Abstract
Idiopathic inflammatory myopathies (IIMs) are chronic autoimmune disorders involving multiple organs, such as the muscle, skin, lungs and joints. Although the detailed pathogenesis of IIMs remains unclear, immune mechanisms have long been recognised as of key importance. Immune cells contribute to many inflammatory processes via intercellular interactions and secretion of inflammatory factors, and many studies have demonstrated the participation of a variety of immune cells, such as T cells and B cells, in the development of IIMs. Here, we summarise the current knowledge regarding immune cells in IIM patients and discuss their potential roles in IIM pathogenesis.
Highlights
Idiopathic inflammatory myopathies (IIMs) comprise a group of heterogeneous rheumatic diseases predominantly characterised by progressive muscle weakness and reduced muscle endurance [1,2,3,4]
Lymphocytes are an important class of immune cells that are subdivided into T cells, B cells and natural killer (NK) cells
1, Bohan/Peter criteria; 2, criteria of the 119th ENMC international workshop; 3, Dalakas’ criteria; 4, Griggs’ criteria; 5, Lloyd’s criteria; 6, criteria of the 188th ENMC international workshop; 7, criteria not mentioned Th, T helper; IFN, Interferon; Tfh, T follicular helper; Tregs, regulatory T cells; T-bet, T-box expressed in T cells; Bregs, regulatory B cells; DCs, dendritic cells; Mesenchymal stem cells (MSCs), mesenchymal stem cells; Low-density granulocytes (LDGs), low-density granulocytes; PBMC, peripheral blood mononuclear cell; Killer cell lectin-like receptor G1 (KLRG1), killer cell lectin-like receptor G1; TCR, T cell receptor; CK, creatinine kinase; LDH, lactate dehydrogenase; MSAs, myositis-specific antibodies; ILD, interstitial lung disease; DM, dermatomyositis; PM, polymyositis; IBM, inclusion body myositis; IMNM, immune-mediated necrotising myopathy
Summary
Idiopathic inflammatory myopathies (IIMs) comprise a group of heterogeneous rheumatic diseases predominantly characterised by progressive muscle weakness and reduced muscle endurance [1,2,3,4]. Treatment with high doses of glucocorticoids combined with immunosuppressive agents has largely improved the prognosis of IIMs, yet a considerable proportion of patients respond poorly and develop sustained muscle weakness [7, 8]. Immune cells in idiopathic inflammatory myopathies [11]. Other innate immune cells mainly include dendritic cells and macrophages, which initiate and support local immune responses by processing and presenting antigens [13].
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