Study of the Disease Process of Lymphangioleiomyomatosis

Launched by NATIONAL HEART, LUNG, AND BLOOD INSTITUTE (NHLBI) · Nov 3, 1999

Keywords

ClinConnect Summary

This clinical trial is studying a lung disease called pulmonary lymphangioleiomyomatosis (LAM), which mainly affects women of childbearing age. The goal of the study is to understand how this disease causes damage to the lungs at a cellular and molecular level. By identifying specific proteins and genes involved in lung destruction, researchers hope to develop more effective treatments in the future. Currently, there are no effective therapies for LAM, and the outlook for patients can be quite serious.

To participate in this study, individuals must be between the ages of 16 and 65 and have specific findings related to LAM on lung biopsies or imaging tests like chest X-rays or CT scans. Pregnant or nursing women can participate, but certain procedures will be limited during pregnancy for safety. Participants can expect to undergo various tests to help researchers gather important information about this rare lung disease. If you or someone you know has LAM, this study might be a valuable opportunity to contribute to the understanding of the disease and potentially improve future treatments.

Gender

ALL

Eligibility criteria

• * INCLUSION CRITERIA:
• General admission criteria for patients include one or both of the following:
• • Findings on lung biopsy diagnostic of LAM;
• • Findings on chest x-ray and/or chest computed axial tomography consistent with LAM.
• • Patients with TSC and pulmonary LAM will be included in the study.
• • Normal non-smokers in the control group are defined as individuals who have not smoked for greater than or equal to 1 year and have no systemic or pulmonary disease.
• • Normal smokers defined as individuals with no systemic or pulmonary disease, who have smoked for greater than or equal to 1 year and have normal chest x-ray and normal pulmonary function tests may be included if needed as controls for a similar population of patients with LAM.
• • Pregnant and or nursing women can be included in accordance with Federal Regulations at Subpart B of 45 CFR 46 Subjects who are pregnant and or nursing will be excluded from procedures during their pregnancy that are greater than minimal risk, until they are no longer pregnant and/or nursing. Procedures that will not be completed while the subject is pregnant and/or nursing including: PFTs, Six Minute Walk Test, thoracentesis, bronchoscopy, and measurements with imaging modalities requiring contrast or with radiation exposure such as Chest x-ray, CT scan, MRI, bone densitometry (DEXA). Allowing subjects to be included in the study may glean important information about individuals with uncommon pulmonary disease during and post pregnancy.
• EXCLUSION CRITERIA:
• Exclusion criteria for patients include:
• • Age less than 16.
• • Advanced stage of a pulmonary or a systemic illness in which the risk of the study is judged to be significant even in the absence of a clear contraindication to the procedures.
• • Exclusion criteria for patients for the formal exercise study and the stress echocardiogram include patients on continuous oxygen. Patients may perform an exercise test that will assess the patient's exercise capacity with activities of daily living.