Lymphangiomyomatosis: hormonal implications in etiology and therapy

When a 34-year-old woman had dyspnea and chylothorax 8 months postpartum, lymphangiomyomatosis was diagnosed by open-lung biopsy. Baseline laboratory studies to evaluate infertility had revealed normal hormonal levels. She was subsequently treated with various hormones to aid in conception and in sustaining pregnancy. Her condition has improved with progesterone therapy. This case raises concern about the possible adverse consequences of hormonal manipulation in treating infertility. The need for open-lung biopsy in patients with classic manifestations of LAM is also questioned, especially with the advent of high-resolution CT scanning, the nonspecificity of hormonal receptors with regard to response to therapy, and the possible need for subsequent lung transplantation.