title: 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.affiliation: Department of Adult Haematology, Centre National de la Recherche Scientifique, Unité Mixte de Recherche 8147, Hôpital-Necker Enfants-Malades, Université de Paris-Descartes, AP-HP, Paris, France. doc.aubert@gmail.com
authors.forename: Olivier
authors.initials: O
authors.lastname: Aubert
authors.forename: Achille
authors.initials: A
authors.lastname: Aouba
authors.forename: Soizic
authors.initials: S
authors.lastname: Deshayes
authors.forename: Sophie
authors.initials: S
authors.lastname: Georgin-Lavialle
authors.forename: Philippe
authors.initials: P
authors.lastname: Rieu
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
mesh_terms: D000328:Adult; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D031249:Erdheim-Chester Disease / Q000000981:diagnostic imaging* / Q000188:drug therapy*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D064847:Multimodal Imaging; D049268:Positron-Emission Tomography; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein