CTICasesPulmonary Langerhans Cell Histiocytosis
Imaging findings
Chest CT in a 45-year-old female heavy smoker with severe pulmonary hypertension shows advanced cystic lung disease. Findings include bizarre, irregular, thick-walled cysts and nodular wall thickening predominantly involving the upper and middle lung zones, with sparing of the lung bases. There is marked cicatricial emphysema, vascular pruning, and right ventricular hypertrophy.
Key takeaways
Pulmonary Langerhans cell histiocytosis (PLCH) is a smoking-related interstitial lung disease. It typically presents with a combination of nodules and bizarrely shaped cysts in the upper and mid lung zones, sparing the lung bases. Over time, cysts can collapse and lead to extensive cicatricial emphysema, and severe pulmonary hypertension (Group 5) is a known complication that can necessitate lung transplantation.
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