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.

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