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Published on: May 1, 2015
Reversible airflow obstruction in lymphangioleiomyomatosis
Angelo M Taveira-DaSilva1, Wendy K Steagall1, Antoinette Rabel1
1Translational Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD.
Chest
|May 19, 2009
Summary
Patients with lymphangioleiomyomatosis (LAM) may experience reversible airflow obstruction. Bronchodilator response in LAM is linked to a faster decline in lung function, indicating disease progression.
Area of Science:
- Pulmonary Medicine
- Rare Diseases
Background:
- Lymphangioleiomyomatosis (LAM) is a rare lung disease.
- Previous findings on bronchodilator response in LAM were inconsistent.
- The study aimed to clarify bronchodilator reversibility prevalence and associated factors in LAM.
Purpose of the Study:
- Determine the prevalence of reversible airflow obstruction in LAM patients.
- Identify clinical and physiological parameters associated with bronchodilator response.
- Investigate the relationship between bronchodilator response and disease progression in LAM.
Main Methods:
- Retrospective analysis of 235 LAM patients' clinical and physiological data.
- Prospective evaluation of bronchodilator response (albuterol and ipratropium) in 130 LAM patients.
- Correlation of bronchodilator response with pulmonary function tests (FEV1, Dlco) and decline rates.
Main Results:
- 51% of patients showed at least one positive bronchodilator response in retrospective analysis.
- 30% of patients responded to bronchodilators in the prospective study.
- Responders exhibited significantly lower FEV1 and Dlco, and accelerated FEV1 and Dlco decline rates compared to non-responders.
Conclusions:
- Partially reversible airflow obstruction can occur in patients with LAM.
- A positive bronchodilator response in LAM is associated with a more rapid decline in pulmonary function.
- These findings suggest bronchodilator response may indicate a more aggressive disease course in LAM.
