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Original Research: LYMPHANGIOLEIOMYOMATOSIS |

Reversible Airflow Obstruction in Lymphangioleiomyomatosis

Angelo M. Taveira-DaSilva, MD, PhD; Wendy K. Steagall, PhD; Antoinette Rabel, CRNP; Olanda Hathaway, CRNP; Sergio Harari, MD; Roberto Cassandro, MD; Mario Stylianou, PhD; Joel Moss, MD, PhD
Author and Funding Information

Affiliations: From the Translational Medicine Branch (Drs. Taveira-DaSilva, Steagall, and Moss, Ms. Rabel, and Ms. Hathaway) and the Office of Biostatistics Research (Dr. Stylianou), National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD; and Unità di Pneumologia e Terapia Semi-Intensiva Respiratoria (Drs. Harari and Cassandro), Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe, Milan, Italy.

Correspondence to: Angelo M. Taveira-DaSilva, MD, PhD, Translational Medicine Branch, NHLBI, NIH, Building 10, Room 6D05, MSC 1590, Bethesda, MD 20892-1590; e-mail: dasilvaa@nhlbi.nih.gov


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).


© 2009 American College of Chest Physicians


Chest. 2009;136(6):1596-1603. doi:10.1378/chest.09-0624
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Background:  We previously reported that approximately one-fourth of patients with lymphangioleiomyomatosis (LAM) may respond to therapy with bronchodilators. However, the validity of those observations has been questioned. The aims of the present study were to determine the prevalence of reversible airflow obstruction in patients with LAM and to identify associated clinical and physiologic parameters.

Methods:  First, the clinical and physiologic characteristics of 235 patients were analyzed to determine the frequency of the response to albuterol during a total of 2,307 visits. Second, we prospectively evaluated the response to albuterol (2.5 mg) and ipratropium (500 μg) in 130 patients, and correlated their responses with their clinical and physiologic characteristics.

Results:  In the retrospective study, 51% of the patients responded at least once to bronchodilators; of these, 12% responded ≥ 50% of the time. A higher frequency of positive bronchodilator responses was associated with greater rates of decline in FEV1 and diffusing capacity of the lung for carbon monoxide (Dlco). In the prospective study, 39 patients (30%) responded to bronchodilators, including 12 to ipratropium, 9 to albuterol, and 18 to both. The prevalence of asthma and smoking in the 39 responders was not different from that seen in the 91 nonresponders. Patients who responded to ipratropium, albuterol, or both had significantly (p < 0.02) lower FEV1 and Dlco, and a greater rate of FEV1 decline (p = 0.044) and Dlco decline (p = 0.039) than patients who did not respond to these bronchodilators. After adjusting for FEV1/FVC ratio, Dlco decline also was greater in responders than in nonresponders (p = 0.009).

Conclusions:  Patients with LAM may have partially reversible airflow obstruction. A positive response to bronchodilators is associated with an accelerated rate of decline in pulmonary function.

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