Iannetti Ludovico, Zito Roberta, Bruschi Simone, Papetti Laura, Ulgiati Fiorenza, Nicita Francesco, Del Balzo Francesca, Spalice Alberto
Department of Ophthalmology, Ocular Immunovirology Service, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy.
Clin Dev Immunol. 2012;2012:698327. doi: 10.1155/2012/698327. Epub 2012 Sep 12.
Central nervous system vasculitides in children may develop as a primary condition or secondary to an underlying systemic disease. Many vasculitides affect both adults and children, while some others occur almost exclusively in childhood. Patients usually present with systemic symptoms with single or multiorgan dysfunction. The involvement of central nervous system in childhood is not frequent and it occurs more often as a feature of subtypes like childhood polyarteritis nodosa, Kawasaki disease, Henoch Schönlein purpura, and Bechet disease. Primary angiitis of the central nervous system of childhood is a reversible cause of severe neurological impairment, including acute ischemic stroke, intractable seizures, and cognitive decline. The first line therapy of CNS vasculitides is mainly based on corticosteroids and immunosuppressor drugs. Other strategies include plasmapheresis, immunoglobulins, and biologic drugs. This paper discusses on current understanding of most frequent primary and secondary central nervous system vasculitides in children including a tailored-diagnostic approach and new evidence regarding treatment.
儿童中枢神经系统血管炎可能作为原发性疾病出现，也可能继发于潜在的全身性疾病。许多血管炎在成人和儿童中均可发生，而其他一些血管炎几乎仅在儿童期出现。患者通常表现为伴有单一或多器官功能障碍的全身症状。中枢神经系统在儿童期受累并不常见，更常作为儿童结节性多动脉炎、川崎病、过敏性紫癜和白塞病等亚型的特征出现。儿童原发性中枢神经系统血管炎是严重神经功能障碍的可逆病因，包括急性缺血性卒中、难治性癫痫和认知功能下降。中枢神经系统血管炎的一线治疗主要基于皮质类固醇和免疫抑制药物。其他治疗策略包括血浆置换、免疫球蛋白和生物药物。本文讨论了目前对儿童最常见的原发性和继发性中枢神经系统血管炎的认识，包括针对性的诊断方法和有关治疗的新证据。
