TY - JOUR T1 - Refractory Primary Central Nervous System Vasculitis of Childhood: Successful Treatment with Infliximab JF - The Journal of Rheumatology JO - J Rheumatol SP - 2227 LP - 2229 DO - 10.3899/jrheum.120616 VL - 39 IS - 11 AU - MICHELLE BATTHISH AU - BRENDA BANWELL AU - SUZANNE LAUGHLIN AU - WILLIAM HALLIDAY AU - CHRISTINE PESCHKEN AU - EMILE PARAS AU - SUSANNE BENSELER Y1 - 2012/11/01 UR - http://www.jrheum.org/content/39/11/2227.abstract N2 - To the Editor:Childhood primary angiitis of the central nervous system (cPACNS) is an increasingly recognized inflammatory brain disease causing devastating brain injury in previously healthy children1. Early recognition and initiation of treatment may reverse the severe deficits caused by inflammation and lead to complete neurological recovery. cPACNS has a broad clinical spectrum including acute ischemic stroke, intractable seizures, and severe cognitive decline2. cPACNS is classified into angiography-positive vasculitis and angiography-negative small-vessel vasculitis (SV-cPACNS)2.We reported the efficacy and safety of a treatment protocol for SV-cPACNS3. This immunosuppressive regimen led to complete neurological recovery in the majority of children3. There is limited knowledge of the approach to children who fail standard therapy. Tumor necrosis factor (TNF) inhibition has been considered in the treatment of refractory systemic vasculitis4. In addition, histological studies of SV-cPACNS brain biopsies demonstrated primary lymphocytic vessel wall infiltrates5.We describe 2 children with refractory SV-cPACNS who failed standard treatment with cyclophosphamide and high-dose corticosteroids. Treatment with anti-TNF therapy with infliximab (IFX) controlled disease activity and resolved neurologic symptoms.In Case 1, a 7-year-old previously healthy girl presented with fever and headaches. She was admitted to hospital and developed a generalized tonic-clonic seizure. … Address correspondence to Dr. S. Benseler, Division of Rheumatology, Department of Pediatrics, The Hospital for Sick Children, 555 University Ave., Toronto, Ontario M5G 1X8, Canada. E-mail: susanne.benseler{at}sickkids.ca ER -