Adult dermatomyositis presenting with rapidly progressive interstitial lung disease (RP-ILD) is a high-acuity clinical scenario that demands a structured, protocol-driven approach from the outset.
Clinical ScenarioThis protocol addresses adult dermatomyositis in the specific setting of rapidly progressive interstitial lung disease — a presentation that substantially shapes both the urgency and the composition of the treatment strategy.
Management is built around an induction strategy using high-dose corticosteroids, with further immunosuppressive agents incorporated early in the course — the specific agents, their combination, and the criteria for escalation are covered in the full protocol.
DOI: 10.1093/rheumatology/keac115
In the treatment of rapidly progressive interstitial lung disease (RP-ILD):
Induction therapy with high dose steroids is to be considered.
The use of ciclosporin or tacrolimus, alongside steroids, is to be considered in patients with RP-ILD.
Cyclophosphamide or rituximab therapy is to be considered early, potentially as part of the induction regimen.
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