Abstract
Immune mediated necrotizing myopathies (IMNM) are part of the idiopathic inflammatory myopathies, precisely defined by international consensus recently. The main characteristics include proximal muscle weakness, substantially increased serum CK levels and detection of the myositis-specific auto-antibodies -SPR54 or -HMGCR in many patients, while 1/3 of IMNM patients do not present those. Notably, both antibodies target proteins of the endoplasmic reticulum (ER)/sarcoplasmic reticulum (SR) and are involved in protein processing.
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