Which of the interstitial lung diseases will in one-third of patients cause a chylothorax?
a. Alveolar proteinosis
b. Idiopathic pulmonary fibrosis
c. Lymphangioleiomyomatosis
d. Nonspecific interstitial pneumonitis
ANS: C
Lymphangioleiomyomatosis (LAM) is a rare disorder of abnormal smooth muscle tissue prolifer-ating around small airways leading to severe obstruction and destruction of alveoli with resultant thin-walled cyst formation. Unilateral or less commonly bilateral chylothorax is seen in approxi-mately one-third of patients. This results from lymphatic obstruction by abnormal smooth muscle tissue.
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