Benefits of pulmonary rehabilitation in patients with advanced lymphangioleiomyomatosis (LAM) compared with COPD – a retrospective analysis

ABSTRACT: Lymphangioleiomyomatosis (LAM) is a rare and progressive cystic lung disease with limited therapeutic options. We retrospectively analyzed the effects of a comprehensive 4-week inpatient pulmonary rehabilitation (PR) program in 58 patients with advanced LAM (FEV1: 45 ± 34%predicted, 6-min...

Descripción completa

Detalles Bibliográficos
Autores principales: Gloeckl, Rainer, Nell, Christoph, Schneeberger, Tessa, Jarosch, Inga, Boensch, Martina, Watz, Henrik, Wirtz, Hubert, Welte, Tobias, Kenn, Klaus, Koczulla, Andreas Rembert
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2020
Materias:
Letter to the Editor
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7507679/
https://www.ncbi.nlm.nih.gov/pubmed/32962746
http://dx.doi.org/10.1186/s13023-020-01540-3
_version_ 1783585277799825408
author Gloeckl, Rainer
Nell, Christoph
Schneeberger, Tessa
Jarosch, Inga
Boensch, Martina
Watz, Henrik
Wirtz, Hubert
Welte, Tobias
Kenn, Klaus
Koczulla, Andreas Rembert
author_facet Gloeckl, Rainer
Nell, Christoph
Schneeberger, Tessa
Jarosch, Inga
Boensch, Martina
Watz, Henrik
Wirtz, Hubert
Welte, Tobias
Kenn, Klaus
Koczulla, Andreas Rembert
author_sort Gloeckl, Rainer
collection PubMed
description ABSTRACT: Lymphangioleiomyomatosis (LAM) is a rare and progressive cystic lung disease with limited therapeutic options. We retrospectively analyzed the effects of a comprehensive 4-week inpatient pulmonary rehabilitation (PR) program in 58 patients with advanced LAM (FEV1: 45 ± 34%predicted, 6-min walk distance (6MWD): 338 ± 167 m). Exercise performance (6MWD: + 49 ± 50 m; p < 0.001) and quality of life (SF-36 physical component: + 2.4 ± 7.8 points; p = 0.049 and mental component: + 5.2 ± 12.1 points; p < 0.001) increased significantly after PR comparable to an COPD cohort. There were no clinical parameters that predicted changes in outcomes following PR. PR seems to be an effective therapeutic option even in patients with advanced LAM. TRIAL REGISTRATION: Clinical-Trials registration number: NCT04184193; date of registration: December 3, 2019.
format Online
Article
Text
id pubmed-7507679
institution National Center for Biotechnology Information
language English
publishDate 2020
publisher BioMed Central
record_format MEDLINE/PubMed
spelling pubmed-75076792020-09-23 Benefits of pulmonary rehabilitation in patients with advanced lymphangioleiomyomatosis (LAM) compared with COPD – a retrospective analysis Gloeckl, Rainer Nell, Christoph Schneeberger, Tessa Jarosch, Inga Boensch, Martina Watz, Henrik Wirtz, Hubert Welte, Tobias Kenn, Klaus Koczulla, Andreas Rembert Orphanet J Rare Dis Letter to the Editor ABSTRACT: Lymphangioleiomyomatosis (LAM) is a rare and progressive cystic lung disease with limited therapeutic options. We retrospectively analyzed the effects of a comprehensive 4-week inpatient pulmonary rehabilitation (PR) program in 58 patients with advanced LAM (FEV1: 45 ± 34%predicted, 6-min walk distance (6MWD): 338 ± 167 m). Exercise performance (6MWD: + 49 ± 50 m; p < 0.001) and quality of life (SF-36 physical component: + 2.4 ± 7.8 points; p = 0.049 and mental component: + 5.2 ± 12.1 points; p < 0.001) increased significantly after PR comparable to an COPD cohort. There were no clinical parameters