CTICasesBurned-out Langerhans Cell Histiocytosis
Imaging findings
High-resolution CT shows a lacy pattern of upper-lung-predominant fibrosis combined with centrilobular emphysema and numerous cysts, with relative sparing of the lung bases. Notably, there is relative preservation of the pulmonary vascular tree, with healthy-appearing pulmonary vessels traversing the severely cystic and emphysematous lung regions.
Key takeaways
End-stage or burned-out Langerhans cell histiocytosis (LCH) can present with upper-lung-predominant cystic disease and lacy fibrosis. A helpful clue in distinguishing advanced cystic lung diseases from pure centrilobular emphysema is the relative preservation of the pulmonary vessels, which appear intact rather than pruned.
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