RECRUITING Phase 1 / Phase 2 ClinicalTrials.gov

Nebulized or Inhaled Albuterol for Lymphangioleiomyomatosis

Sponsor: National Heart, Lung, and Blood Institute (NHLBI) • ClinicalTrials.gov ID: NCT01799538

Registry updated: 2026-08-31

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Description

Background: \- Lymphangioleiomyomatosis (LAM) is a rare type of lung disease that occurs almost exclusively in women. In LAM, muscle tissue grows in the lungs and starts to block the flow of air. It is a progressive disease, and in severe cases may require a lung transplant. One possible treatment to improve breathing in people with LAM is inhaled albuterol. Albuterol can be given in a metered dose inhaler (MDI) or with a nebulizer. Researchers want to compare these methods to see which method best improves lung function in women with LAM. Objectives: \- To see whether a nebulizer or MDI can better improve lung function in women with LAM. Eligibility: \- Women at least 18 years of age who have impaired lung function because of LAM. Design: * Participants will be screened with a physical exam and medical history. No lab tests will be needed for this study. * Participants will have a 3-day overnight stay at the National Institutes of Health. Those who are using long-acting inhalers will have to stop taking these drugs 1 week before the study. * Participants will receive either the nebulizer or two or four puffs of the inhaler. Four puffs of albuterol is a higher dose than is normally prescribed, and is being tested on this study. * Participants will have each treatment around the same time of day on each of the 3 days. Before and after taking the albuterol, participants will have lung function tests.

Interventions

albuterol inhaler (Drug)
2 puffs of Metered Dose inhaler for Bronchodilation
albuterol nebulizer (Drug)
Albuterol administered by nebulization will produce a greater increase in FEV1
PFT (Procedure)
Breathing Test

Eligibility criteria

* INCLUSION CRITERIA: * Diagnosis of LAM either by tissue biopsy, evidence of lung and other organ involvement (renal angiomyolipomas, chylous effusions, lymphangioleiomyomas), high serum levels of vascular endothelial growth factor D (VGEF-D)(1) or a diagnosis of TSC associated with cystic lung lesions. * Age 18 years or over * Evidence of airflow obstruction: FEV1/VC ratio \< fifth percentile of predicted normal and an FEV(1) \<80% predicted of the normal values. EXCLUSION CRITERIA: Subjects will be excluded from the study if they meet one or more of the following criteria: * History of hypersensitivity to albuterol or any of its components. * Moderate or large pleural effusions (chest x-ray and or CT scan procedure completed under Protocol 95-H-0186) * History of seizures other than during infancy * Inability to withhold bronchodilators for 24 hours * Cognitive Impairment * Age less than 18 years * Male sex * Status-post lung or kidney transplantation * Pregnant or breast feeding (women of childbearing potential will undergo a blood or urine pregnancy test under Protocol 95-H-0186). * Treatment with monoaminoxidase inhibitors, tricyclic antidepressants or Beta-adrenergic receptor antagonists or long acting anticholinergic bronchodilators who are unable to be discontinued for at least seven days before enrollment. * Patients with URI, uncontrolled hyperthyroidism or severe gastro-esophageal reflux. Major systemic diseases (i.e., malignancy; myocardial infarction or unstable angina; type 1 diabetes, severe hypertension; liver cirrhosis).

Conditions

Lymphangioleiomyomatosis

Locations

National Institutes of Health Clinical Center — Bethesda, Maryland, United States

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Official Source

Data sourced from ClinicalTrials.gov, a service of the U.S. National Institutes of Health.

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