Pulmonary rehabilitation in lymphangioleiomyomatosis: a controlled clinical trial

Abstract

Lymphangioleiomyomatosis (LAM) is a cystic lung disease frequently associated with reduced exercise capacity. The aim of this study was to assess safety and efficacy of pulmonary rehabilitation in LAM.This controlled clinical trial included 40 patients with LAM and a low physical activity level. The pulmonary rehabilitation programme comprised 24 aerobic and muscle strength training sessions and education. The primary outcome was exercise capacity (endurance time during a constant work rate exercise test). Secondary outcomes included health-related quality of life (St George's Respiratory Questionnaire (SGRQ)), 6-min walking distance (6MWD), dyspnoea, peak oxygen consumption (V'O2 ), daily physical activity (pedometer), symptoms of anxiety and depression, lung function and peripheral muscle strength (one-repetition maximum).The baseline characteristics were well balanced between the groups. The pulmonary rehabilitation group exhibited improvements in the following outcomes versus controls: endurance time (median (interquartile range) 169 (2-303) s versus -33 (-129-39) s; p=0.001), SGRQ (median (interquartile range) -8 (-16-2) versus 2 (-4-5); p=0.002) and 6MWD (median (interquartile range) 59 (13-81) m versus 20 (-12-30) m; p=0.002). Dyspnoea, peak V'O2 , daily physical activity and muscle strength also improved significantly. No serious adverse events were observed.Pulmonary rehabilitation is a safe intervention and improves exercise capacity, dyspnoea, daily physical activity, quality of life and muscle strength in LAM.

Effects of yoga on exercise capacity in patients with lymphangioleiomyomatosis: a nonrandomized controlled study

“…Such therapy can reduce dyspnea, increase exercise capacity, and improve quality of life. A controlled clinical trial consisting of 40 patients with LAM examined the effects of pulmonary rehabilitation [4]. The pulmonary rehabilitation group exhibited improved exercise endurance time, quality of life, 6MWD, and peak oxygen consumption.…”

“…Pulmonary rehabilitation consisting of a 24-week aerobic exercise and muscle strength training significantly improved endurance time, St. George Respiratory Questionnaire (SGRQ) scores, 6-min walking distance (6MWD), dyspnea and peak oxygen consumption (VO 2 peak) [4]. Yoga is an attractive alternative option of pulmonary rehabilitation for LAM patients.…”

Current best practice in rehabilitation in interstitial lung disease

“…Although robust studies of PR are difficult to conduct in the rarer ILDs, consistent evidence of benefit continues to emerge. Recently, beneficial effects of PR in patients with lymphangioleiomyomatosis (LAM) were reported [Araujo et al 2016]. Forty patients with LAM performed 24-exercise training sessions over 12 weeks and received disease-specific education.…”

Lymphangioleiomyomatosis

“…In a controlled study involving 40 patients, 3 months of pulmonary rehabilitation improved exercise endurance time, 6-minute walking distance (6MWD), quality of life, and peak oxygen consumption. 71 Pulmonary rehabilitation should be incorporated into the standard care for LAM patients.…”