CTICasesPulmonary Langerhans Cell Histiocytosis
Imaging findings
Chest CT shows severe cystic lung disease with bizarrely shaped, thick-walled cysts and small stellate nodules predominating in the upper and middle lobes with relative sparing of the lung bases. Calcification of the pulmonary arteries is also seen.
Key takeaways
Long-standing or burned-out pulmonary Langerhans cell histiocytosis (PLCH) presents with upper-zone predominant bizarre cysts and stellate scars. Severe vascular remodeling and pulmonary artery atherosclerosis can occur over decades.
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