Benefits of pulmonary rehabilitation in patients with advanced lymphangioleiomyomatosis (LAM) compared with COPD - a retrospective analysis.
Exercise
LAM
Lymphangioleiomyomatosis
Pulmonary rehabilitation
Quality of life
Therapy
Journal
Orphanet journal of rare diseases
ISSN: 1750-1172
Titre abrégé: Orphanet J Rare Dis
Pays: England
ID NLM: 101266602
Informations de publication
Date de publication:
22 09 2020
22 09 2020
Historique:
received:
17
06
2020
accepted:
10
09
2020
entrez:
23
9
2020
pubmed:
24
9
2020
medline:
19
5
2021
Statut:
epublish
Résumé
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.
Identifiants
pubmed: 32962746
doi: 10.1186/s13023-020-01540-3
pii: 10.1186/s13023-020-01540-3
pmc: PMC7507679
doi:
Banques de données
ClinicalTrials.gov
['NCT04184193']
Types de publication
Letter
Langues
eng
Sous-ensembles de citation
IM
Pagination
255Références
Eur Respir J. 2014 Dec;44(6):1428-46
pubmed: 25359355
Eur Respir Rev. 2013 Jun 1;22(128):178-86
pubmed: 23728873
Eur Respir J. 2016 May;47(5):1452-60
pubmed: 26917604
Thorax. 2016 Jul;71(7):662-3
pubmed: 26968971
Eur Respir J. 2014 Dec;44(6):1447-78
pubmed: 25359356
Am J Respir Crit Care Med. 2013 Oct 15;188(8):e13-64
pubmed: 24127811
Eur Respir J. 2010 Jan;35(1):14-26
pubmed: 20044458
Thorax. 2019 Oct;74(10):999-1002
pubmed: 31363020
Am J Respir Crit Care Med. 2012 Aug 15;186(4):341-8
pubmed: 22700863
Am J Respir Crit Care Med. 2016 Sep 15;194(6):748-61
pubmed: 27628078