Pulmonary Rehabilitation in Lymphangioleiomyomatosis

Lymphangioleiomyomatosis Clinical Trial

Official title:

Evaluation of the Impact of a Pulmonary Rehabilitation Program on Exercise Capacity in Patients With Lymphangioleiomyomatosis

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT02009241
• Other study ID #: 22729413.6.0000.0068
• Status: Completed
• Phase: N/A
• First received: December 4, 2013
• Last updated: December 9, 2015
• Start date: November 2013
• Est. completion date: May 2015

Study information

• Verified date: December 2015
• Source: University of Sao Paulo General Hospital
• Contact: n/a
• Is FDA regulated: No
• Health authority: Brazil: National Committee of Ethics in Research
• Study type: Interventional

Clinical Trial 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.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 40
• Est. completion date: May 2015
• Est. primary completion date: April 2015
• Accepts healthy volunteers: No
• Gender: Female
• Age group: N/A and older

Eligibility

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

Allocation: Non-Randomized, Endpoint Classification: Efficacy Study, Intervention Model: Parallel Assignment, Masking: Open Label, Primary Purpose: Supportive Care

Related Conditions & MeSH terms

• Lymphangioleiomyomatosis

Intervention

Other:
• Pulmonary Rehabilitation

Locations

Country	Name	City	State
Brazil	Pulmonary Division, Heart Institute (InCor), University of Sao Paulo Medical School	Sao Paulo	SP

Sponsors (1)

Lead Sponsor	Collaborator
University of Sao Paulo General Hospital

Country where clinical trial is conducted

Brazil, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Endurance time during constant work rate cycle ergometry		12 weeks	No
Secondary	Peak oxygen consumption (VO2 peak) during constant work rate cycle ergometry		12 weeks	No
Secondary	Dynamic hyperinflation and ventilatory parameters during constant work rate cycle ergometry		12 weeks	No
Secondary	Dyspnea and fatigue in the lower limbs during constant work rate cycle ergometry		12 weeks	No
Secondary	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	12 weeks	No
Secondary	Health factors related to quality of life	St. George's Respiratory Questionnaire (SGRQ) pre - and post - intervention	12 weeks	No
Secondary	Anxiety and depression	Hospital Anxiety and Depression Scale (HADS) performed pre - and post - intervention	12 weeks	No
Secondary	Daily physical activity	Evaluated using a pedometer for one week pre - and post - intervention	12 weeks	No
Secondary	Changes in six minute walking distance and in desaturation - distance ratio (DDR)		12 weeks	No
Secondary	Changes in pulmonary function parameters		12 weeks	No
Secondary	Changes in peripheral muscle force	Using one repetition maximum (1 RM) pre - and post - intervention	12 weeks	No