title: Pulmonary lymphangioleiomyomatosis: prolonged survival despite multiple pregnancies and no hormonal intervention.
abstract: Pulmonary lymphangioleiomyomatosis (PLAM) is rare progressive, fatal interstitial lung disease, considered to be sex hormones related. We report a follow-up, after 19 years, in a patient with PLAM without intervention. Despite excessive oestrogen production during four pregnancies there was only mild deterioration of her respiratory disease. Patients with good functional reserve should be followed-up closely before deciding on hormonal ablative treatment.
authors.affiliation: Division of Pulmonary Diseases, Rambam Medical Center and Faculty of Medicine, Technion Institute of Technology, Haifa, Israel.
authors.forename: M
authors.initials: M
authors.lastname: Yigla
authors.forename: L
authors.initials: L
authors.lastname: Bentur
authors.forename: O
authors.initials: O
authors.lastname: Ben Izhak
authors.forename: A H
authors.initials: AH
authors.lastname: Rubin
mesh_terms: D018572:Disease-Free Survival; D004967:Estrogens / Q000502:physiology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008175:Lung Neoplasms / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D018192:Lymphangioleiomyomatosis / Q000175:diagnosis / Q000503:physiopathology* / Q000628:therapy; D008875:Middle Aged; D011247:Pregnancy / Q000502:physiology*; D012129:Respiratory Function Tests; D014057:Tomography, X-Ray Computed
chemical_list: D004967:Estrogens