Lymphangioleiomyomatosis (LAM) is a rare disease in which reduced exercise capacity is frequently present. The mechanisms applied are airflow obstruction, abnormal diffusion capacity and dynamic hyperinflation (DH).

Pulmonary rehabilitation (PR) has proved benefit in improving exercise tolerance, dyspnea, and quality of life in chronic obstructive pulmonary disease. There are no studies evaluating the impact of PR in patients with LAM.

The hypothesis under study is that PR determine improvement in exercise capacity, dyspnea, quality of life, muscle force, functional limitation and DH in these patients.

Inclusion Criteria:

• Diagnosis of lymphangioleiomyomatosis established by tissue biopsy and / or a combination of history and high-resolution computed tomography scanning
• Clinical stability, defined as no exacerbations or hospitalizations related to the underlying disease for a minimum of 6 weeks and no change in the treatment regimen in the last 3 months
• Low level of physical activity, defined as patients who walk less than 10 thousand daily steps and / or are not performing regular physical activity for at least 4 months before the start of rehabilitation
• Signature of the free, prior and informed consent for participation in the study

Exclusion Criteria:

• Lung transplant recipients
• Musculoskeletal disorders that would prevent the patient from performing exercise training
• Severe (NYHA IV) or uncontrolled heart disease