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Original Research: INTERSTITIAL LUNG DISEASE |

Effect of a Gonadotrophin-Releasing Hormone Analogue on Lung Function in Lymphangioleiomyomatosis*

Sergio Harari, MD; Roberto Cassandro, MD; Iacopo Chiodini, MD; Angelo M. Taveira-DaSilva, MD, PhD; Joel Moss, MD, PhD
Author and Funding Information

*From the Unità di Pneumologia e Terapia Semi-Intensiva Respiratoria (Drs. Harari and Cassandro), Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, and Unidade Operativa di Endocrinologia (Dr. Chiodini), Ospedale San Giuseppe AFAR, Milan, Italy; and Translational Medicine Branch (Drs. Taveira-DaSilva and Moss), National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD.

Correspondence to: Angelo M. Taveira-DaSilva, MD, PhD, Translational Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Building 10, Room 6D05, MSC 1590, Bethesda, MD 20892-1590; e-mail: dasilvaa@nhlbi. nih.gov



Chest. 2008;133(2):448-454. doi:10.1378/chest.07-2277
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Background: Lymphangioleiomyomatosis (LAM), a multisystem disease occurring primarily in women, is characterized by cystic lung destruction, and kidney and lymphatic tumors, caused by the proliferation of abnormal-appearing cells (ie, LAM cells) with a smooth muscle cell phenotype that express melanoma antigens and are capable of metastasizing. Estrogen receptors are present in LAM cells, and this finding, along with reports of disease progression during pregnancy or following exogenous estrogen administration, suggest the involvement of estrogens in the pathogenesis of LAM. Consequently, antiestrogen therapies have been employed in treatment. The goal of this prospective study was to evaluate the efficacy of triptorelin, a gonadotrophin-releasing hormone analogue, in 11 premenopausal women with LAM.

Methods: Patients were evaluated at baseline and every 3 to 6 months thereafter, for a total of 36 months. Hormonal assays, pulmonary function tests, 6-min walk tests, high-resolution CT scans of the chest, and bone mineral density studies were performed.

Results: Gonadal suppression was achieved in all patients. Overall, a significant decline in lung function was observed; two patients underwent lung transplantation 1 year after study enrollment, and another patient was lost to follow-up. Treatment with triptorelin was associated with a decline in bone mineral density.

Conclusions: Triptorelin appears not to prevent a decline in lung function in patients with LAM. Its use, however, may be associated with the loss of bone mineral density.

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