The evidence for the use of rituximab, abatacept and complement inhibitors in IIM is largely uncertain. Abatacept may improve achievement of IMACS DOI at three or six months, although the evidence is of low certainty. More research is needed to investigate the benefits and harms of targeted immunosuppressive and immunomodulatory therapies in IIM. Ideally, studies should be sufficiently powered to ensure detection of effects in subgroups (e.g. IMNM, DM, ASS). Because of the rarity of IIM, international collaborative efforts should be encouraged to embark on multicentre trials.