Erdheim-Chester disease (pulmonary manifestations)

Pulmonary manifestations of Erdheim-Chester disease are uncommon.

The lungs are affected in ~25% (range 20-35%) of cases 5

HRCT chest

Described findings include 1

Look also extra-pulmonary features such as

  • peri-aortic infiltration: may extend to the ascending aorta, coronary arteries, and aortic branches
  • infiltration of the pulmonary arteries, superior vena cava, pericardium, and myocardium

Pulmonary involvement is generally an indicator or poor prognosis 4.

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Article information

rID: 29370
System: Chest
Section: Pathology
Synonyms or Alternate Spellings:
  • Pulmonary involvement in Erdheim Chester disease

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