Autoimmune necrotizing myositis (AINM) is a recently described entity related to the deleterious effect of autoantibodies directed against an enzyme, HMG CoA reductase, most often in the context of prolonged statin exposure. Israeli researchers report the observation, at this unique stage, of a family of Bedouin origin in whom :
- a homozygous mutation in the gene coding for HMGCoA reductase resulted in a late-onset and rapidly progressive limb-girdle muscular dystrophy.
- The same researchers synthesised and purified the metabolite of HMG-CoA reductase, mevalonolactone, and were able to demonstrate its clinical efficacy, both in this family and in the mouse model of AINM.
This highly original work holds great promise for the treatment of AINM.
