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Correspondence |

Variability in the Prevalence of Acute Bronchoresponsiveness in Different Populations of Patients With Lymphangioleiomyomatosis Response FREE TO VIEW

Charles D. Burger, MD, FCCP
Author and Funding Information

Affiliations: Mayo Clinic, Jacksonville, FL,  University of Cincinnati, Cincinnati, OH

Correspondence to: Charles D. Burger, MD, FCCP, Division of Pulmonary Medicine and Department of Critical Care, Mayo Clinic, 4500 San Pablo Rd, Jacksonville, FL 32224; e-mail: burger.charles@mayo.edu


Chest. 2008;134(1):217-218. doi:10.1378/chest.08-0454
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Published online

I read with great interest the review by McCormack1 on lymphangioleiomyomatosis (LAM). The article was well written and provides a substantive update from an expert in this disease.

Two comments merit consideration. First, the experience with acute bronchoreactivity may be more variable than the 17% quoted in the article. A review of our experience2 demonstrated that only 3 of 50 LAM patients (6%) had acutely reversible airway obstruction as measured by improvement in FEV1 after inhalation of a β-agonist. Review of the medical records of these three patients indicated little in the way of symptomatic relief with long-term use of metered-dose inhalers. Confounding issues may include stage and severity of disease, smoking history, and predominant physiology (obstructive vs restrictive vs mixed). Although we did not confirm an influential role of these other factors, study limitations preclude any firm conclusions.

Next, a case report of LAM in a man who did not have tuberous sclerosis was published recently.3 The report introduces the possibility that LAM is not exclusively a disease of women, although more study is necessary.

The author has no conflict of interest to disclose.

The author has reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

McCormack, FX (2008) Lymphangioleiomyomatosis: a clinical update.Chest133,507-516
 
Yen, KT, Putzke, JD, Staats, BA, et al The prevalence of acute response to bronchodilator in pulmonary lymphangioleiomyomatosis.Respirology2005;10,643-648
 
Schiavina, M, Di Scioscio, V, Contini, P, et al Pulmonary lymphangioleiomyomatosis in a karyotypically normal man without tuberous sclerosis complex.Am J Respir Crit Care Med2007;176,96-98
 
To the Editor:

We appreciate the comments of Dr. Burger. The National Heart, Lung, and Blood Institute Lymphangioleiomyomatosis Registry collected quality-controlled pulmonary function data on 230 patients with lymphangioleiomyomatosis (LAM) from around the United States.1Thirty-eight percent of patients were receiving therapy with bronchodilators at the time of study enrollment, and 46% of patients reported symptoms of wheezing. Approximately 17% of patients with LAM had evidence of a bronchodilator response on their baseline pulmonary function test results, as defined by an increase in FEV1 of at least 12% and 200 mL after the inhalation of a β-agonist. Of the 57% of patients (n = 125) who had obstructive physiology at baseline, almost 30% had a bronchodilator response. The prevalence of airway reactivity in this cohort was similar to that in prior reports from the United States,3 although the populations in those studies were partially overlapping. The ongoing Multicenter International LAM Efficacy of Sirolimus trial (MILES NCT 00414648) is collecting data on baseline bronchodilator responsiveness in up to 120 patients. We agree with Dr. Burger’s assessment of the variables, which could account for the differences between his patients and other populations.

We were aware of the occurrence of biopsy-documented LAM in men, and referenced the case report mentioned by Dr. Burger in our article.1 No cases of LAM were found as a result of the high-resolution CT scan screening of 11 patients with tuberous sclerosis complex (TSC) at the National Institutes of Health4 and 12 patients in our clinic (unpublished data). Thus, while it is clear that LAM occurs in men with TSC, the prevalence is almost certainly much less than the 30 to 40% reported in women with TSC.46 Like the association with TSC, female gender restriction, and metastatic potential, the rare occurrence of LAM in men is a precious clue from nature that has the potential to shed new light on the pathogenesis of this disease.

References
Ryu, JH, Moss, J, Beck, GJ, et al The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment.Am J Respir Crit Care Med2006;173,105-111
 
Chu, SC, Horiba, K, Usuki, J, et al Comprehensive evaluation of 35 patients with lymphangioleiomyomatosis.Chest1999;115,1041-1052
 
Taveira-DaSilva, AM, Hedin, C, Stylianou, MP, et al Reversible airflow obstruction, proliferation of abnormal smooth muscle cells, and impairment of gas exchange as predictors of outcome in lymphangioleiomyomatosis.Am J Respir Crit Care Med2001;164,1072-1076
 
Moss, J, Avila, NA, Barnes, PM, et al Prevalence and clinical characteristics of lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex.Am J Respir Crit Care Med2001;164,669-671
 
Franz, DN, Brody, A, Meyer, C, et al Mutational and radiographic analysis of pulmonary disease consistent with lymphangioleiomyomatosis and micronodular pneumocyte hyperplasia in women with tuberous sclerosis.Am J Respir Crit Care Med2001;164,661-668
 
Costello, LC, Hartman, TE, Ryu, JH High frequency of pulmonary lymphangioleiomyomatosis in women with tuberous sclerosis complex.Mayo Clin Proc2000;75,591-594
 

Figures

Tables

References

McCormack, FX (2008) Lymphangioleiomyomatosis: a clinical update.Chest133,507-516
 
Yen, KT, Putzke, JD, Staats, BA, et al The prevalence of acute response to bronchodilator in pulmonary lymphangioleiomyomatosis.Respirology2005;10,643-648
 
Schiavina, M, Di Scioscio, V, Contini, P, et al Pulmonary lymphangioleiomyomatosis in a karyotypically normal man without tuberous sclerosis complex.Am J Respir Crit Care Med2007;176,96-98
 
Ryu, JH, Moss, J, Beck, GJ, et al The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment.Am J Respir Crit Care Med2006;173,105-111
 
Chu, SC, Horiba, K, Usuki, J, et al Comprehensive evaluation of 35 patients with lymphangioleiomyomatosis.Chest1999;115,1041-1052
 
Taveira-DaSilva, AM, Hedin, C, Stylianou, MP, et al Reversible airflow obstruction, proliferation of abnormal smooth muscle cells, and impairment of gas exchange as predictors of outcome in lymphangioleiomyomatosis.Am J Respir Crit Care Med2001;164,1072-1076
 
Moss, J, Avila, NA, Barnes, PM, et al Prevalence and clinical characteristics of lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex.Am J Respir Crit Care Med2001;164,669-671
 
Franz, DN, Brody, A, Meyer, C, et al Mutational and radiographic analysis of pulmonary disease consistent with lymphangioleiomyomatosis and micronodular pneumocyte hyperplasia in women with tuberous sclerosis.Am J Respir Crit Care Med2001;164,661-668
 
Costello, LC, Hartman, TE, Ryu, JH High frequency of pulmonary lymphangioleiomyomatosis in women with tuberous sclerosis complex.Mayo Clin Proc2000;75,591-594
 
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