Abstract

Challenges in timing of lung transplantation (LTx) in patients on antifibrotic treatment were recently acknowledged in 2021 ISHLT consensus document.

Aim:To retrospectively analyze, if idiopathic pulmonary fibrosis (IPF) patients under 65 years at diagnosis, who fulfill criteria for LTx listing, profit from antifibrotic treatment or LTx (assessed with survival).

Cohort A included 67 IPF patients, who underwent LTx for IPF in 2000-2021. Cohort B included subjects from EMPIRE registry diagnosed IPF in 1999-2021 meeting criteria for LuTx listing: under 65 years, BMI ? 30, nonsmokers or exsmokers, drop in forced vital capacity (FVC) of more than 10 % and/or drop in lung diffusing capacity for carbon monoxide (DLco) of for more than 15 % in previous six months and absence of LTx absolute contraindications (Figure 1). LTx listing criteria were enriched with dyspnea grade NYHA III at the time of diagnosis, as historic patients were usually diagnosed very lately. Only those never listed for LTx were put in cohort B.

Median survival since diagnosis of transplanted patients was longer then in those, who were not transplanted (158 vs 95 months). Difference was not statistically significant (HR 0.66, 95% CI 0.42-1.05; p=0.17). Survival of transplanted patients adjusted to age was significantly higher than in not transplanted group (HR 0.53, 95% CI 0.32-0.91; p=0.02). Patients in cohort B on antifibrotic treatment had similar survival as patients in cohort A, adjusted to age at the time of diagnosis (HR 0.75, 95% CI 0.41-1.37; p=0.35).

LTx in carefully selected IPF patients adds years of life to them and prolongs patient´s survival compared to antifibrotic treatment without LTx.