Mechanisms of exercise limitation and pulmonary rehabilitation in patients with cystic lung diseases

Reduced exercise capacity is common and multifactorial, including ventilatory, cardiocirculatory, and peripheral limitations, pulmonary hypertension (PH), and impaired gas exchange in LAM and PLCH. Variables from field tests are correlated with pulmonary function tests, and PR is safe and beneficial in LAM. Further studies are necessary to evaluate exercise in other DCLDs, the impact of other therapeutic modalities on DH, hypoxemia, and exercise-induced PH in LAM and PLCH, and the safety and benefits of PR mostly in PLCH.