Purpose of review In the past decade, immune-mediated necrotizing myopathies have emerged as a separate entity in the heterogenous group of autoimmune myopathies. This group is characterized by clinical manifestations restricted to the muscle tissue, and until recently, the definition was based on muscular pathological features. Recent findings It was shown that they are the most severe autoimmune myopathies in term of muscle damages. They have been associated with two myositis-specific antibodies: either anti-signal recognition particle (anti-SRP) or anti-hydroxy-3-methylglutaryl-CoA reductase (anti-HMGCR) antibodies. These two antibodies are now considered as immune-mediated necrotizing myopathy (IMNM) diagnostic criteria. Each antibody delineates a homogenous subgroup of IMNM patients in terms of severity and IMNM without myositisspecific antibodies have a high risk of malignancy. In addition, pathological observations as well as in-vitro experiments suggest the pathogenic role of anti-SRP and anti-HMGCR antibodies. Summary IMNM are muscle-specific autoimmune diseases associated with a severe weakness and a risk poor muscle strength recovery. Anti-SRP and anti-HMGCR antibodies are specifically associated with this condition and are crucial for the diagnosis and the prognosis. The muscle biopsy remains necessary for IMNM diagnosis in absence of myositis-specific antibodies.