Immune mediated necrotizing myopathy (IMNM), a recently recognized but a common entity among the heterogenous group of inflammatory myopathies, has been classified by the ENMC Immune-Mediated Necrotizing Myopathies Working Group into 1) anti-signal recognition particle (anti-SRP) positive IMNM or anti-SRP myopathy, 2) anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) positive IMNM or anti-HMGCR myopathy, and 3) antibody negative IMNM using integrated clinical, serological, and pathological criteria [1].

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