Chest
Original ResearchInterstitial Lung DiseaseEffect of a Gonadotrophin-Releasing Hormone Analogue on Lung Function in Lymphangioleiomyomatosis
Section snippets
Study Population
This study evaluated the response to treatment with triptorelin, a synthetic analogue of Gn-RH (11.25 mg IM every 3 months) in 11 premenopausal LAM patients. Informed consent was obtained from all patients, and the protocol was approved by the San Giuseppe Hospital Institutional Review Board.
Study Design
Patients were evaluated at baseline, and at 3, 6, 12, 18, 24, and 36 months after initiating therapy. Routine blood testing and hormonal assays (ie, for FSH, LH, 17 β-estradiol, and total testosterone) were
Characteristics of the Patients
Of the 11 patients (mean age, 37 ± 7 years; age range, 24 to 49 years), 9 had a histologically proven diagnosis of LAM, and 2 patients exhibited clinical and radiographic features consistent with LAM. Radiographic features consisted of the presence of thin-walled cysts scattered throughout the lungs combined with abdominal angiomyolipomas and/or lymphangioleiomyomas.2 Three patients were ex-smokers; the remaining patients were nonsmokers. One patient had LAM/TSC. Four patients had renal
Discussion
The goal of this study was to evaluate the effect of treatment with triptorelin, a Gn-RH analogue, on lung function in patients with LAM. Although triptorelin was effective in suppressing ovarian function, all but 1 of the 10 patients who completed the study experienced a decline in FEV1; a reduction in Dlco was observed in all patients. In two cases, an accelerated loss of lung function resulted in lung transplantation. Only one patient showed improvement in FEV1 and 6MWT distance, despite
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Supported in part by the Intramural Research Program, National Institutes of Health, National Heart, Lung, and Blood Institute.
The authors have 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.