Classification and management of adult inflammatory myopathies

Roles of aminoacyl-tRNA synthetases in immune regulation and immune diseases

“…Of note, murine Jo-1 induced autoreactive B and T cells targeting its own epitopes, and the epitope spreading occurred uniformly 8 weeks after the single immunization, suggesting that autoantibody Jo-1 was able to drive a sustained immune response. When PBMCs and bronchoalveolar lavage fluid (BALF) cells from ASSD patients were stimulated with HisRS or a HisRS-derived peptide (HisRS [11][12][13][14][15][16][17][18][19][20][21][22][23] ), the expression of CD40L in CD4 + T cells from the corresponding compartments was upregulated 78 . Compared with PBMCs, BALF CD4 + T cells showed a remarkable Th1 phenotype after stimulation, such as the production of more IFN-γ and IL-2, indicating the presence of HisRS-specific CD4 + T cells in the blood and lung of patients with ASSD.…”

“…It has been found that the pathogenic variants in lysyl-tRNA synthetase (KARS) cause a progressive leukodystrophy, and the patients exhibit severe phenotypes including developmental delay or arrest, deafness, and immunological abnormalities 14 . Interestingly, diverse autoantibodies against ARSs were found in antisynthetase syndrome (ASSD), suggesting that ARSs are likely to be involved in the development and progression of autoimmune diseases [15][16][17] . Strikingly, full-length tryptophanyl-tRNA synthetase (WRS) induced immune cells to release inflammatory cytokines and type I interferons (IFNs), thereby playing a role in antiviral immunity 18 .…”

Neutrophil dysregulation is pathogenic in idiopathic inflammatory myopathies

“…Idiopathic inflammatory myopathies (IIM) are heterogeneous autoimmune conditions that affect adults and children and are characterized by muscle inflammation and frequent development of extramuscular manifestations in lung, skin, and joints (1). IIM include several clinicopathologic subtypes: dermatomyositis (DM), polymyositis (PM), immune-mediated necrotizing myopathy, sporadic inclusion body myositis, and myositis in association with other autoimmune conditions.…”

Rituximab and other anti-B-cell agents in immune-mediated inflammatory rheumatic diseases

“…Заболевания Показания РА [14,15] АНЦА-СВ [16,17] СКВ [18][19][20] СШ [21,22] ИВМ [23,24] Возможно назначение в качестве первого ГИБП у высоко-серопозитивных по АЦЦП и IgM РФ Другие показания для назначения в качестве первого ГИБП ционной и/или поддерживающей терапии у пациентов с АНЦА-СВ и недостаточной эффективностью ГК и иммуносупрессивных препаратов, в первую очередь циклофосфамида (ЦФ) [31,32]. В многоцентровое рандомизированное контролируемое исследование RAVE были включены пациенты с рецидивирующим или впервые диагностированным АНЦА-СВ [33].…”