Cano Jiménez, EstebanVillar Gómez, AnaVelez Segovia, EduardoAburto Barrenechea, MyriamSellarés, JacoboFrancesqui, JoelPortillo, KarinaSolis Solis, Alan JhuniorAcosta Fernández, OrlandoLlanos González, Ana BelénBordas Martínez, JaumeCabrera César, EvaBalcells Vilarnau, Eva, 1967-Castillo, DiegoReyes Pardessus, AnaGonzález Fernández, CoralGarcía Moyano, MartaUrrutia Gajate, AmaiaBlanco Hortas, AndrésMolina-Molina, María2024-10-142024-10-142024Cano-Jiménez E, Villar Gómez A, Velez Segovia E, Aburto Barrenechea M, Sellarés Torres J, Francesqui J, et al. Prognostic factors of progressive fibrotic hypersensitivity pneumonitis: a large, retrospective, multicentre, observational cohort study. ERJ Open Res. 2024 Feb 26;10(1):00405-2023. DOI: 10.1183/23120541.00405-20232312-0541http://hdl.handle.net/10230/61379Background: Fibrotic hypersensitivity pneumonitis (fHP) is an immune-mediated interstitial lung disease caused by sensitisation to chronic allergen inhalation. This study aimed to determine prognostic indicators of progression and mortality in fHP. Methods: This was a retrospective, multicentre, observational, cross-sectional cohort study of consecutive patients diagnosed with fHP from 1 January 2012 to 31 December 2021. Multivariate Cox regression analyses were used to calculate hazard ratios (HRs) with 95% confidence intervals for predictors of progression and survival. Results: A total of 403 patients were diagnosed with fHP: median (interquartile range) age 66.5 (14.0) years, 51.9% females and 55.1% never-smokers. The cause of fHP was mainly fungal (39.7%) or avian (41.4%). Lung biopsy was performed in 269 cases (66.7%). In the whole cohort the variables that were related to mortality or lung transplant were older age (HR 1.08; p<0.001), percentage predicted forced vital capacity (HR 0.96; p=0.001), lymphocytosis in bronchoalveolar lavage (BAL) (HR 0.93; p=0.001), presence of acute exacerbation during follow-up (HR 3.04; p=0.001) and GAP (gender, age and lung physiology) index (HR 1.96; p<0.01). In the group of biopsied patients, the presence of fibroblastic foci at biopsy (HR 8.39; p<0.001) stands out in multivariate Cox regression analyses as a highly significant predictor for increased mortality or lung transplant. GAP index (HR 1.26; p=0.009), lymphocytosis in BAL (HR 0.97; p=0.018) and age (HR 1.03; p=0.018) are also predictors of progression. Conclusions: The study identified several prognostic factors for progression and/or survival in fHP. The presence of fibroblastic foci at biopsy was a consistent predictor for increased mortality and the presence of lymphocytosis in BAL was inversely related to mortality.application/pdfengCopyright © The authors 2024. This version is distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0 (http://creativecommons.org/licenses/by-nc/4.0/). For commercial reproduction rights and permissions contact permissions@ersnet.orgPneumonitis per hipersensibilitatinfo:eu-repo/semantics/articlehttp://dx.doi.org/10.1183/23120541.00405-2023info:eu-repo/semantics/openAccess