Pulmonary Rehabilitation in Lymphangioleiomyomatosis

N/ACompletedNCT02009241

University of Sao Paulo General Hospital40 enrolled

Overview

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.

Study Type

INTERVENTIONAL

Allocation

NON_RANDOMIZED

Purpose

SUPPORTIVE_CARE

Masking

NONE

Enrollment

Conditions

Lymphangioleiomyomatosis

Interventions

Pulmonary RehabilitationOTHER

Eligibility

Sex: FEMALE

Medical Language ↔ Plain English

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

Locations (1)

Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School

São Paulo, São Paulo, Brazil

Outcomes

Primary Outcomes

Endurance time during constant work rate cycle ergometry

Time frame: 12 weeks

Secondary Outcomes

Peak oxygen consumption (VO2 peak) during constant work rate cycle ergometry

Time frame: 12 weeks

Dynamic hyperinflation and ventilatory parameters during constant work rate cycle ergometry

Time frame: 12 weeks

Dyspnea and fatigue in the lower limbs during constant work rate cycle ergometry

Time frame: 12 weeks

Dyspnea and functional disability related to daily life activities

The Modified Medical Research Council Dyspnea Scale (mMRC) and Baseline Dyspnea Index (BDI) will be evaluated pre - and post - intervention

Time frame: 12 weeks

Health factors related to quality of life

St. George's Respiratory Questionnaire (SGRQ) pre - and post - intervention

Time frame: 12 weeks

Anxiety and depression

Hospital Anxiety and Depression Scale (HADS) performed pre - and post - intervention

Time frame: 12 weeks

Daily physical activity

Evaluated using a pedometer for one week pre - and post - intervention

Time frame: 12 weeks

Changes in six minute walking distance and in desaturation - distance ratio (DDR)

Time frame: 12 weeks

Data from ClinicalTrials.gov

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