CTICasesPulmonary Langerhans Cell Histiocytosis
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Imaging findings
Chest radiographs show increased lung volumes, increased intercostal spacing, and multiple subtle reticular opacities predominantly in the lower lung zones on the lateral view, with enlargement of the retrosternal clear space. High-resolution CT demonstrates multiple irregularly shaped, thick-walled cavitary lesions interspersed with centrilobular micronodules representing bronchiolar plugging and surrounding fibrosis. The lesions show a clear upper and mid-lung predominance with relative sparing of the lung bases and costophrenic angles.
Key takeaways
Pulmonary Langerhans Cell Histiocytosis (PLCH) is a smoking-related interstitial lung disease characterized by a combination of nodules, cavitating nodules, and thick- or thin-walled cysts that typically progress to bizarre, confluent shapes. The disease characteristically spares the lung bases, lingula, and the medial segment of the right middle lobe, although atypical distributions can sometimes occur. It typically starts as centrilobular bronchiolitis and progresses to peribronchiolar fibrosis.
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