Pulmonary Rehabilitation in Lymphangioleiomyomatosis

University of Sao Paulo General Hospital

Status: Completed

Conditions: Lymphangioleiomyomatosis

Treatments: Other: Pulmonary Rehabilitation

Study type: Interventional

Funder types: Other

Identifiers

NCT02009241

22729413.6.0000.0068

Details and patient eligibility

About

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.

Enrollment: 40 patients

Sex: Female

Volunteers: No Healthy Volunteers

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

Trial design

Primary purpose: Supportive Care

Allocation: Non-Randomized

Interventional model: Parallel Assignment

Masking: None (Open label)

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40 participants in 2 patient groups

Pulmonary Rehabilitation

Active Comparator group

Description:

The intervention group will perform a 12 week pulmonary rehabilitation program consisting of 30 minutes of treadmill aerobic exercise training and 30 minutes of muscle strength training.

Treatment:

Other: Pulmonary Rehabilitation

Control

No Intervention group