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Completed

NCT Number: NCT02009241

Pulmonary Rehabilitation in Lymphangioleiomyomatosis

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.

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Key information

Conditions

Lymphangioleiomyomatosis Behavior Hemic and Lymphatic Diseases Immune System Diseases Immunoproliferative Disorders Lymphangiomyoma Lymphatic Diseases Lymphoproliferative Disorders Motor Activity Neoplasm, Lymphatic Tissue Neoplasms Neoplasms by Histologic Type Neoplasms, Connective and Soft Tissue Perivascular Epithelioid Cell Neoplasms

Sex eligibility

Female

Study type

Interventional

Phase

Not applicable

Primary location

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

São Paulo, 05403-000, Brazil

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

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

Treatment and study plan

Pulmonary Rehabilitation

Other

Primary outcomes

  1. Endurance time during constant work rate cycle ergometry

    Time frame: 12 weeks

Secondary outcomes

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

    Time frame: 12 weeks

  2. Dynamic hyperinflation and ventilatory parameters during constant work rate cycle ergometry

    Time frame: 12 weeks

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

    Time frame: 12 weeks

  4. Dyspnea and functional disability related to daily life activities

    Time frame: 12 weeks

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

  5. Health factors related to quality of life

    Time frame: 12 weeks

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

  6. Anxiety and depression

    Time frame: 12 weeks

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

  7. Daily physical activity

    Time frame: 12 weeks

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

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

    Time frame: 12 weeks

  9. Changes in pulmonary function parameters

    Time frame: 12 weeks

  10. Changes in peripheral muscle force

    Time frame: 12 weeks

    Using one repetition maximum (1 RM) pre - and post - intervention

Sponsors and collaborators

Lead sponsor

University of Sao Paulo General Hospital

Other

Registry information

Official study title

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

Important dates

Study start
2013
Primary completion
2015
Study completion
2015
First posted
Dec 11, 2013
Registry last updated
Dec 11, 2015

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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