Pulmonary fibrosis in patients with autoimmune pulmonary alveolar proteinosis: a retrospective nationwide cohort study
Background Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease that may progress towards pulmonary fibrosis. Data about fibrosis prevalence and risk factors are lacking. Methods In this retrospective multicentre nationwide cohort, we included patients newly diagnosed with aPAP between...
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European Respiratory Society
2024-12-01
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| Series: | ERJ Open Research |
| Online Access: | http://openres.ersjournals.com/content/10/6/00314-2024.full |
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| author | Yoann Guirriec David Luque-Paz Gontran Bernard Axelle Mabo Mallorie Kerjouan Cédric Ménard Delphine Monnier Hilario Nunes Yurdagül Uzunhan Martine Reynaud-Gaubert Julien Bermudez Raphaël Borie Bruno Crestani Julie Traclet Lidwine Wémeau-Stervinou Cécile Chenivesse Emmanuel Gomez Grégoire Prévot Arnaud Bourdin Benjamin Bondue Anne Bergeron Vincent Cottin Mathieu Lederlin Stéphane Jouneau |
| author_facet | Yoann Guirriec David Luque-Paz Gontran Bernard Axelle Mabo Mallorie Kerjouan Cédric Ménard Delphine Monnier Hilario Nunes Yurdagül Uzunhan Martine Reynaud-Gaubert Julien Bermudez Raphaël Borie Bruno Crestani Julie Traclet Lidwine Wémeau-Stervinou Cécile Chenivesse Emmanuel Gomez Grégoire Prévot Arnaud Bourdin Benjamin Bondue Anne Bergeron Vincent Cottin Mathieu Lederlin Stéphane Jouneau |
| author_sort | Yoann Guirriec |
| collection | DOAJ |
| description | Background
Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease that may progress towards pulmonary fibrosis. Data about fibrosis prevalence and risk factors are lacking.
Methods
In this retrospective multicentre nationwide cohort, we included patients newly diagnosed with aPAP between 2008 and 2018 in France and Belgium. Data were collected from medical records using a standardised questionnaire.
Results
61 patients were included in the final analysis. We identified 5 patients (8%) with fibrosis on initial computed tomography (CT) and 16 patients (26%) with fibrosis on final CT after a median time of 3.6 years. Dust exposure was associated with pulmonary fibrosis occurrence (OR 4.3; p=0.038). aPAP patients treated with whole-lung lavage, rituximab or granulocyte–monocyte colony-stimulating factor therapy did not have more fibrotic evolution than patients who did not receive these treatments (n=25 out of 45, 57% versus n=10 out of 16, 62%; p=0.69). All-cause mortality was significantly higher in fibrotic than in nonfibrotic cases (n=4 out of 16, 25% versus n=2 out of 45, 4.4%; p=0.036, respectively).
Conclusion
In our population, a quarter of aPAP patients progressed towards pulmonary fibrosis. Dust exposure seems to be an important factor associated with this complication. More studies are needed to analyse precisely the impact of dust exposure impact, especially silica, in patients with aPAP. |
| format | Article |
| id | doaj-art-c6dfb032889b4d5d9a2bee98ea68e4a3 |
| institution | Kabale University |
| issn | 2312-0541 |
| language | English |
| publishDate | 2024-12-01 |
| publisher | European Respiratory Society |
| record_format | Article |
| series | ERJ Open Research |
| spelling | doaj-art-c6dfb032889b4d5d9a2bee98ea68e4a32025-01-14T09:50:21ZengEuropean Respiratory SocietyERJ Open Research2312-05412024-12-0110610.1183/23120541.00314-202400314-2024Pulmonary fibrosis in patients with autoimmune pulmonary alveolar proteinosis: a retrospective nationwide cohort studyYoann Guirriec0David Luque-Paz1Gontran Bernard2Axelle Mabo3Mallorie Kerjouan4Cédric Ménard5Delphine Monnier6Hilario Nunes7Yurdagül Uzunhan8Martine Reynaud-Gaubert9Julien Bermudez10Raphaël Borie11Bruno Crestani12Julie Traclet13Lidwine Wémeau-Stervinou14Cécile Chenivesse15Emmanuel Gomez16Grégoire Prévot17Arnaud Bourdin18Benjamin Bondue19Anne Bergeron20Vincent Cottin21Mathieu Lederlin22Stéphane Jouneau23 Pneumologie, Hôpital Pontchaillou, CHU Rennes, Rennes, France Pneumologie, Hôpital Pontchaillou, CHU Rennes, Rennes, France Imagerie médicale, Hôpital Pontchaillou, CHU Rennes, Rennes, France Pneumologie, Hôpital Pontchaillou, CHU Rennes, Rennes, France Pneumologie, Hôpital Pontchaillou, CHU Rennes, Rennes, France Service d'Immunologie, Laboratoire de Biologie Médicale de Référence Lipoprotéinose Alvéolaire, Hôpital Pontchaillou, CHU Rennes, Rennes, France Service d'Immunologie, Laboratoire de Biologie Médicale de Référence Lipoprotéinose Alvéolaire, Hôpital Pontchaillou, CHU Rennes, Rennes, France Centre de référence constitutif des maladies pulmonaires rares, Service de Pneumologie, AP-HP, Hôpital Avicenne, INSERM U1272, Université Sorbonne Paris Nord, Bobigny, France Centre de référence constitutif des maladies pulmonaires rares, Service de Pneumologie, AP-HP, Hôpital Avicenne, INSERM U1272, Université Sorbonne Paris Nord, Bobigny, France Centre de compétence