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Pulmonary lymphangioleiomyomatosis: prolonged survival despite multiple pregnancies and no hormonal intervention
M Yigla1, L Bentur, O Ben Izhak
1Division of Pulmonary Diseases, Rambam Medical Center and Faculty of Medicine, Technion Institute of Technology, Haifa, Israel.
Respirology (Carlton, Vic.)
|September 1, 1996
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.