This Article Full Text Full Text (PDF) All Versions of this Article: chest.07-2277v1 133/2/448    most recent Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in ISI Web of Science Similar articles in PubMed Alert me to new issues of the journal Add to My Personal Article Archive Download to citation manager Citing Articles Citing Articles via ISI Web of Science (1) Citing Articles via Google Scholar Google Scholar Articles by Harari, S. Articles by Moss, J. Search for Related Content PubMed PubMed Citation Articles by Harari, S. Articles by Moss, J.

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

^* 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{at}nhlbi nih.gov

Abstract

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.

Key Words: gonadotrophin-releasing hormone analogues • lung function • lymphangioleiomyomatosis • osteoporosis • triptorelin