Erdheim Chester Disease

Key Facts
  • Rare non-langerhans cell histiocytosis
  • Pleural and perirenal soft tissue thickening
  • Diffuse septal thickening
Imaging Findings

Chest radiograph

  • Cardiomegaly, Kerley B lines, blunting costophrenic angles (mimic CHF)
  • Sclerotic bone lesions


  • Ground glass opacites and centrilobular nodules
  • Smooth septal thickening
  • Diffuse pleural thickening
  • Encasement great vessels or kidneys
Differential Diagnosis
  • CHF
  • Lymphangitic tumor
  • Diffuse pulmonary lymphangiomatosis
  • Pulmonary venoocclusive disease
  • Pulmonary alveolar proteinosis
  • Amyloidosis
Pathological Features
  • Proliferation of non-langerhans cell histiocytes
Clinical Presentation
  • Middle age adults
  • Pituitary involvment presents with diabetes insipidus and exopthlmos
  • Slow progression of respiratory failure
  • No known treatment

Wittenberg, KH et al. Pulmonary involvement with Erdheim-Chester Disease: Radiographic and CT Findings. AJR 174:1327-31, 2000