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Combined obstructive airflow limitation associated with interstitial lung diseases (O-ILD): the bad phenotype ?
BACKGROUND: Patients suffering from combined obstructive and interstitial lung disease (O-ILD) represent a pathological entity which still has to be well clinically described. The aim of this descriptive and explorative study was to describe the phenotype and functional characteristics of a cohort o...
Autores principales: | , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8996531/ https://www.ncbi.nlm.nih.gov/pubmed/35410260 http://dx.doi.org/10.1186/s12931-022-02006-9 |
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author | Guiot, Julien Henket, Monique Frix, Anne-Noëlle Gester, Fanny Thys, Marie Giltay, Laurie Desir, Colin Moermans, Catherine Njock, Makon-Sébastien Meunier, Paul Corhay, Jean-Louis Louis, Renaud |
author_facet | Guiot, Julien Henket, Monique Frix, Anne-Noëlle Gester, Fanny Thys, Marie Giltay, Laurie Desir, Colin Moermans, Catherine Njock, Makon-Sébastien Meunier, Paul Corhay, Jean-Louis Louis, Renaud |
author_sort | Guiot, Julien |
collection | PubMed |
description | BACKGROUND: Patients suffering from combined obstructive and interstitial lung disease (O-ILD) represent a pathological entity which still has to be well clinically described. The aim of this descriptive and explorative study was to describe the phenotype and functional characteristics of a cohort of patients suffering from functional obstruction in a population of ILD patients in order to raise the need of dedicated prospective observational studies and the evaluation of the impact of anti-fibrotic therapies. METHODS: The current authors conducted a retrospective study including 557 ILD patients, with either obstructive (O-ILD, n = 82) or non-obstructive (non O-ILD, n = 475) pattern. Patients included were mainly males (54%) with a mean age of 62 years. RESULTS: Patients with O-ILD exhibited a characteristic functional profile with reduced percent predicted forced expired volume in 1 s (FEV1) [65% (53–77) vs 83% (71–96), p < 0.00001], small airway involvement assessed by maximum expiratory flow (MEF) 25/75 [29% (20–41) vs 81% (64–108), p < 0.00001], reduced sGaw [60% (42–75) vs 87% (59–119), p < 0.01] and sub-normal functional residual capacity (FRC) [113% (93–134) vs 92% (75–109), p < 0.00001] with no impaired of carbon monoxide diffusing capacity of the lung (DLCO) compared to those without obstruction. Total lung capacity (TLC) was increased in O-ILD patients [93% (82–107) vs 79% (69–91), p < 0.00001]. Of interest, DLCO sharply dropped in O-ILD patients over a 5-year follow-up. We did not identify a significant increase in mortality in patients with O-ILD. Interestingly, the global mortality was increased in the specific sub-group of patients with O-ILD and no progressive fibrosing ILD phenotype and in those with connective tissue disease associated ILD especially in case of rheumatoid arthritis. CONCLUSIONS: The authors individualized a specific functional-based pattern of ILD patients with obstructive lung disease, who are at risk of increased mortality and rapid DLCO decline over time. As classically those patients are excluded from clinical trials, a dedicated prospective study would be of interest in order to define more precisely treatment response of those patients. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12931-022-02006-9. |
format | Online Article Text |
id | pubmed-8996531 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-89965312022-04-12 Combined obstructive airflow limitation associated with interstitial lung diseases (O-ILD): the bad phenotype ? Guiot, Julien Henket, Monique Frix, Anne-Noëlle Gester, Fanny Thys, Marie Giltay, Laurie Desir, Colin Moermans, Catherine Njock, Makon-Sébastien Meunier, Paul Corhay, Jean-Louis Louis, Renaud Respir Res Research BACKGROUND: Patients suffering from combined obstructive and interstitial lung disease (O-ILD) represent a pathological entity which still has to be well clinically described. The aim of this descriptive and explorative study was to describe the phenotype and functional characteristics of a cohort of patients suffering from functional obstruction in a population of ILD patients in order to raise the need of dedicated prospective observational studies and the evaluation of the impact of anti-fibrotic therapies. METHODS: The current authors conducted a retrospective study including 557 ILD patients, with either obstructive (O-ILD, n = 82) or non-obstructive (non O-ILD, n = 475) pattern. Patients included were mainly males (54%) with a mean age of 62 years. RESULTS: Patients with O-ILD exhibited a characteristic functional profile with reduced percent predicted forced expired volume in 1 s (FEV1) [65% (53–77) vs 83% (71–96), p < 0.00001], small airway involvement assessed by maximum expiratory flow (MEF) 25/75 [29% (20–41) vs 81% (64–108), p < 0.00001], reduced sGaw [60% (42–75) vs 87% (59–119), p < 0.01] and sub-normal functional residual capacity (FRC) [113% (93–134) vs 92% (75–109), p < 0.00001] with no impaired of carbon monoxide diffusing capacity of the lung (DLCO) compared to those without obstruction. Total lung capacity (TLC) was increased in O-ILD patients [93% (82–107) vs 79% (69–91), p < 0.00001]. Of interest, DLCO sharply dropped in O-ILD patients over a 5-year follow-up. We did not identify a significant increase in mortality in patients with O-ILD. Interestingly, the global mortality was increased in the specific sub-group of patients with O-ILD and no progressive fibrosing ILD phenotype and in those with connective tissue disease associated ILD especially in case of rheumatoid arthritis. CONCLUSIONS: The authors individualized a specific functional-based pattern of ILD patients with obstructive lung disease, who are at risk of increased mortality and rapid DLCO decline over time. As classically those patients are excluded from clinical trials, a dedicated prospective study would be of interest in order to define more precisely treatment response of those patients. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12931-022-02006-9. BioMed Central 2022-04-11 2022 /pmc/articles/PMC8996531/ /pubmed/35410260 http://dx.doi.org/10.1186/s12931-022-02006-9 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data. |
spellingShingle | Research Guiot, Julien Henket, Monique Frix, Anne-Noëlle Gester, Fanny Thys, Marie Giltay, Laurie Desir, Colin Moermans, Catherine Njock, Makon-Sébastien Meunier, Paul Corhay, Jean-Louis Louis, Renaud Combined obstructive airflow limitation associated with interstitial lung diseases (O-ILD): the bad phenotype ? |
title | Combined obstructive airflow limitation associated with interstitial lung diseases (O-ILD): the bad phenotype ? |
title_full | Combined obstructive airflow limitation associated with interstitial lung diseases (O-ILD): the bad phenotype ? |
title_fullStr | Combined obstructive airflow limitation associated with interstitial lung diseases (O-ILD): the bad phenotype ? |
title_full_unstemmed | Combined obstructive airflow limitation associated with interstitial lung diseases (O-ILD): the bad phenotype ? |
title_short | Combined obstructive airflow limitation associated with interstitial lung diseases (O-ILD): the bad phenotype ? |
title_sort | combined obstructive airflow limitation associated with interstitial lung diseases (o-ild): the bad phenotype ? |
topic | Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8996531/ https://www.ncbi.nlm.nih.gov/pubmed/35410260 http://dx.doi.org/10.1186/s12931-022-02006-9 |
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