Systemic sclerosis & Raynaud's
Survival and immunomodulatory therapy in autoimmune disease-associated progressive pulmonary fibrosis: a multicenter registry (NEREA)
Main finding · Specific-cause mortality related to ILDResult — go to the resultSHR for ILD-related mortality 0.35 95% CI 0.19–0.66Authors' conclusion
The study shows a considerable mortality directly attributable to ILD. Furthermore, it demonstrates a survival benefit in patients receiving immunomodulatory therapy regardless other factors.
Study context
- Population
- Autoimmune-ILD patients with progressive pulmonary fibrosis enrolled in the NEREA registry, Madrid, 2005 to 2024
- Participants
- 158 patients
- Exposure
- Immunomodulatory therapy vs no immunomodulatory therapy
- Outcome
- Specific-cause mortality related to ILD
Methodological detailsregistry (NEREA), Fine-Gray competing risk regression models
- Registry (NEREA)
- Fine-Gray competing risk regression models
Main finding
As reported in the abstract
Subhazard ratio (SHR) for interstitial lung disease (ILD)-related mortality
0.35
95% CI 0.19–0.66
P = 0.001
IMMUNOMODULATORY THERAPYvs UNTREATED
SHR for ILD-related mortality 0.35 (95% CI 0.19–0.66); P = 0.001
- ILD-related mortality rate (MR), per 100 patients-year
- 6.2 [4.5-8.5]
- Deaths documented
- 47
- Deaths attributed to ILD-related causes
- 79%
Observational data: associations, not effects.
Further findings
| Outcome | IMMUNOMODULATORY THERAPY | UNTREATED |
|---|---|---|
| Mean survival | mean over 10 years | mean 4.8 years |
Immunomodulatory therapy use
- On immunomodulatory therapy at PPF baseline
- 70.3%
- During follow-up
- 81%
Other risk factors for ILD-related mortality
- DLCO% (<60) independently increased the risk of ILD-related mortality
- Emphysema independently increased the risk of ILD-related mortality
Original publication
Survival and immunomodulatory therapy in autoimmune disease-associated progressive pulmonary fibrosis: a multicenter registry (NEREA). Rheumatology (Oxford). Published 28 October 2026. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →