This 33 y.o. The CXRs demonstrate pulmonary hyperexpansion and fine linear, cystic or nodular opacities. HRCT demonstrates diffuse replacement of lung parenchyma with cysts of somewhate uniform size. Some patients also have angiomyolipomas of the kidneys so it is important to image the kidneys in these patients. It is seen in women of reproductive age.
Patients often present with pneuomothorax, dyspnea, hemoptysis and chylous pleural effusions.
Textbook of Respiratory Medicine. W.B. Saunders 1988.