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Erdheim-Chester disease: pulmonary infiltration responding to cyclophosphamide and prednisolone
  1. S C Bourke1,
  2. A G Nicholson2,
  3. G J Gibson3
  1. 1Consultant Respiratory Physician, Northumbria Healthcare Trust and Freeman Hospital, Newcastle upon Tyne, UK
  2. 2Department of Histopathology, Royal Brompton Hospital, London, UK
  3. 3Professor of Respiratory Medicine, University of Newcastle and Freeman Hospital, Newcastle upon Tyne NE7 7DN, UK
  1. Correspondence to:
    Dr S C Bourke, North Tyneside General Hospital, Rake Lane, North Shields NE29 8NH, UK;


Erdheim-Chester disease is a rare multisystem histiocytic disorder in which death most commonly results from pulmonary or cardiac involvement. We report the case history of a 55 year old man with this condition with extensive and progressive pulmonary disease. He had no response to prednisolone alone, but treatment with prednisolone plus cyclophosphamide was associated with a rapid improvement in symptoms, lung function, and the chest radiographic appearance. He subsequently showed a symptomatic, functional, and radiological deterioration, followed by a marked slowing in the rate of decline. He currently remains stable 41 months after treatment was initiated. This is the first report of pulmonary Erdheim-Chester disease showing improvement in both lung function and symptoms with any form of treatment.

  • Erdheim-Chester disease
  • interstitial lung disease
  • cyclophosphamide

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