Decreased dystrophin expression and elevated dystrophin-targeting miRNAs in anti-HMGCR immune-mediated necrotizing myopathy.

The possibility that inflammatory changes affect dystrophin expression has been proposed in a recent myositis mouse model study where miRNA-mediated dystrophin deficiency correlated with myositis severity [[3]]. To explore whether decreased dystrophin may be a feature of dystrophic IMNM, we performed dystrophin immunofluorescence on three control cases of adult anti-HMGCR IMNM and noted reductions in dystrophin staining similar to those observed in our patient (Supplementary Fig. The role of dystrophin-targeting miRNAs in IMNM is currently elusive but these first data in patient biopsies raise the possibility of a new potential pathophysiological mechanism and warrant further exploration. [Extracted from the article]