Prognostic significance of 1-year pulmonary function changes in inflammatory myopathy-related interstitial lung disease
Authors' conclusion
Even modest FVC decline (≥5 %) over one year predicts mortality and transplant in IIM-ILD, while stabilization or improvement in FVC associates with improved survival
Study context
- Population
- Adults with IIM-ILD (2017 EULAR/ACR criteria; ILD on high-resolution CT) with baseline and follow-up pulmonary function test (PFTs) 6–18 months apart, single centre; mean age 50.5 years, 63% female, 73% ASySmean 6.3-year follow-up
- Participants
- 149 adults
- Exposure
- 1-year forced vital capacity (FVC) decline ≥5% vs no such decline
- Outcome
- 10-year mortality and lung transplantation
- Follow-up
- 10 years
Methodological detailssingle-center, multivariable Cox regression, Kaplan-Meier analysis
- Single-center
- Multivariable Cox regression
- Kaplan-Meier analysis
Main finding
As reported in the abstract
10-year mortality
Hazard ratio (CI)P value
FVC decline ≥5%absolute FVC decline ≥5%; multivariate
2.78(1.27–6.09)
P = 0.01FVC decline ≥5%relative FVC decline ≥5%; multivariate
2.37(1.11–5.05)
P = 0.025Stable or improved FVC
0.38(0.18–0.82)
P = 0.01Observational data: associations, not effects.
Further findings
| Outcome | P value |
|---|---|
| Survival by FVC decline ≥5% (Kaplan-Meier)Worse survival | P = 0.028 |
Deaths and transplantation over follow-up · mean 6.3 years
- Died
- 41 (27.5 %)
- Underwent transplantation
- 6 (4.0 %)
DLCO decline and survival
- DLCO decline was not associated with survival
Survival by autoantibody subtype or HRCT pattern
- Survival did not differ by autoantibody subtype or HRCT pattern
Original publication
Prognostic significance of 1-year pulmonary function changes in inflammatory myopathy-related interstitial lung disease. Semin Arthritis Rheum. Published 11 March 2026. doi:10.
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