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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Lymphangioleiomyomatosis
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2016, Clinics in Chest MedicineCitation Excerpt :Patient groups are an excellent source of patient-specific support and on-line communities are accessible to those with all levels of disability. Pneumothorax, chylous effusions, worsening of respiratory symptoms, hemorrhage of angiomyolipomas, and premature births are over-represented during pregnancy in women with LAM.72–74 Pregnancy should be discouraged in patients with severe disease or those in whom lung function is declining rapidly.
Lymphangioleiomyomatosis: Current understanding and potential treatments
2016, Pharmacology and TherapeuticsCitation Excerpt :This is currently being investigated in a phase 2 clinical trial (NCT01799538; www.clinicaltrials.gov; Table 1). It should be noted that a positive response to bronchodilator treatment has been associated with an accelerated rate of decline in pulmonary function (Taveira-DaSilva et al., 2009). However, bronchodilators provide only symptomatic relief and are not a cure for LAM.
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).