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To investigate specific muscle pathologies of different kinds of myositis-specific autoantibodies (MSAs) in idiopathic inflammatory myopathy (IIM) patients. One hundred eleven Chinese patients from Xiangya Hospital, Central South University diagnosed with IIMs according to European Neuromuscular Centre (ENMC) criteria were included. Clinical manifestation, myositis-specific autoantibodies, and histologic findings were evaluated to explore the pattern of necrosis, regeneration, and perifascicular atrophy, inflammatory cells in IIM patients with different MSAs. Anti-SRP group has the lowest muscle strength scores, the highest creatine kinase levels, the most severe degree of necrosis and regeneration (1.90[0.80-3.95], 1.00[0.30-1.71]), and the lowest positive rate of MHC-I staining (35.71%). The anti-MDA5 group demonstrates the mildest pathological changes, with the fewest necrotic and regenerated muscle fibers (0.00[0.00-0.50], 0.00[0.00-0.00]), and the fewest inflammatory cell infiltration, and the highest muscle strength scores. The anti-NXP2 group has the most frequent inflammatory infiltrates, especially CD4+ T cells (31.14[15.00-39.00]). The patients with anti-NXP2 and the anti-TIF1γ antibodies show higher frequency of punched-out fibers (1.50[0.00-3.70], 0.00[0.00-1.00]) and perifascicular atrophy (71.43%, 55.56%). As for anti-synthetase antibodies (ASAs), the anti-Jo-1 group shows the most frequent rate of perifascicular necrosis (60%), while other ASA groups do not show perifascicular necrosis. Of the MSAs, the anti-SRP antibody leads to the most severe muscle involvement, while the anti-MAD5 antibody the mildest. The anti-NXP2 and anti-TIF1γ groups have the most typical "DM" pathology. Key Points • Anti-SRP group shows severe muscle pathology while anti-MDA5 group shows the mildest. • Anti-NXP2 group has the most frequent inflammatory infiltrates. Pouch-out fibers and perifascicular atrophy are more prevalent in anti-NXP2 and anti-TIF1γ groups. • Anti-Jo-1 group is often accompanied by perifascicular necrosis, while other anti-synthetase antibody groups are not.


Qiu Xu, Qiu-Xiang Li, Fang-Fang Bi, Hui-Qian Duan, Yue-Bei Luo, Huan Yang. The association between myositis-specific autoantibodies and muscle pathologies in idiopathic inflammatory myopathies. Clinical rheumatology. 2021 Feb;40(2):613-624

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PMID: 32671659

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