This 33 y.o. female presented with SOB, oxygen desaturation
with exercise and exertional breathless for 3 years.
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.
Hypertrophy of the smooth muscle of lymphatics, bronchioles,
venules and other tissues results in this disease. It is
seen in women of reproductive age. Patients often present
with pneuomothorax, dyspnea, hemoptysis and chylous pleural
effusions. Pulmonary function tests show increased TLC and
RV, decreased DLCO and V/Q mismatch.
Differential Diagnosis (DDx):
Hay, J.G. Turner-Warwick,M. "Pulmonary Hemosiderosis,
HemorrhagicSyndromes and Other Rare Infiltrative Disorders"
in Murray, J.F.,Nadel, J.A. eds. Textbook of Respiratory
Medicine. W.B. Saunders 1988. pp. 1508.
Case contributed by Valerie Hale, MD.
Debra Dyer, MD