Pulmonology · Rheumatology · 2 h ago
Fibrotic morphology explains survival differences in IPAF cohorts
A multicentre retrospective cohort study applied IPAF criteria to six US and UK idiopathic interstitial pneumonia cohorts, including 5,042 people. The apparent transplant-free survival advantage associated with IPAF classification was no longer statistically significant after adjustment for fibrotic morphology.
- 649 of 5,042 patients met IPAF criteria.
- Survival advantage attenuated after adjustment for fibrotic morphology.
- Clinical-domain features were informative in non-UIP morphology.
Researchers retrospectively applied the 2015 Interstitial Pneumonia with Autoimmune Features (IPAF) criteria to six idiopathic interstitial pneumonia cohorts in the US and UK. Among 5,042 individuals, 649 (12.9%) met IPAF criteria. The team assessed fibrotic morphology using imaging and, in separate analyses, histopathology, and compared three-year transplant-free survival before and after adjustment. Outcomes were also benchmarked against a connective tissue disease-associated interstitial lung disease reference cohort of 1,566 people.
Before adjustment for imaging-based morphology, IPAF classification was associated with better transplant-free survival (hazard ratio 0.79; 95% CI 0.68–0.92; p=0.003). After adjustment, the association was attenuated and not statistically significant (HR 0.91; 95% CI 0.77–1.07; p=0.25); findings were similar using histopathological morphology. IPAF classification was not prognostic within common morphology groups. However, clinical-domain features identified a group with a CTD-ILD-like natural history among patients with non-UIP morphology.
The findings suggest that differences in fibrotic morphology, rather than IPAF classification itself, may account for the observed survival advantage in these cohorts. The retrospective design and cohort-based analyses limit conclusions about individual prognosis or clinical decisions; the results do not establish a change in management.
In practice
For clinicians assessing patients with interstitial pneumonia and autoimmune features, this retrospective multicentre analysis indicates that IPAF classification alone did not predict transplant-free survival independently of fibrotic morphology. The apparent survival advantage versus idiopathic interstitial pneumonia was no longer statistically significant after adjustment, and classification was not prognostic within common morphology groups. Clinical-domain features were associated with a CTD-ILD-like natural history in non-UIP morphology, but these cohort findings do not establish treatment or monitoring changes and need cautious interpretation.
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European Respiratory Journal: Fibrotic Morphology and Survival in Interstitial Pneumonia with Autoimmune Features ↗This is an automated AI-condensed summary that has not yet been reviewed by an editor. Always consult the full item at the original source.