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.
Joint Bone Spine. 2013 Mar;80(2):206-7. doi: 10.1016/j.jbspin.2012.07.005. Epub 2012 Sep 19.
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.
厄尔-当斯-切斯特病是一种罕见的非朗格汉斯细胞组织细胞增生症，其特征是泡沫状 CD68 阳性但 CD1a 阴性的巨噬细胞浸润和纤维炎症性病变，累及腹膜后、肾盂周围区域或骨骼。干扰素-α治疗已被用作治疗方法，但疗效不一，耐受性有限。最近，白细胞介素-1 受体拮抗剂（阿那白滞素）的重组形式被成功应用，但未记录到骨骼影像学改善。我们在此报告一例白细胞介素-1 受体拮抗剂治疗厄尔-当斯-切斯特病难治性骨浸润。治疗 1 年后，正电子发射断层扫描-计算机断层扫描显示骨骼受累有显著反应，代谢明显降低，且较小的高代谢图像。
