Interstitial lung disease with and without progressive fibrosing phenotype in patients with idiopathic inflammatory myopathies: data from a large multicentric cohort
Study context
- Population
- Patients with IIM from a multicentric prospective cohort125 with interstitial lung disease (ILD)
- Participants
- 253 patients
- Outcome
- Occurrence of PF-ILD (2022 American Thoracic Society (ATS)/European Respiratory Society (ERS)/Japanese Respiratory Society (JRS)/ALAT guidelines) in IIM and factors predicting it
- Follow-up
- 1-year follow-up
Methodological detailsmulticentre, retrospective evaluation of a prospective cohort, univariate and multivariate analyses
- Multicentre
- Retrospective evaluation of a prospective cohort
- Univariate and multivariate analyses
Main finding
As reported in the abstract
PF-ILD in IIM-ILD
Progressive fibrosing ILD (PF-ILD) occurrence among IIM-ILD
18%
Observational data: associations, not effects.
Further findings
ILD in IIM
- ILD among patients with IIM
- 125 (49%)
- ILD at IIM diagnosis
- 99 (78%)
- ILD during follow-up
- 26 (22%)
- ILD during follow-up within 5 years
- 21/26
- Independently associated with ILD (multivariate): anti-Jo-1, anti-MDA5, anti-Ro52, high manual muscle test score, mechanic's hands, RP
Predominant high-resolution CT (HRCT) pattern in IIM-ILD
- Non-specific interstitial pneumonia (NSIP)
- 50%
- Usual interstitial pneumonia (UIP)
- 28%
- Organising pneumonia (OP)
- 22%
Original publication
Interstitial lung disease with and without progressive fibrosing phenotype in patients with idiopathic inflammatory myopathies: data from a large multicentric cohort. RMD Open. Published 4 August 2023. doi:10.
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