pulmonary disorders
Lymphangioleiomyomatosis
Treatment of Lymphangioleiomyomatosis
Sirolimus Lung transplantation Standard treatment of lymphangioleiomyomatosis is Lung and Heart-Lung Transplantation , but the disorder can recur in transplanted lungs. Sirolimus(an mTOR inhibitor) may help stabilize or slow the decline in pulmonary function among patients with moderate lung impairment (forced expiratory volume in 1 second [FEV1] < 70% predicted). Treatment withsirolimusis recommended for patients with abnormal or declining lung function. Alternative treatments, such as hormonal manipulation with progestins,tamoxifen, and oophorectomy, are largely ineffective and not recommended. Pneumothorax may be difficult to manage because it often recurs, is bilateral, and is less responsive to standard measures. Recurrent pneumothorax requires pleural abrasion,talcor chemical pleurodesis, or pleurectomy. Embolization to prevent bleeding should be considered for angiomyolipomas>4 cm. Air travel is well-tolerated by most patients. 