Risk score including family history for patients with interstitial lung disease waiting for lung transplantation

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Background: Familial pulmonary fibrosis (FPF) is a potential predictor of mortality in patients with idiopathic pulmonary fibrosis and other fibrotic interstitial lung disease (ILD). Aims and Objectives: To investigate the epidemiology and clinical relevance of FPF in patients with fILD who were registered for deceased-donor lung transplantation (DDLT). Methods: A retrospective observational study at a LT center. FPF was diagnosed when at least one blood relative first- or second-degree family members of the patient had any fibrotic ILD on the self-reported family history. To develop the prognostic model, the whole cohort was divided into the discovery and the validation cohorts. Results: Of the 260 patients with fibrotic ILD who were registered for DDLT from Kyoto University Hospital between 2008 and 2022, 49 (18.9%) fulfilled the diagnosis of FPF. The most common diagnosis of FPF was idiopathic pulmonary fibrosis (36.7%). Patients with FPF had a higher risk for death or living-donor LT on the waitlist than those with sporadic ILD (log-rank, P=0.02). FPF was an independent predictor for the waitlist death or living-donor LT (hazard ratio = 1.72 [1.03-2.89]) as well as percentage predicted diffusion capacity (%DLCO). The risk score (FD score) was developed in the discovery cohort (n=182) as familial pulmonary fibrosis plus %DLCO < 22% or unavailable DLCO. FD score ≥ 1 predicted death or living-donor LT on the waitlist in both discovery and validation cohorts. Conclusions: FPF is relatively common in patients with fibrotic ILD on the waitlist for LT and predictive for death or living-donor LT on the waitlist independent from the physiological impairment.

Ce résumé expose les affirmations des auteurs. BNTIC ne l’interprète pas comme une validation indépendante des résultats.