Characterization of the Pathogenesis of Lymphangioleiomyomatosis (LAM)

Inclusion 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.

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.