Pregnancy unmasking lymphangioleiomyomatosis

A 30 year-old housewife presented with cough and shortness of breath which progressed during her ensuing pregnancy, culminating in a still-birth at 9 months of gestation and requiring her hospitalisation in the immediate postpartum period. HRCT scan of the thorax showed thin-walled cysts and open lung biopsy confirmed the clinical impression of lymphangioleiomyomatosis. She was put on oral medroxyprogesterone acetate. After disease flare-up in the postpartum period her symptoms have stabilised.