Pulmonary Rehabilitation in Lymphangioleiomyomatosis

Not Applicable

Completed

• Conditions: Lymphangioleiomyomatosis
• Interventions: Other: Pulmonary Rehabilitation

• Registration Number: NCT02009241
• Lead Sponsor: University of Sao Paulo General Hospital

Brief Summary

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.

Detailed Description

Not available

Study Timeline

• Study Start: 2013-11
• Study First Submitted: 2013-12-04
• Study First Submitted QC: 2013-12-08
• Study First Posted: 2013-12-11
• Primary Completion: 2015-04
• Study Completion: 2015-05
• Status Verified: 2015-12
• Last Update Submitted: 2015-12-09
• Last Update Posted: 2015-12-11

Recruitment & Eligibility

• Status: COMPLETED
• Sex: Female
• Target Recruitment: 40

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

Study & Design

• Study Type: INTERVENTIONAL
• Study Design: PARALLEL

Arm && Interventions

Group	Intervention	Description
Pulmonary Rehabilitation	Pulmonary Rehabilitation	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.

Primary Outcome Measures

Name	Time	Method
Endurance time during constant work rate cycle ergometry	12 weeks

Secondary Outcome Measures

Name	Time	Method
Daily physical activity	12 weeks	Evaluated using a pedometer for one week pre - and post - intervention
Peak oxygen consumption (VO2 peak) during constant work rate cycle ergometry	12 weeks
Dynamic hyperinflation and ventilatory parameters during constant work rate cycle ergometry	12 weeks
Anxiety and depression	12 weeks	Hospital Anxiety and Depression Scale (HADS) performed pre - and post - intervention
Changes in pulmonary function parameters	12 weeks
Dyspnea and functional disability related to daily life activities	12 weeks	The Modified Medical Research Council Dyspnea Scale (mMRC) and Baseline Dyspnea Index (BDI) will be evaluated pre - and post - intervention
Health factors related to quality of life	12 weeks	St. George's Respiratory Questionnaire (SGRQ) pre - and post - intervention
Changes in six minute walking distance and in desaturation - distance ratio (DDR)	12 weeks
Dyspnea and fatigue in the lower limbs during constant work rate cycle ergometry	12 weeks
Changes in peripheral muscle force	12 weeks	Using one repetition maximum (1 RM) pre - and post - intervention

Trial Locations

Locations (1)

Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School; 🇧🇷 Sao Paulo, SP, Brazil