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Pulmonary Langerhans Cell Histiocytosis | Radiology Board Review Case

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CT appearance of Pulmonary Langerhans Cell Histiocytosis (LCH) and approach to imaging of cystic lung diseases

Pulmonary LCH is a rare disease found almost exclusively in cigarette smokers.

Radiologic findings of pulmonary LCH include

  • Nodules, usually smaller than 1 to 5 mm, centrilobular, and peribronchiolar, may be cavitary, may be seen in association with cysts.
  • Thick-walled or thin-walled cysts. – Progression over time from nodules to thick-walled cysts to thin-walled cysts.
  • Upper lobe predominance in size and number of nodules or cysts, costophrenic angles spared.
  • It can be associated with lymphadenopathy.

Pulmonary LCH vs lymphangioleiomyomatosis radiology

Imaging features of Pulmonary Langerhans Cell HistiocytosisImaging features of Lymphangioleiomyomatosis
– Thick-walled, or thin-walled cysts
– Nodules, usually smaller than 1 to 5 mm, centrilobular and peribronchiolar, may be cavitary, may be seen in association with cysts
– Progression over time from nodules to thick-walled cysts to thin-walled cysts
– Upper lobe predominance in size and number of nodules or cysts, costophrenic angles spared
– Fine reticular opacities
– Ground-glass opacity
– Mosaic perfusion or air trapping
– Pulmonary hypertension
– Thin-walled lung cysts, usually round in shape
– Diffuse distribution, costophrenic angles involved
– Mild septal thickening or ground-glass opacity
– Lymph node enlargement
– Small nodules in patients with TSC
– Pleural effusion
– Pneumothorax
Differentiating features of Pulmonary LCH and lymphangioleiomyomatosis

Common causes of cystic lung disease

Common (not truly cystic lung diseases but often confused as cysts)

  • Emphysema (e.g., bullae) – Thin imperceptible walls.
  • Cystic bronchiectasis
  • Honeycombing

Less Common

  • Pulmonary Langerhans Cell Histiocytosis
  • Lymphangioleiomyomatosis (isolated LAM or associated with the TSC)
  • Lymphocytic interstitial pneumonia (LIP) – Associated with Sjogren’s, AIDS, SLE
  • Cystic metastases
  • Pneumatoceles –
  • Birt-Hogg-Dube syndrome = Lung cysts and renal oncocytomas.

Approach to imaging of cystic lung diseases

Imaging approach to cystic lung diseases
Imaging approach to cystic lung diseases

Reference: A Stepwise Diagnostic Approach to Cystic Lung Diseases for Radiologists

Watch the video for the detailed case discussion and PACS based image stack.

Reference and further reading:

HRCT by Richard Webb

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