Favorable radiological outcome of skeletal Erdheim-Chester disease involvement with anakinra.

Abstract

Erdheim-Chester disease is a rare non-langerhans cell histiocytosis characterized by infiltration of foamy CD68-positive but CD1a-negative macrophages and fibro-inflammatory lesions as retroperitoneal, periureteral areas or bones. Interferon-α therapy has been used as treatment but it had variable efficiency and limited tolerance. More recently, a recombinant form of interleukin-1 receptor antagonist (anakinra) was used with success but no skeletal radiological improvement was recorded. We report here a case of interleukin-1 receptor antagonist in the treatment of refractory bones infiltration in Erdheim-Chester disease. After 1 year of treatment, the positron emission tomography-computed tomography showed an outstanding response of the skeletal involvement with clearly lower and smaller hypermetabolism images.

• Authors: Olivier Aubert, Achille Aouba, Soizic Deshayes, Sophie Georgin-Lavialle, Philippe Rieu, Olivier Hermine
• Journal: Joint bone spine (Joint Bone Spine) Vol. 80 Issue 2 Pg. 206-7 (Mar 2013) ISSN: 1778-7254 [Electronic] France
• PMID: 22999905 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2012 Société française de rhumatologie. Published by Elsevier SAS. All rights reserved.

Chemical References

• Antirheumatic Agents
• Interleukin 1 Receptor Antagonist Protein

Topics

• Adult
• Antirheumatic Agents (therapeutic use)
• Erdheim-Chester Disease (diagnostic imaging, drug therapy)
• Female
• Follow-Up Studies
• Humans
• Interleukin 1 Receptor Antagonist Protein (therapeutic use)
• Multimodal Imaging
• Positron-Emission Tomography
• Tomography, X-Ray Computed
• Treatment Outcome