Polymyositis
ICD-10 M33.2 · ICD-11 4A41.1

Treatment of Polymyositis in Adults with Rapidly Progressive Interstitial Lung Disease

In adults aged 18 years or older, polymyositis can present alongside rapidly progressive interstitial lung disease (RP-ILD) — a severe complication that demands prompt and structured management. This protocol addresses that specific clinical situation.

Adults (≥18 years) with polymyositis and concurrent rapidly progressive interstitial lung disease (RP-ILD) — a comorbidity that directly determines both the urgency and composition of the therapeutic approach.

Induction therapy with high-dose steroids is central to the initial approach, with specific immunosuppressive agents considered alongside — particularly in the setting of RP-ILD. Additional agents may be introduced early, potentially as part of the induction regimen itself.

Full agent selection, dosing, sequencing, and monitoring guidance is available in the structured protocol.

References

DOI: 10.1093/rheumatology/keac115

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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