Inflammatory myopathies

Factors associated with interstitial lung disease among patients with idiopathic inflammatory myopathies

Rheumatology (Oxford)Published Cross-sectional study

Authors' conclusion

This sample of IIM patients showed autoantibodies to be the strongest predictive or protective factors for ILD, yet the full range of associations of IIM-ILD remain undefined.

Study context

Population
Adult and juvenile DM, PM and overlap myositis patients enrolled in studies at the National Institutes of Health262 adult, 188 juvenile
Participants
450 participants
Exposure
Idiopathic inflammatory myopathies (IIM) patients with ILD vs without ILD
  • ILDn = 162
  • No ILDn = 288reference
Outcome
Predictors of IIM-ILD (clinical, autoantibody, HLA and SNP data)
Methodological detailsMeta-ANalysis of Transethnic Associations (MANTRA), logistic regression, classification and regression tree (CART) and gradient boosting machine learning
  • MANTRA
  • Logistic regression
  • CART and gradient boosting machine learning

Main finding

As reported in the abstract

Predictors of IIM-ILD
  • Smoking status, older age, African American heritage and certain HLA genes associated with IIM-ILD
  • Anti-synthetase, myositis-associated and anti-MDA5 autoantibodies: strongest risk associations, increased odds of ILD by up to 20-fold
  • Anti-signal recognition particle, anti-TIF1 (P155/140) and anti-NXP2 autoantibodies: strongest protective effects, decreased odds of ILD by up to 40%
  • Effects of some HLA allele groups and idiopathic pulmonary fibrosis (IPF) single nucleotide polymorphism (SNPs) on ILD inconsistent and weaker

Observational data: associations, not effects.

Original publication

Factors associated with interstitial lung disease among patients with idiopathic inflammatory myopathies. Rheumatology (Oxford). Published 29 November 2025. doi:10.1093/rheumatology/keaf625

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