A mini-whole lung lavage to treat autoimmune pulmonary alveolar proteinosis (PAP)
Abstract Background PAP is an ultra-rare respiratory syndrome characterized by the accumulation of surfactant within the alveoli. Whole lung lavage (WLL) is the current standard of care of PAP, however it is not a standardized procedure and the total amount of fluid used to wash each lung is still d...
| Main Authors: | , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
BMC
2022-03-01
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| Series: | Respiratory Research |
| Subjects: | |
| Online Access: | https://doi.org/10.1186/s12931-022-01982-2 |
| _version_ | 1818315567619112960 |
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| author | Francesca Mariani Elena Salvaterra Sara Lettieri Annalisa De Silvestri Alessandra Corino Matteo Bosio Elia Fraolini Davide Piloni Giuseppe Rodi Angelo Guido Corsico Ilaria Campo |
| author_facet | Francesca Mariani Elena Salvaterra Sara Lettieri Annalisa De Silvestri Alessandra Corino Matteo Bosio Elia Fraolini Davide Piloni Giuseppe Rodi Angelo Guido Corsico Ilaria Campo |
| author_sort | Francesca Mariani |
| collection | DOAJ |
| description | Abstract Background PAP is an ultra-rare respiratory syndrome characterized by the accumulation of surfactant within the alveoli. Whole lung lavage (WLL) is the current standard of care of PAP, however it is not a standardized procedure and the total amount of fluid used to wash each lung is still debated. Considering ICU hospitalization associated risks, a “mini-WLL” with anticipated manual clapping and reduced total infusion volume and has been proposed in our center. The aim of the study is to retrospectively analyze the efficacy of mini-WLL compared to standard WLL at the Pavia center. Methods 13 autoimmune PAP patients eligible for WLL were included: 7 patients were admitted to mini-WLL (9 L total infusion volume for each lung) and 6 patients underwent standard WLL (14 L of infusion volume). Functional data (VC%, FVC%, TLC%, DLCO%) and alveolar-arterial gradient values (A-aO2) were collected at the baseline and 1, 3, 6, 12, 18 months after the procedure. Results A statistically significant improvement of VC% (p = 0.013, 95%CI 3.49–30.19), FVC% (p = 0.016, 95%CI 3.37–32.09), TLC% (p = 0.001, 95%CI 7.38–30.34) was observed in the mini-WLL group in comparison with the standard WLL group, while no significant difference in DLCO% and A-aO2 mean values were reported. Conclusion Mini-WLL has demonstrated higher efficacy in ameliorating lung volumes, suggesting that a lower infusion volume is sufficient to remove the surfactant accumulation and possibly allows a reduced mechanical insult of the bronchi walls and the alveoli. However, no statistically significant differences were found in terms of DLCO% and Aa-O2. |
| first_indexed | 2024-12-13T09:07:35Z |
| format | Article |
| id | doaj.art-edc962aaf4bf413a947a7b389d53560a |
| institution | Directory Open Access Journal |
| issn | 1465-993X |
| language | English |
| last_indexed | 2024-12-13T09:07:35Z |
| publishDate | 2022-03-01 |
| publisher | BMC |
| record_format | Article |
| series | Respiratory Research |
| spelling | doaj.art-edc962aaf4bf413a947a7b389d53560a2022-12-21T23:53:02ZengBMCRespiratory Research1465-993X2022-03-012311610.1186/s12931-022-01982-2A mini-whole lung lavage to treat autoimmune pulmonary alveolar proteinosis (PAP)Francesca Mariani0Elena Salvaterra1Sara Lettieri2Annalisa De Silvestri3Alessandra Corino4Matteo Bosio5Elia Fraolini6Davide Piloni7Giuseppe Rodi8Angelo Guido Corsico9Ilaria Campo10Pneumology Unit, IRCCS San Matteo Hospital FoundationDepartment of Internal Medicine and Therapeutics, University of PaviaDepartment of Internal Medicine and Therapeutics, University of PaviaClinical Epidemiology and Biometry Service, IRCCS San Matteo Hospital FoundationPneumology Unit, IRCCS San Matteo Hospital FoundationPneumology Unit, IRCCS San Matteo Hospital FoundationPneumology Unit, IRCCS San Matteo Hospital FoundationPneumology Unit, IRCCS San Matteo Hospital FoundationIntensive Care Unit- IRCCS San Matteo Hospital FoundationPneumology Unit, IRCCS San Matteo Hospital FoundationPneumology Unit, IRCCS San Matteo Hospital FoundationAbstract Background PAP is an ultra-rare respiratory syndrome characterized by the accumulation of surfactant within the alveoli. Whole lung lavage (WLL) is the current standard of care of PAP, however it is not a standardized procedure and the total amount of fluid used to wash each lung is still debated. Considering ICU hospitalization associated risks, a “mini-WLL” with anticipated manual clapping and reduced total infusion volume and has been proposed in our center. The aim of the study is to retrospectively analyze the efficacy of mini-WLL compared to standard WLL at the Pavia center. Methods 13 autoimmune PAP patients eligible for WLL were included: 7 patients were admitted to mini-WLL (9 L total infusion volume for each lung) and 6 patients underwent standard WLL (14 L of infusion volume). Functional data (VC%, FVC%, TLC%, DLCO%) and alveolar-arterial gradient values (A-aO2) were collected at the baseline and 1, 3, 6, 12, 18 months after the procedure. Results A statistically significant improvement of VC% (p = 0.013, 95%CI 3.49–30.19), FVC% (p = 0.016, 95%CI 3.37–32.09), TLC% (p = 0.001, 95%CI 7.38–30.34) was observed in the mini-WLL group in comparison with the standard WLL group, while no significant difference in DLCO% and A-aO2 mean values were reported. Conclusion Mini-WLL has demonstrated higher efficacy in ameliorating lung volumes, suggesting that a lower infusion volume is sufficient to remove the surfactant accumulation and possibly allows a reduced mechanical insult of the bronchi walls and the alveoli. However, no statistically significant differences were found in terms of DLCO% and Aa-O2.https://doi.org/10.1186/s12931-022-01982-2Pulmonary alveolar proteinosisWhole lung lavageICU stay |
| spellingShingle | Francesca Mariani Elena Salvaterra Sara Lettieri Annalisa De Silvestri Alessandra Corino Matteo Bosio Elia Fraolini Davide Piloni Giuseppe Rodi Angelo Guido Corsico Ilaria Campo A mini-whole lung lavage to treat autoimmune pulmonary alveolar proteinosis (PAP) Respiratory Research Pulmonary alveolar proteinosis Whole lung lavage ICU stay |
| title | A mini-whole lung lavage to treat autoimmune pulmonary alveolar proteinosis (PAP) |
| title_full | A mini-whole lung lavage to treat autoimmune pulmonary alveolar proteinosis (PAP) |
| title_fullStr | A mini-whole lung lavage to treat autoimmune pulmonary alveolar proteinosis (PAP) |
| title_full_unstemmed | A mini-whole lung lavage to treat autoimmune pulmonary alveolar proteinosis (PAP) |
| title_short | A mini-whole lung lavage to treat autoimmune pulmonary alveolar proteinosis (PAP) |
| title_sort | mini whole lung lavage to treat autoimmune pulmonary alveolar proteinosis pap |
| topic | Pulmonary alveolar proteinosis Whole lung lavage ICU stay |
| url | https://doi.org/10.1186/s12931-022-01982-2 |
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