that predicted changes in outcomes following PR. PR seems to be an effective therapeutic option even in patients with advanced LAM. TRIAL REGISTRATION: Clinical-Trials registration number: NCT04184193; date of registration: December 3, 2019. BioMed Central 2020-09-22 /pmc/articles/PMC7507679/ /pubmed/32962746 http://dx.doi.org/10.1186/s13023-020-01540-3 Text en © The Author(s) 2020 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/. The Creative Commons Public Domain Dedication waiver (http://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 Letter to the Editor
Gloeckl, Rainer
Nell, Christoph
Schneeberger, Tessa
Jarosch, Inga
Boensch, Martina
Watz, Henrik
Wirtz, Hubert
Welte, Tobias
Kenn, Klaus
Koczulla, Andreas Rembert
Benefits of pulmonary rehabilitation in patients with advanced lymphangioleiomyomatosis (LAM) compared with COPD – a retrospective analysis
title Benefits of pulmonary rehabilitation in patients with advanced lymphangioleiomyomatosis (LAM) compared with COPD – a retrospective analysis
title_full Benefits of pulmonary rehabilitation in patients with advanced lymphangioleiomyomatosis (LAM) compared with COPD – a retrospective analysis
title_fullStr Benefits of pulmonary rehabilitation in patients with advanced lymphangioleiomyomatosis (LAM) compared with COPD – a retrospective analysis
title_full_unstemmed Benefits of pulmonary rehabilitation in patients with advanced lymphangioleiomyomatosis (LAM) compared with COPD – a retrospective analysis
title_short Benefits of pulmonary rehabilitation in patients with advanced lymphangioleiomyomatosis (LAM) compared with COPD – a retrospective analysis
title_sort benefits of pulmonary rehabilitation in patients with advanced lymphangioleiomyomatosis (lam) compared with copd – a retrospective analysis
topic Letter to the Editor
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7507679/
https://www.ncbi.nlm.nih.gov/pubmed/32962746
http://dx.doi.org/10.1186/s13023-020-01540-3
work_keys_str_mv AT gloecklrainer benefitsofpulmonaryrehabilitationinpatientswithadvancedlymphangioleiomyomatosislamcomparedwithcopdaretrospectiveanalysis
AT nellchristoph benefitsofpulmonaryrehabilitationinpatientswithadvancedlymphangioleiomyomatosislamcomparedwithcopdaretrospectiveanalysis
AT schneebergertessa benefitsofpulmonaryrehabilitationinpatientswithadvancedlymphangioleiomyomatosislamcomparedwithcopdaretrospectiveanalysis
AT jaroschinga benefitsofpulmonaryrehabilitationinpatientswithadvancedlymphangioleiomyomatosislamcomparedwithcopdaretrospectiveanalysis
AT boenschmartina benefitsofpulmonaryrehabilitationinpatientswithadvancedlymphangioleiomyomatosislamcomparedwithcopdaretrospectiveanalysis
AT watzhenrik benefitsofpulmonaryrehabilitationinpatientswithadvancedlymphangioleiomyomatosislamcomparedwithcopdaretrospectiveanalysis
AT wirtzhubert benefitsofpulmonaryrehabilitationinpatientswithadvancedlymphangioleiomyomatosislamcomparedwithcopdaretrospectiveanalysis
AT weltetobias benefitsofpulmonaryrehabilitationinpatientswithadvancedlymphangioleiomyomatosislamcomparedwithcopdaretrospectiveanalysis
AT kennklaus benefitsofpulmonaryrehabilitationinpatientswithadvancedlymphangioleiomyomatosislamcomparedwithcopdaretrospectiveanalysis
AT koczullaandreasrembert benefitsofpulmonaryrehabilitationinpatientswithadvancedlymphangioleiomyomatosislamcomparedwithcopdaretrospectiveanalysis

Ejemplares similares