Treatment of Interstitial Lung Disease in Idiopathic Inflammatory Myopathies

Clinical scenario

This protocol addresses patients with idiopathic inflammatory myopathies (IIM) complicated by interstitial lung disease (IIM-ILD) — a serious pulmonary manifestation that warrants prompt, structured management guided by strong clinical evidence.

Treatment approach

Immunosuppressive treatment forms the basis of management in IIM-ILD. The specific regimen — including which agents are used, how they are combined, and how the approach is adapted to disease severity and rate of progression — is detailed in the full protocol.

Full agent selection, combination strategies, and sequencing are available in the complete structured regimen below.

Treatment goals

The primary aims are improvement of lung function, reduction of ILD extent on imaging, and relief of breathlessness.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1183/13993003.02533-2024

We recommend using immunosuppressive treatment in patients with IIM-ILD (strong recommendation, very low certainty of evidence).

We suggest using combination therapy with immunosuppressants including glucocorticoids in patients with IIM-ILD (conditional recommendation, very low certainty of evidence).

We recommend using immunosuppressive therapy (including glucocorticoids) for patients with IIM-ILD, as it has been shown to improve survival, lung function, ILD extent, breathlessness and extrapulmonary organ involvement, with an acceptable safety profile.

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