Pulmonary lymphangioleiomyomatosis: prolonged survival despite multiple pregnancies and no hormonal intervention.
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.
Yigla
M
M
Division of Pulmonary Diseases, Rambam Medical Center and Faculty of Medicine, Technion Institute of Technology, Haifa, Israel.
Bentur
L
L
Ben Izhak
O
O
Rubin
A H
AH
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
D004967:Estrogens