Prognostic factors of progressive fibrotic hypersensitivity pneumonitis: a large, retrospective, multicentre, observational cohort study.

Autor:	Cano-Jiménez E; Hospital Universitario Lucus Augusti, Lugo, Spain., Villar Gómez A; Hospital Vall d'Hebrón, Barcelona, Spain.; CIBER de Respiratorio (CIBERES), Madrid, Spain., Velez Segovia E; Hospital Vall d'Hebrón, Barcelona, Spain., Aburto Barrenechea M; Hospital Universitario Galdakao, Galdakao, Spain., Sellarés Torres J; CIBER de Respiratorio (CIBERES), Madrid, Spain.; Hospital Clínic, Barcelona, Spain., Francesqui J; Hospital Clínic, Barcelona, Spain., Portillo Carroz K; Hospital Universitari Germans Trias i Pujol, Barcelona, Spain., Solis Solis AJ; Hospital Universitari Germans Trias i Pujol, Barcelona, Spain., Acosta Fernández O; Complejo Hospitalario Universitario de Canarias, Santa Cruz De Tenerife, Spain., Llanos González AB; Complejo Hospitalario Universitario de Canarias, Santa Cruz De Tenerife, Spain., Bordas-Martinez J; Hospital Universitari de Bellvitge, IDIBELL, Barcelona, Spain., Cabrera Cesar E; Hospital Universitario Virgen de la Victoria, Málaga, Spain., Balcells Vilarnau E; CIBER de Respiratorio (CIBERES), Madrid, Spain.; Hospital del Mar, Barcelona, Spain., Castillo Villegas D; CIBER de Respiratorio (CIBERES), Madrid, Spain.; Hospital de la Santa Creu i Sant Pau, Barcelona, Spain., Reyes Pardessus A; Hospital de la Santa Creu i Sant Pau, Barcelona, Spain., González Fernández C; Complejo Hospitalario Universitario de Ourense, Ourense, Spain., García Moyano M; Hospital Universitario de Cruces, Barakaldo, Spain., Urrutia Gajate A; Hospital Universitario de Cruces, Barakaldo, Spain., Blanco Hortas A; Fundación Instituto de Investigación Sanitaria de Santiago de Compostela, Hospital Universitario Lucus Augusti, Lugo, Spain., Molina-Molina M; CIBER de Respiratorio (CIBERES), Madrid, Spain.; Hospital Universitari de Bellvitge, IDIBELL, Barcelona, Spain.
Jazyk:	angličtina
Zdroj:	ERJ open research [ERJ Open Res] 2024 Feb 26; Vol. 10 (1). Date of Electronic Publication: 2024 Feb 26 (Print Publication: 2024).
DOI:	10.1183/23120541.00405-2023
Abstrakt:	Background: 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. Competing Interests: Conflict of interest: E. Cano-Jiménez has received grants and fees for research purposes or speaking from Roche, Bristol Myers and Boehringer Ingelheim. Conflict of interest: A. Villar Gómez has received travel grants, consulting fees, speaking fees or research grants from Boehringer Ingelheim, Roche, Glaxo and Chiesi. Conflict of interest: M. Aburto Barrenechea reports lecture fees and support for attending meetings from Boehringer Ingelheim in the last 36 months, outside the submitted work. Conflict of interest: J. Sellarés Torres has received funding from Boehringer and Roche, outside the submitted work. Conflict of interest: D. Castillo Villegas reports personal fees and nonfinancial support from Roche; grants, personal fees and nonfinancial support from Boehringer Ingelheim; grants from Fujirebio; and personal fees from Veracyte, outside the submitted work. Conflict of interest: C. González Fernández has participated in conferences, scientific meetings, consulting, research and scientific dissemination activities funded by AstraZeneca, Chiesi, Teva, Sanofi, Novartis, GlaxoSmithKline, Boehringer Ingelheim, Bristol Myers Squibb and Roche. Conflict of interest: M. Molina-Molina has received grants and fees for research purposes and scientific advice from Ferrer, Boehringer Ingelheim, Roche, Esteve-Teijin, Chiesi and Janssen. Conflict of interest: E. Velez Segovia, J. Francesqui, O. Acosta Fernández, J. Bordas-Martinez, K. Portillo Carroz, A.J. Solis Solis, A.B. Llanos Gonzáles, E. Cabrera Cesar, E. Balcells Vilarnau, A. Reyes Pardessus, M. García Moyano, A. Urrutia Gajate and A. Blanco Hortas have no conflicts of interest to report. (Copyright ©The authors 2024.)
Databáze:	MEDLINE
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