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Lymphangioleiomyomatosis (LAM) is a rare progressive multisystemic cystic lung disease. It commonly presents as fatigue, progressive dyspnea, and spontaneous pneumothorax which can progress to chronic respiratory failure. Previously, patients were diagnosed with LAM through histopathological testing. In 2017, new guidelines were released by the American Thoracic Society allowing the clinical diagnosis of LAM. This is the case of a 46-year-old female with a history of recurrent pneumothorax and progressive dyspnea. During episodes of pneumothorax, she underwent multiple chest tube insertions. High-resolution chest CT scan revealed a small right pneumothorax with septations, pleurodiaphragmatic adhesions, minimal pleural effusion, diffuse cystic lung disease, and a fat-containing right renal nodule consistent with an angiomyolipoma thereby fulfilling the clinical criteria for the diagnosis of LAM. The patient eventually underwent talc pleurodesis and was started on sirolimus. LAM should be considered in women of childbearing age without co-morbidities presenting with spontaneous pneumothorax.
Antonio et al. (Fri,) studied this question.