pour les maladies pulmonaires rares, Service de Pneumologie, AP-HM, CHU Nord, Marseille, France Centre de compétence pour les maladies pulmonaires rares, Service de Pneumologie, AP-HM, CHU Nord, Marseille, France Service de Pneumologie A, Hôpital Bichat, AP-HP, Paris, France Service de Pneumologie A, Hôpital Bichat, AP-HP, Paris, France Centre national coordonnateur de référence des maladies pulmonaires rares, Service de Pneumologie, Hôpital Louis-Pradel, Hospices Civils de Lyon, UMR754, INRAE, Université Lyon 1, ERN-LUNG, Lyon, France Univ. Lille, CNRS, Inserm, CHU de Lille, U1019 – UMR 9017 – CIIL – Center for Infection and Immunity of Lille, Centre de référence constitutif des maladies pulmonaires rares, Institut Cœur-Poumon, Service de Pneumologie et Immuno-allergologie, Lille, France Univ. Lille, CNRS, Inserm, CHU de Lille, U1019 – UMR 9017 – CIIL – Center for Infection and Immunity of Lille, Centre de référence constitutif des maladies pulmonaires rares, Institut Cœur-Poumon, Service de Pneumologie et Immuno-allergologie, Lille, France Centre de compétence pour les maladies pulmonaires rares, département de pneumologie, Hôpitaux de Brabois, CHRU de Nancy, Vandoeuvre-les Nancy, France Centre de compétence pour les maladies pulmonaires rares, Service de Pneumologie, CHU Larrey, Toulouse, France Service de Pneumologie, Centre Hospitalier Universitaire de Montpellier, Université de Montpellier, Montpellier, France Service de Pneumologie, Hôpital Universitaire de Bruxelles, Université libre de Bruxelles, Brussels, Belgium Centre de référence constitutif des maladies pulmonaires rares, Service de Pneumologie, AP-HP, Hôpital Saint Louis, Paris, France Centre national coordonnateur de référence des maladies pulmonaires rares, Service de Pneumologie, Hôpital Louis-Pradel, Hospices Civils de Lyon, UMR754, INRAE, Université Lyon 1, ERN-LUNG, Lyon, France Imagerie médicale, Hôpital Pontchaillou, CHU Rennes, Rennes, France Pneumologie, Hôpital Pontchaillou, CHU Rennes, Rennes, France Background Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare disease that may progress towards pulmonary fibrosis. Data about fibrosis prevalence and risk factors are lacking. Methods In this retrospective multicentre nationwide cohort, we included patients newly diagnosed with aPAP between 2008 and 2018 in France and Belgium. Data were collected from medical records using a standardised questionnaire. Results 61 patients were included in the final analysis. We identified 5 patients (8%) with fibrosis on initial computed tomography (CT) and 16 patients (26%) with fibrosis on final CT after a median time of 3.6 years. Dust exposure was associated with pulmonary fibrosis occurrence (OR 4.3; p=0.038). aPAP patients treated with whole-lung lavage, rituximab or granulocyte–monocyte colony-stimulating factor therapy did not have more fibrotic evolution than patients who did not receive these treatments (n=25 out of 45, 57% versus n=10 out of 16, 62%; p=0.69). All-cause mortality was significantly higher in fibrotic than in nonfibrotic cases (n=4 out of 16, 25% versus n=2 out of 45, 4.4%; p=0.036, respectively). Conclusion In our population, a quarter of aPAP patients progressed towards pulmonary fibrosis. Dust exposure seems to be an important factor associated with this complication. More studies are needed to analyse precisely the impact of dust exposure impact, especially silica, in patients with aPAP.http://openres.ersjournals.com/content/10/6/00314-2024.full |
| spellingShingle | Yoann Guirriec David Luque-Paz Gontran Bernard Axelle Mabo Mallorie Kerjouan Cédric Ménard Delphine Monnier Hilario Nunes Yurdagül Uzunhan Martine Reynaud-Gaubert Julien Bermudez Raphaël Borie Bruno Crestani Julie Traclet Lidwine Wémeau-Stervinou Cécile Chenivesse Emmanuel Gomez Grégoire Prévot Arnaud Bourdin Benjamin Bondue Anne Bergeron Vincent Cottin Mathieu Lederlin Stéphane Jouneau Pulmonary fibrosis in patients with autoimmune pulmonary alveolar proteinosis: a retrospective nationwide cohort study ERJ Open Research |
| title | Pulmonary fibrosis in patients with autoimmune pulmonary alveolar proteinosis: a retrospective nationwide cohort study |
| title_full | Pulmonary fibrosis in patients with autoimmune pulmonary alveolar proteinosis: a retrospective nationwide cohort study |
| title_fullStr | Pulmonary fibrosis in patients with autoimmune pulmonary alveolar proteinosis: a retrospective nationwide cohort study |
| title_full_unstemmed | Pulmonary fibrosis in patients with autoimmune pulmonary alveolar proteinosis: a retrospective nationwide cohort study |
| title_short | Pulmonary fibrosis in patients with autoimmune pulmonary alveolar proteinosis: a retrospective nationwide cohort study |
| title_sort | pulmonary fibrosis in patients with autoimmune pulmonary alveolar proteinosis a retrospective nationwide cohort study |
| url | http://openres.ersjournals.com/content/10/6/00314-2024.full |
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