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Erdheim-Chester disease: Pulmonary infiltration responding to cyclophosphamide and prednisolone

Lookup NU author(s): Professor Stephen BourkeORCiD, Emeritus Professor John Gibson


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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.

Publication metadata

Author(s): Bourke SC, Nicholson AG, Gibson GJ

Publication type: Article

Publication status: Published

Journal: Thorax

Year: 2003

Volume: 58

Issue: 11

Pages: 1004-1005

Print publication date: 01/11/2003

ISSN (print): 0040-6376

ISSN (electronic): 1468-3296

Publisher: BMJ Group


DOI: 10.1136/thorax.58.11.1004

PubMed id: 14586060


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