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Clinical and Developmental Immunology
Volume 2012 (2012), Article ID 698327, 9 pages
Review Article

Recent Understanding on Diagnosis and Management of Central Nervous System Vasculitis in Children

1Department of Ophthalmology, Ocular Immunovirology Service, Sapienza University of Rome, Viale del Policlinico 155, 00161 Rome, Italy
2Department of Pediatrics, Child Neurology Division, Sapienza University of Rome, 00185 Rome, Italy

Received 30 March 2012; Revised 28 May 2012; Accepted 13 August 2012

Academic Editor: Rolando Cimaz

Copyright © 2012 Ludovico Iannetti et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


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.