Erdheim-Chester disease: pulmonary infiltration responding to cyclophosphamide and prednisolone.
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.
Bourke
S C
SC
Consultant Respiratory Physician, Northumbria Healthcare Trust and Freeman Hospital, Newcastle upon Tyne, UK. sbourke@doctors.org.uk
Nicholson
A G
AG
Gibson
G J
GJ
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000627:therapeutic use*; D031249:Erdheim-Chester Disease / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008171:Lung Diseases / Q000188:drug therapy* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use*; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D011239:Prednisolone