Division of Rheumatology and Immunology, University Children's Hospital, Technical University Dresden, Fetscherstrasse 74, Dresden, Germany.
J Clin Rheumatol. 2011 Jan;17(1):23-7. doi: 10.1097/RHU.0b013e318205092d.
We describe 2 patients with systemic juvenile idiopathic arthritis and macrophage activation syndrome. Treatment with recombinant interleukin 1 receptor antagonist (anakinra) and a corticosteroid rapidly induced remission, which could be maintained with anakinra monotherapy at a stable dose of 2 mg/kg per day. Pain at the injection site during the initial injections was the only adverse effect attributable to anakinra. Untoward effects of corticosteroid treatment were mild because prolonged therapy with high-dose corticosteroids could be avoided. These results suggest that early institution of interleukin 1 blockade merits further investigation for the treatment of macrophage activation syndrome and, perhaps, related conditions such as hemophagocytic lymphohistiocytosis.
我们描述了 2 例系统性幼年特发性关节炎伴巨噬细胞活化综合征患者。采用重组白细胞介素 1 受体拮抗剂（阿那白滞素）和皮质类固醇治疗后迅速缓解，每天以 2mg/kg 的稳定剂量用阿那白滞素单药治疗即可维持缓解。最初注射时注射部位疼痛是唯一与阿那白滞素相关的不良反应。皮质类固醇治疗的不良反应较轻，因为避免了长期使用大剂量皮质类固醇治疗。这些结果表明，白细胞介素 1 阻断的早期应用值得进一步研究，以治疗巨噬细胞活化综合征，以及可能相关的噬血细胞性淋巴组织细胞增生症等疾病。
