Clinical trial NCT02009241: Pulmonary Rehabilitation in Lymphangioleiomyomatosis
Official title: Evaluation of the Impact of a Pulmonary Rehabilitation Program on Exercise Capacity in Patients With Lymphangioleiomyomatosis
Study type: INTERVENTIONAL
Overall status: COMPLETED
Phase: NA
Conditions: Lymphangioleiomyomatosis
Keywords: Lymphangioleiomyomatosis
Rehabilitation
Exercise
Lead sponsor: University of Sao Paulo General Hospital
Sponsor class: OTHER
First posted: 2013-12-11
Enrollment: 40
Sex: FEMALE
Standard ages: CHILD
ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Allocation: NON_RANDOMIZED
Intervention model: PARALLEL
Primary purpose: SUPPORTIVE_CARE

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.


Eligibility criteria: 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

Primary outcomes:
measure: Endurance time during constant work rate cycle ergometry
timeFrame: 12 weeks
Secondary outcomes:
measure: Peak oxygen consumption (VO2 peak) during constant work rate cycle ergometry
timeFrame: 12 weeks
measure: Dynamic hyperinflation and ventilatory parameters during constant work rate cycle ergometry
timeFrame: 12 weeks
measure: Dyspnea and fatigue in the lower limbs during constant work rate cycle ergometry
timeFrame: 12 weeks
measure: Dyspnea and functional disability related to daily life activities
timeFrame: 12 weeks
measure: Health factors related to quality of life
timeFrame: 12 weeks
measure: Anxiety and depression
timeFrame: 12 weeks
measure: Daily physical activity
timeFrame: 12 weeks
measure: Changes in six minute walking distance and in desaturation - distance ratio (DDR)
timeFrame: 12 weeks
measure: Changes in pulmonary function parameters
timeFrame: 12 weeks
measure: Changes in peripheral muscle force
timeFrame: 12 weeks
Other outcomes:
Locations:
city: Sao Paulo
country: Brazil
facility: Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School
state: SP
Study officials:
affiliation: Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School
name: Mariana S Araujo, MD
role: PRINCIPAL_INVESTIGATOR
affiliation: Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School
name: Carlos RR Carvalho, PhD
role: PRINCIPAL_INVESTIGATOR
References:
citation: Araujo MS, Baldi BG, Freitas CS, Albuquerque AL, Marques da Silva CC, Kairalla RA, Carvalho CR, Carvalho CR. Pulmonary rehabilitation in lymphangioleiomyomatosis: a controlled clinical trial. Eur Respir J. 2016 May;47(5):1452-60. doi: 10.1183/13993003.01683-2015. Epub 2016 Feb 25.