Response to treatment with progesterone in a patient with pulmonary lymphangioleiomyomatosis

Lymphangioleiomyomatosis (LAM) is defined as an abnormal proliferation of smooth muscles around lymphatics, venules, and brochioles. This article describes our experiences treating a 21-year-old, white female who experienced recurrent shortness of breath during air travel last year. Her episode was severe and the patient was transported to the hospital as soon as the airplane landed. Physical exam in the emergency room was significant for absent breath sounds on the right side and the chest X-ray revealed a pneumothorax. She required two chest tubes for complete lung re-expansion. Further evaluation showed an obstructive pattern and air trapping on pulmonary function tests. This patient was treated with Medroxyprogesterone acetate (MPA) for six months and subsequent pulmonary function tests revealed improvement in her condition.