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American Journal of Respiratory and Critical Care Medicine Vol 177. pp. 356, (2008)
© 2008 American Thoracic Society


Correspondence

S-LAM in Men: Is Pulmonary Function Different from That Seen in Women?

To the Editor:

In their case report, Schiavina and colleagues (1) described a pathologically proven case of sporadic lymphangioleiomyomatosis (S-LAM) in a man. The report would have been more complete if it had included physiologic data describing the patient's pulmonary function. Such data are particularly important to determine any difference in the abnormalities expected in women with LAM and their response to treatment. The pulmonary function pattern in women with LAM is well described (2) and is generally obstructive in nature, although mixed restrictive and obstructive findings may occur as well. Pulmonary mechanics studies have isolated the obstructive lesion to the airway as opposed to loss of elastic recoil (3). In addition, acute bronchoreactivity to β-agonists is unusual in patients with severe disease (4).

Specific improvement in these physiologic abnormalities is necessary to determine response to treatment. In this case, "a decision was made based on the clinicians' judgment ... to use hormonal manipulation therapy" (1). The patient was said to be "clinically and functionally stable" (1). Generally, the clinical response to hormonal therapy is limited (5). Was this case different? If so, what sort of physiologic variables were measured and which ones were stable or improved?

I am very interested in this unique case but believe that the lack of physiologic data lessens the value of this report. I look forward to the authors' providing additional information that will enhance the body of literature on this condition, which is even rarer in men than in women.

Charles D. Burger

Mayo Clinic
Jacksonville, Florida

FOOTNOTES

Conflict of Interest Statement: C.D.B. does not have a financial relationship with a commercial entity that has an interest in the subject of this manuscript.

REFERENCES

  1. Schiavina M, Di Scioscio V, Contini P, Cavazza A, Fabiani A, Barberis M, Bini A, Altimari A, Cooke RMT, Grigioni WF, et al. Pulmonary lymphangioleiomyomatosis in a karyotypically normal man without tuberous sclerosis complex. Am J Respir Crit Care Med 2007;176:96–98.[Abstract/Free Full Text]
  2. Ryu JH, Moss J, Beck GJ, Lee JC, Brown KK, Chapman JT, Finlay GA, Olson EJ, Ruoss SJ, Maurer JR, et al. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. Am J Respir Crit Care Med 2006;173:105–111.[Abstract/Free Full Text]
  3. Burger CD, Hyatt RF, Staats BA. Pulmonary mechanics in lymphangioleiomyomatosis. Am Rev Respir Dis 1991;143:1030–1033.[Medline]
  4. Yen KT, Putzke JD, Staats BA, Burger CD. The prevalence of acute response to bronchodilator in pulmonary lymphangioleiomyomatosis. Respirology 2005;10:643–648.[CrossRef][Medline]
  5. Urban T, Lazor R, Lacronique J, Murris M, Labrune S, Valeyre D, Cordier JF; Groupe d'Etudes et de Recherche sur les Maladies "Orphelines" Pulmonaires (GERM"O"P). Pulmonary lymphangioleiomyomatosis: a study of 69 patients. Medicine (Baltimore) 1999;78:321–337.[CrossRef][Medline]




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HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS
Proc. Am. Thorac. Soc. Am. J. Respir. Cell Mol. Biol.
Copyright © 2008 American Thoracic Society