© 2008 American Thoracic Society
S-LAM in Men: Is Pulmonary Function Different from That Seen in Women?From the Authors:We thank Dr. Sandrini and colleagues and Dr. Burger for their observations on our case report of sporadic lymphangioleiomyomatosis (S-LAM) in a male patient (1). We fully agree with Dr. Burger's comment on the relevance of accurately measuring pulmonary function in patients with S-LAM. At first clinical observation when the patient was referred as a consequence of a spontaneous pneumothorax, he presented with lung function within normal limits: FVC was 5.4 L (105% of predicted), FEV1 was 4.2 L (99% of predicted), and the carbon monoxide transfer was 36.5 ml/min per mm Hg (103% of predicted). A diagnosis of LAM with normal lung function is compatible with an early stage of the disease, as it was in our case. This observation is also supported by our past experience, based on the data of 51 female patients with LAM evaluated in our center: about one-third of them received a diagnosis of LAM at a time when the pulmonary function tests were within normal limits. Obviously, this is not in contrast with the findings reported by Dr. Burger that a significant fraction of patients show an obstructive pattern (as it was in fact in 44% of our 51 female patients). Therefore, in our male patient with LAM, the absence of any measurable airway obstruction did not allow us to test the presence of an acute bronchial reactivity to β-agonists. As a consequence, we are unable to provide any further support to the interesting hypothesis that male patients with LAM may respond in a different way to any medical therapy, and we are looking forward to reporting the longitudinal follow-up of the clinical and HRCT data for this patient. Dr. Burger's observation further underlines the need for large prospective collaborative international registries to achieve a better knowledge of the natural history of LAM and response to therapies. In this context, the experience reported in Sandrini and colleagues' and Burger's letters certainly represents an illuminating example on how to build a strong network of scientists and chest physicians committed to unraveling the secrets of this fascinating disease.
Azienda Ospedaliera S. Orsola-Malpighi FOOTNOTES Conflict of Interest Statement: None of the authors has a financial relationship with a commercial entity that has an interest in the subject of this manuscript. REFERENCES
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