This protocol applies to adult dermatomyositis that has not responded adequately to initial corticosteroid-based treatment combined with disease-modifying therapy. When the expected level of disease control is not reached, a structured escalation approach is indicated.
Standard first-line management combines a corticosteroid — oral prednisolone (with dose tapering) or intravenous methylprednisolone when there are concerns about gastrointestinal absorption — with a DMARD: methotrexate, azathioprine, tacrolimus, ciclosporin, or mycophenolate mofetil. This is supported by a supervised exercise programme and sun protection measures.
Escalation to this next-line protocol is triggered when substantial improvement in disease activity across all domains has not been achieved, typically assessed at around 6 weeks after treatment initiation.
For severe and/or refractory muscle inflammation that has not responded to initial therapy, this protocol involves specific intravenous agents — the selection among available options depends on individual clinical factors detailed in the full regimen.
The complete protocol specifies which agent to consider first, how to choose between options, and the relevant monitoring requirements.
DOI: 10.1093/rheumatology/keac115
Intravenous immunoglobulin should be considered as a treatment of severe and/or refractory muscle inflammation.
Rituximab is to be considered as a treatment option for refractory myositis.
Cyclophosphamide should be considered as a treatment option for severe and/or refractory IIM.
CYC is usually administered by i.v. infusion, reducing risk of leucopenia, haemorrhagic cystitis, and gonadal toxicity.
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