Abstract
Background: Real-life data on suspected familial fibrosis, defined as the occurrence of the disease in a patient younger than 50 and/or having at least one first-degree relative affected by pulmonary fibrosis remain scarce.
Methods: The Belgian and Luxembourg IPF registry (PROOF-Next) is a multicentric prospective longitudinal and observational study set in Belgium and Luxembourg. We compared characteristics and clinical course of patients with suspected familial (f)IPF and sporadic (s)IPF.
Results: We included 607 patients in the analysis, of whom 74 (12%) fulfilled criteria for suspected fIPF. They were significantly younger than sIPF (median age (range) 65 (43-87), VS 72 (51-98), P=0.0001). Male gender proportion and smoking status did not differ between groups, but the number of pack-year was lower in suspected fIPF (17.5 VS 25, P=0.02).
74% of fIPF and 68% of sIPF were treated with antifibrotics and the proportion of patients treated with nintedanib was significantly higher in the suspected fIPF group (26% VS 16%, P=0.02).
Baseline pulmonary function tests were similar in both groups, as well as median time before progression and transplant-free survival.
Conclusion: Although younger and exposed to less tobacco, patients with suspected fIPF show an equally aggressive progression as observed in sIPF patients. These results warrant early referral of fIPF patients to expert centres for optimal management.
Footnotes
Cite this article as: European Respiratory Journal 2021; 58: Suppl. 65, OA4328.
This abstract was presented at the 2021 ERS International Congress, in session “Prediction of exacerbations in patients with COPD”.
This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).
- Copyright ©the authors 2021