Choice (A) Is The Best Answer

Choice "C" is the best answer. Lymphangioleiomyomatosis (LAM) is a disease where abnormal smooth muscle proliferation leads to progressive cystic destruction of the lungs. There is progressive decline of respiratory function with a typically obstructive pattern due to destruction of bronchioles. It is also characterized by pneumothorax and chylothorax due to obstruction of lymphatics. Multiple cysts are also typically present throughout the lungs, though this is often only evident on CT. Angioleiomyomas is seen in abdominal organs, especially the kidney. LAM is commonly associated with tuberous sclerosis, but also occurs sporadically in reproductive-age women.

Chylothorax results from disruption of the thoracic duct, and can be traumatic or nontraumatic. The most common cause of nontraumatic chylothorax is malignancy, with less common causes including LAM, sarcoidosis, tuberculosis, histoplasmosis, Castleman disease, Down syndrome, and venous thrombosis. The obstructive findings on spirometry, history of prior pneumothorax, and lack of known prior malignancy make LAM the most likely etiology.
Damage to the pancreatic duct from chronic pancreatitis can lead to fistula formation to the abdomen or chest. This can manifest as ascites or pleural effusion. The amylase content of this fluid is very high (typically > 1000 u/L). There is no associated intrinsic lung disease associated with chronic pancreatitis.
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive interstitial lung disease characterized by a restrictive spirometry pattern and radiographic features of fibrosis. It is not associated with pleural effusions or