Erdheim-Chester disease: pulmonary infiltration responding to cyclophosphamide and prednisolone.

Abstract

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.

• Authors: S C Bourke, A G Nicholson, G J Gibson
• Journal: Thorax (Thorax) Vol. 58 Issue 11 Pg. 1004-5 (Nov 2003) ISSN: 0040-6376 [Print] England
• PMID: 14586060 (Publication Type: Case Reports, Journal Article)

Chemical References

• Anti-Inflammatory Agents
• Immunosuppressive Agents
• Cyclophosphamide
• Prednisolone

Topics

• Anti-Inflammatory Agents (therapeutic use)
• Cyclophosphamide (therapeutic use)
• Erdheim-Chester Disease (drug therapy, pathology)
• Humans
• Immunosuppressive Agents (therapeutic use)
• Lung Diseases (drug therapy, pathology)
• Male
• Middle Aged
• Prednisolone (therapeutic use)
• Treatment Outcome