In vivo pathogenicity of IgG from patients with anti-SRP or anti-HMGCR autoantibodies in immune-mediated necrotising myopathy.
Cécile BerguaHélène ChiavelliYves AllenbachLouiza Arouche-DelapercheChristophe ArnoultGwladys BourdenetLaetitia JeanRachid ZoubairiNicolas GueroutMichael MahlerOlivier BenvenisteLaurent DrouotOlivier BoyerPublished in: Annals of the rheumatic diseases (2018)
This study demonstrates that patient-derived anti-SRP+ and anti-HMGCR+ IgG are pathogenic towards muscle in vivo through a complement-mediated mechanism, definitively establishing the autoimmune character of IMNM. These data support the use of plasma exchanges and argue for evaluating complement-targeting therapies in IMNM.