- Design
- Multicentre longitudinal registry cohort
- Population
- 158 patients with autoimmune-associated progressive pulmonary fibrosis
- Primary outcome
- Lung-related mortality
- Effect
- SHR 0.35 (95% CI 0.19 to 0.66) with immunomodulatory therapy
A Madrid registry followed 158 patients with interstitial lung disease due to autoimmune disease who met criteria for progressive pulmonary fibrosis, from 2005 to 2024.
Of 47 deaths, 79% were from lung disease, a rate of 6.2 per 100 patient-years. Patients on immunomodulatory therapy had lower lung-related mortality (subhazard ratio 0.35, 95% CI 0.19 to 0.66), with mean survival of over 10 years against 4.8 years untreated. DLCO below 60% and coexisting emphysema were independent risk factors.
Treatment was not randomised, and patients well enough to treat may have done better anyway. But the size of the association supports continuing immunomodulation once fibrosis progresses, rather than treating progression as a reason to stop.
- Monitor DLCO and lung function regularly in autoimmune interstitial lung disease
- Consider continuing or escalating immunomodulation when fibrosis progresses
- Look for emphysema on CT, which marked higher risk
- Manage these patients jointly with respiratory physicians
Why it matters
Progression can tempt clinicians to see immunosuppression as failed; these data argue otherwise.
Don't overread it
Observational registry data — it cannot prove immunomodulation caused the survival difference.
The statistics, in plain English
A subhazard ratio of 0.35 means roughly a third of the risk of lung-related death among treated patients. Because treatment was chosen by clinicians, some of that difference may reflect who was selected.
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