Lymphangioleiomyomatosis (LAM): CT Findings, Recurrent Spontaneous Pneumothorax, and the Role of Sirolimus
How to Recognize Diffuse Pulmonary Cysts, Establish the Diagnosis, and Understand the Clinical Significance of Recurrent Pneumothorax Executive Answer Lymphangioleiomyomatosis (LAM) is a rare, progressive cystic lung disease that predominantly affects women. Its characteristic imaging appearance consists of multiple, diffuse, thin-walled pulmonary cysts distributed throughout both lungs. Spontaneous pneumothorax may be the first major clinical manifestation and can recur because of progressive cystic destruction of the lung parenchyma. On chest CT, the combination of diffuse pulmonary cysts and pneumothorax should immediately raise suspicion for LAM. Definitive diagnosis can be established through characteristic histopathology and immunohistochemical findings. Sirolimus, an inhibitor of the mechanistic target of rapamycin (mTOR) pathway, is an important disease-modifying treatment for patients with abnormal or declining lung function. 1. Why This Case Matters A spontaneous pneumo...