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Pulmonary lymphangioleiomyomatosis: Prolonged survival despite multiple pregnancies and no hormonal intervention
Author(s) -
YIGLA Mordechai,
BENTUR Lea,
IZHAK Offer BEN,
RUBIN Ami Hai E
Publication year - 1996
Publication title -
respirology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.857
H-Index - 85
eISSN - 1440-1843
pISSN - 1323-7799
DOI - 10.1111/j.1440-1843.1996.tb00035.x
Subject(s) - medicine , lymphangioleiomyomatosis , hormone , respiratory system , disease , interstitial lung disease , lung , rare disease , intervention (counseling) , physiology , psychiatry
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.