Acta Endicronologica
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ACTA ENDOCRINOLOGICA (BUC)

The International Journal of Romanian Society of Endocrinology / Registered in 1938

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January - March 2005, Volume 1, Issue 1
Case Report

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Acta Endocrinologica (Buc)


Fica SV, Popescu L, Ciprut T, Ardeleanu C, Terzea D, Trifanescu R, Coculescu M

Beneficial effects of gonadotropin releasing hormone analogs in pulmonary lymphangioleiomyomatosis

Acta Endo (Buc) 2005, 1 (1): 109-119
doi: 10.4183/aeb.2005.109

OBJECTIVE: To report an unusual cause of respiratory failure in a 33-year old Caucasian woman, diagnosed at 26 years with pulmonary lymphangioleiomyomatosis (LAM) and treated with gonadoliberin analogs (aGnRH) four years.\r\nMETHODS: The respiratory failure was diagnosed on functional tests (spirometry, oxymetry, diffusing capacity of carbon monoxide). High resolution chest computed tomographic (HRCT) scan and open lung biopsy with specific immunohistochemistry certified the diagnosis.\r\nRESULTS: The diagnosis of pulmonary LAM was established after one year on chest HRCT and lung biopsy which revealed the proliferation of smooth muscle of pulmonary vessels, positive for actin, desmin, vimentin, estrogen- and progesterone- receptors. Spirometry revealed mixed obstructive and restrictive dysfunction. A correlation between worsening of dyspnea and estradiol peaks occurred during three gestation periods. Despite a short treatment with medroxyprogesterone 10 mg/day and tamoxifen (20 mg/day), the patient?s symptoms and pulmonary function tests worsened. aGnRH treatment improved both symptoms and pulmonary function tests during the first year and was associated with a slow decline in pulmonary function tests and stabilization of the cystic lesions during the following 3 years. The patient did not develop LAM-complications such as: pneumothorax, chylothorax, or hemoptysis.\r\nCONCLUSION: Treatment with aGnRH is effective in slowing the evolution of pulmonary LAM.

Keywords: estrogen receptors, gonadoliberin analogs, lymphangioleiomyomatosis

Correspondence: Mihai Coculescu, Endocrinology Department, ?Carol Davila? University of Medicine and Pharmacy, 34-36 Bd. Aviatorilor, 011863, Bucharest, Romania, Tel/Fax: + 4021 3198718, e-mail: m.coculescu@uni-davila.ro

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