CTICasesNiemann-Pick Disease Type B
Imaging findings
Chest CT shows smooth interlobular septal thickening and ground-glass opacities, predominantly in the lower lung zones. Abdominal CT shows massive splenomegaly (larger than the liver) with splenic cysts.
Key takeaways
Niemann-Pick disease Type B is a lysosomal storage disorder that presents in adulthood with hepatosplenomegaly and lung involvement. The classic CT finding is smooth interlobular septal thickening, mimicking lymphangitic carcinomatosis.
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