytotoxicity. Intravenous immunoglobulins (IVIGs) administration could represent a suitable immunomodulator with this respect. We focus on mechanisms of action of immunoglobulins in muscular dystrophies and inflammatory myopathies highlighting results of IVIGs from pre-clinical and case reports evidences
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