[Treatment of pulmonary lymphangioleiomyomatosis using a GnRH agonist]

Pulmonary lymphangioleiomyomatosis is a rare and exclusively female disease which is oestrogen dependent and has a serious prognosis. We present two cases of young women aged 30 and 33 with the same clinical history of renal angiomyolipomas and recurring pneumothoraces. The computerised tomographic scans of the lung were strongly suggestive of LAM (there were fine wall cysts which were disseminated throughout both lung fields). The histological proof was provided by lung biopsy which was carried out during a thoracotomy for pleurectomies. The first patient who is currently asymptomatic and in excellent general health, was treated for three months with tamoxifen (Nolvadex), then for 20 months with 3.75 mg per month of triptoline (Decapeptyl), an agonist of GnRH. The second patient received triptoline in the same dose. After 40 months of treatment this patient was asymptomatic with satisfactory lung function tests. Agonists of GnRH (gonadotropic release hormone) seem to provide a useful alternative in the treatment of LAM.