title: Lymphangioleiomyomatosis.
abstract: Lymphangioleiomyomatosis is a rare multisystem disease predominantly affecting women that can occur sporadically or in association with tuberous sclerosis. Lung cysts progressively replace the lung parenchyma, which leads to dyspnea, recurrent pneumothorax, and in some cases respiratory failure. Patients may also have lymphatic disease in the thorax, abdomen, and pelvis, and renal angiomyolipomas. Treatment includes supportive care, bronchodilators, and for those with progressive disease, mammalian target of rapamycin (mTOR) inhibitors.
authors: Division of Respiratory Medicine, National Centre for Lymphangioleiomyomatosis, University Hospitals NHS Trust, University of Nottingham, Nottingham, NG7 2UH, UK. Electronic address: simon.johnson@nottingham.ac.uk.
authors: Simon R
authors: SR
authors: Johnson
authors: Cardiovascular and Pulmonary Branch, National Institutes of Health, National Heart Lung and Blood Institute, Building 10, Room 6D05, Bethesda, MD 20814, USA.
authors: Angelo M
authors: AM
authors: Taveira-DaSilva
authors: Cardiovascular and Pulmonary Branch, National Institutes of Health, National Heart Lung and Blood Institute, Building 10, Room 6D03, Bethesda, MD 20814, USA.
authors: Joel
authors: J
authors: Moss
mesh_terms: D018207:Angiomyolipoma / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology / Q000628:therapy; D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use; D001993:Bronchodilator Agents / Q000627:therapeutic use; D000068338:Everolimus / Q000627:therapeutic use; D006801:Humans; D007680:Kidney Neoplasms / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology / Q000628:therapy; D008175:Lung Neoplasms / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology / Q000628:therapy; D018192:Lymphangioleiomyomatosis / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology / Q000628:therapy; D018700:Pleurodesis; D011030:Pneumothorax / Q000209:etiology / Q000628:therapy; D020123:Sirolimus / Q000627:therapeutic use; D014057:Tomography, X-Ray Computed; D014402:Tuberous Sclerosis / Q000150:complications / Q000000981:diagnostic imaging* / Q000473:pathology; D014463:Ultrasonography
chemical_list: D000903:Antibiotics, Antineoplastic; D001993:Bronchodilator Agents; D000068338:Everolimus; D020123:Sirolimus
keywords: Angiomyolipoma; Cystic lung disease; Lymphatic disease; Pneumothorax; mTOR inhibitor