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Characterization of the Pathogenesis of Lymphangioleiomyomatosis (LAM)

16 Years
80 Years
Open (Enrolling)
Lung Disease, Pneumothorax, Tuberous Sclerosis

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Trial Information

Characterization of the Pathogenesis of Lymphangioleiomyomatosis (LAM)

Individuals with pulmonary lymphangioleiomyomatosis develop severe destructive lung disease.
Most of them are females of childbearing age. Currently, there is no proven effective
therapy and the prognosis is variable. This study is designed to (a) define the clinical
course of the disease and (b) elucidate the pathogenesis of the disease at the cellular and
molecular levels, in order to develop more effective therapy. To accomplish this, we intend
to identify the proteins and genes that contribute to the process of lung destruction in
affected individuals.

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 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

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.

Type of Study:


Study Design:


Principal Investigator

Joel Moss, M.D.

Investigator Role:

Principal Investigator

Investigator Affiliation:

National Heart, Lung, and Blood Institute (NHLBI)


United States: Federal Government

Study ID:




Start Date:

September 1995

Completion Date:

Related Keywords:

  • Lung Disease
  • Pneumothorax
  • Tuberous Sclerosis
  • Smooth Muscle Proliferation
  • Bronchoscopy
  • Female
  • Pneumothorax
  • Tuberous Sclerosis
  • Lymphangioleiomyomatosis (LAM)
  • Lung Diseases
  • Pneumothorax
  • Sclerosis
  • Tuberous Sclerosis
  • Lymphangioleiomyomatosis



National Institutes of Health Clinical Center, 9000 Rockville Pike Bethesda, Maryland  20892