Pulmonary lymphangioleiomyomatosis: Prolonged survival despite multiple pregnancies and no hormonal intervention
- 1 September 1996
- journal article
- case report
- Published by Wiley in Respirology
- Vol. 1 (3) , 213-215
- https://doi.org/10.1111/j.1440-1843.1996.tb00035.x
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.Keywords
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