Department of Paediatric Rheumatology, Bristol Royal Hospital for Children, Bristol, UK.
Rheumatology (Oxford). 2010 Apr;49(4):806-11. doi: 10.1093/rheumatology/kep453. Epub 2010 Jan 25.
To assess the use of mycophenolate mofetil (MMF) in the treatment of refractory primary angiitis of the CNS in childhood (cPACNS).
A retrospective chart review was performed in patients with cPACNS who were treated with MMF following failure of a combination of corticosteroids and another immunosuppressant.
Three patients from two centres were included in this study. The age of onset of disease was 5, 6 and 9 years. All the patients improved when treated with MMF, such that the dose of corticosteroids could be weaned or stopped.
MMF should be considered for maintenance treatment in the management of patients with cPACNS refractory to the combination of corticosteroids and first-line immunosuppressive agents.
评估霉酚酸酯（MMF）在儿童难治性中枢神经系统原发性血管炎（cPACNS）治疗中的应用。
对接受 MMF 治疗的 cPACNS 患者进行回顾性图表分析，这些患者在联合应用皮质类固醇和另一种免疫抑制剂治疗失败后接受 MMF 治疗。
本研究纳入了来自两个中心的 3 名患者。疾病发病年龄分别为 5 岁、6 岁和 9 岁。所有患者在接受 MMF 治疗后均有所改善，使得皮质类固醇的剂量可以逐渐减少或停止。
对于对皮质类固醇和一线免疫抑制剂联合治疗无效的 cPACNS 患者，应考虑使用 MMF 进行维持治疗。
