Taveira-Dasilva AM, Steagall WK, Rabel A, Hathaway O, Harari S, Cassandro R, Stylianou M, Moss J
Reversible Airflow Obstruction in Lymphangioleiomyomatosis. [JOURNAL ARTICLE]
Chest 2009 May 15.
Background We previously reported that approximately one fourth of patients with lymphangioleiomyomatosis may respond to bronchodilators. A recent study, however, questioned the validity of those observations. The aims of this study were to determine the prevalence of reversible airflow obstruction in lymphangioleiomyomatosis, and to identify associated clinical and physiologic parameters. Methods First, clinical and physiologic characteristics of 235 patients were analyzed to determine the frequency of response to albuterol during a total of 2,307 visits. Second, we prospectively evaluated the response to albuterol (2.5 mg) and ipratropium (500 mug) in 130 patients and correlated 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. Higher frequency of positive bronchodilator responses was associated with greater rates of decline in FEV(1) and DL(CO). In the prospective study, thirty-nine (30%) responded to bronchodilators: 12 to ipratropium, 9 to albuterol, and 18 to both. The prevalence of asthma and smoking in 39 responders was not different from that seen in 91 nonresponders. Patients who responded to ipratropium and/or albuterol had significantly (p < 0.02) lower FEV(1) and DL(CO) and a greater rate of FEV1 (p = 0.044) and DL(CO) decline (p = 0.039). After adjusting for FEV1/FVC ratio, DL(CO) decline was also greater in responders than nonresponders (p = 0.009). Conclusions Patients with lymphangioleiomyomatosis 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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