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 registrationClinical-Trials registration number: NCT04184193; date of registration: December 3, 2019.
Highlights
Lymphangioleiomyomatosis (LAM) is a very rare, systemic neoplastic disease associated with progressive cystic lung destruction mostly affecting young women [1]
Since LAM is a rare disease data collection is difficult and we performed a retrospective analysis of LAM patients that were referred to pulmonary rehabilitation (PR). Data for this analysis were consecutively collected between July 2000 and November 2019 during a 4-week inpatient PR program at the Schoen Klinik Berchtesgadener Land (Schoenau, Germany)
Results of the logistic regression analysis showed that none of the included variables was a significant predictor related to the improvements in 6-min walk distance (6MWD) or quality of life
Summary
Lymphangioleiomyomatosis (LAM) is a very rare, systemic neoplastic disease associated with progressive cystic lung destruction mostly affecting young women (prevalence: 3.4 to 7.8 per million women) [1]. LAM results in airflow limitation, hyperinflation, and reduced diffusion capacity which in turn leads to dyspnea and impaired exercise performance, physical activity, and quality of life [2, 3]. Available drugs (mTOR-inhibitor) may slow down lung destruction,
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