ILD in Idiopathic Inflammatory Myopathy: Next Step When Initial Immunosuppressive Treatment Fails
Interstitial lung disease is a recognised complication of idiopathic inflammatory myopathies (IIM). When a first-line immunosuppressive regimen has been used and the expected goals — improvement in lung function, reduction in ILD extent, and relief of breathlessness — have not been achieved, an escalation protocol applies.
Clinical scenario
Patient with idiopathic inflammatory myopathy and associated interstitial lung disease (IIM-ILD), currently on mono or combination immunosuppressive therapy, with insufficient response in lung function or symptoms.
Trigger for escalation — previous treatment failure
Initial immunosuppressive therapy — which may have included glucocorticoids, calcineurin inhibitor, rituximab, mycophenolate, or azathioprine, and in higher-risk settings combination regimens with cyclophosphamide, IVIG, or other agents — did not achieve the treatment goals of improvement in lung function, extent of ILD, or breathlessness. This failure of the first treatment line is the defined trigger for the protocol below.
Next-step approach (partial overview)
The approach at this stage centres on modifying the existing immunosuppressive regimen — either by adding a further agent or switching to a different class of immunosuppressive therapy. Where progressive pulmonary fibrosis is identified as a feature, a distinct additional therapeutic option may be incorporated alongside immunosuppression.
The complete agent selection criteria, sequencing, and clinical decision points are available in the full structured regimen below.
References
DOI: 10.1183/13993003.02533-2024
- We recommend using immunosuppressive treatment in patients with IIM-ILD (strong recommendation, very low certainty of evidence).
- If on mono or combination immunosuppressive treatment, add or switch to other CNI, rituximab, JAK inhibitors, cyclophosphamide, IVIG.
- If progressive pulmonary fibrosis, add nintedanib.
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