The multisystem disease affecting the lungs, kidneys and lymph system called lymphangioleiomyomatosis (LAM) does not appear to respond to treatment with the gonadotropin-releasing hormone (GnRH) analogue, triptorelin.
The anti-estrogen triptorelin was selected as a possible treatment option because LAM cells generally have a high number of estrogen receptors, and the disease primarily affects women. But according to Dr. Angelo M. Taveira-DaSilva and colleagues at the National Heart, Lung, and Blood Institute of the National Institutes of Health in Bethesda, Maryland, triptorelin not only did not slow cystic lung destruction, it also induced a significant loss of bone mineral density.
Dr. Taveira-DaSilva's team treated 11 premenopausal women with LAM with triptorelin. Subjects were evaluated at baseline, and then every three to six months for three years. Monitoring was done with hormonal assays, high-resolution CT scans of the chest, bone mineral density studies, pulmonary function testing and six-minute walk tests.
"Gonadal suppression was achieved in all patients," Dr. Taveira-DaSilva and associates report in the February issue of
Chest. The researchers observed a "significant decline in lung function" in the patients. Two patients underwent lung transplantation within a year of study enrollment. One patient was lost to follow-up.
Treatment was associated with a decline in bone mineral density of approximately 23 per cent during the three-year follow-up period. Two patients showed evidence of lumbar spine osteopenia at the end of the study period.
"Please note that this was a small study done in only ten patients," Dr. Taveira-DaSilva cautioned in an interview with Reuters Health. "One has to be cautious to generalize to all patients with lymphangioleiomyomatosis."
"In the cases studied, triptorelin did not prevent progression of disease. In general, it is accepted that in most patients with [LAM], hormonal therapy is not helpful and prolonged therapy with this type of drug may accelerate bone loss," Dr. Taveira-DaSilva stated.
