Abstract
Introduction: Familial interstitial pneumonia (FIP) is defined as the presence of any fibrotic interstitial lung disease (ILD) in at least two blood relatives' first or second-degree family members. Data suggests that FIP survival is similar to patients with idiopathic pulmonary fibrosis (IPF), regardless of the aetiology. Aims: Compare survival between patients with FIP regarding the phenotype.
Methods: This was a multicentric study. The diagnosis was based on international guidelines. Continuous variables were presented as mean and standard deviation or median, minimum and maximum when indicated—categorical variables like proportions. For survival analysis, the outcome considered was death from any cause. Survival data were presented using the Kaplan-Meier estimator and calculated using the Log Rank test. The study was approved by the Ethics and Research Committee of the Federal University of Minas Gerais (number: 44843215.5.0000.5149). Results: 119 participants were included. The mean age was 66.9 ± 8.92 years, with a predominance of women (56.3%). The most frequent phenotype was IPF (40.3%), followed by hypersensitivity pneumonia (27.7%), undefined ILD (20.2%) and connective tissue disease (2.5%). Median survival was 66 months (95% CI: 48.3-83.7 months). No significant difference was observed when comparing survival between patients with FIP according to diagnosis. The presence of honeycombing was the only factor associated with worse survival (p = 0.009).
Conclusions: FIP survival is poor, regardless of phenotype. Therefore, the possibility of a family history of fibrosis should be strongly questioned when evaluating patients with ILD.
Footnotes
Cite this article as: European Respiratory Journal 2023; 62: Suppl. 67, PA1717.
This abstract was presented at the 2023 ERS International Congress, in session “Inflammatory endotyping: the macrophage across disease areas”.
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 2023