The association between myositis-specific autoantibodies and muscle pathologies in idiopathic inflammatory myopathies.

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.