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Pulmonary Langerhans Cell Histiocytosis (PLCH)

Imaging findings

Chest radiograph and CT show bilateral, symmetric reticular and nodular opacities with tiny cystic spaces primarily located in the upper and mid-lung zones, with characteristic sparing of the lung bases and costophrenic angles.

Key takeaways

Pulmonary Langerhans cell histiocytosis is a smoking-related interstitial lung disease that almost exclusively affects young to middle-aged smokers. The disease typically presents with an upper-lung-predominant distribution of nodules that eventually cavitate to form thin-walled, bizarrely shaped cysts, which can be distinguished from lymphangioleiomyomatosis by the sparing of the lung bases.

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