Chest
Original ResearchLymphangioleiomyomatosisReversible Airflow Obstruction in Lymphangioleiomyomatosis
Section snippets
Study Populations
For the retrospective analysis, the population comprised 235 patients with LAM who had at least five visits to the National Institutes of Health (NIH) [National Heart, Lung, and Blood Institute Protocol 95-H-0186]. For the prospective study, the population consisted of 106 patients participating in the same protocol at NIH (Bethesda, MD) and 24 patients who were followed up at the Ospedale San Giuseppe (Milan, Italy). Physiologic data from 21 of the 106 NIH patients participating in the
Retrospective Study
Age and pulmonary function data of the population are listed in Table 1. Of the study population, 211 were white, 13 were African American, 9 were Asian, and 2 were Hispanic. The number of visits per patient was 9.8 ± 0.3 (range, 5 to 22), for a total of 2,307 visits. The response to a β-adrenergic bronchodilator was tested at all visits.
Bronchodilator Response, Severity of Lung Disease, and Decline in Lung Function
Twenty-eight patients (12%) responded to albuterol at ≥ 50% of the visits, 93 patients (40%) responded < 50% of the time, and 114 patients (48%) never
Discussion
Our retrospective analysis showed that a greater frequency of a positive bronchodilator response to β-adrenergic bronchodilators was associated with worse lung function and a greater rate of decline in FEV1 and Dlco. Although patients with worse lung function were more likely to be treated with bronchodilators, we found no evidence that the use of these agents changed the rate of decline in lung function.
The prospective study confirmed that responders to bronchodilators have greater rates of FEV
Acknowledgments
Author contributions: Drs. Taveira-DaSilva and Moss were responsible for writing the manuscript, with assistance from Drs. Steagall and Stylianou. Drs. Steagall and Stylianou carried out most of the data analysis for the retrospective study. Ms. Hathaway and Ms. Rabel collected and organized the demographic data for both the retrospective and the prospective studies. Drs. Harari and Cassandro performed the studies and organized the data for the Italian patients. Dr. Stylianou wrote the
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2016, Clinics in Chest MedicineCitation Excerpt :Cerebral involvement may cause epilepsy, learning difficulties, autism, and obsessive-compulsive disorder, and there may be skin lesions, including facial angiofibromas, which may require treatment for cosmetic reasons. Approximately 25% to 30% of LAM patients have a significant bronchodilator response either to β-adrenergic or anticholinergic agents.74,78 In those with airflow obstruction who respond to bronchodilators, use of short-acting bronchodilators is recommended during exacerbations associated with upper respiratory infections, before exercise, or every 6 hours as recommended for other obstructive lung diseases.
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2016, Pharmacology and TherapeuticsCitation Excerpt :Patients with S-LAM often have poorer pulmonary function and a higher frequency of pulmonary nodules that those with TSC-LAM, whereas patients with TSC-LAM have a higher frequency of pneumothorax and higher incidence of AMLs (Taveira-DaSilva et al., 2010, 2015; Tobino et al., 2015). Bronchodilators have been used to ease airflow obstruction in patients with LAM with mixed results with between 6% and 30% of patients responding to inhalation of treatments such as albuterol and ipratropium (Taveira-DaSilva et al., 2009; Baldi et al., 2013). One of the reasons for the variation in responses between studies may be the ways in which the drugs are administered (e.g., nebulised versus metered dose inhaler).
Funding/Support: This study was funded in part by the Intramural Research Program, National Heart, Lung, and Blood Institute, National Institutes of Health.
Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/site/misc/reprints.xhtml).