Arthritis & Rheumatism, Volume 63,
November 2011 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Chicago, Illinois November 4-9, 2011.

Vessel Remodeling in Childhood Primary CNS Vasculitis: Impact of Corticosteroid Therapy.

Soon,  Gordon S., Yau,  Ivanna, Armstrong,  Derek, Tyrrell,  Pascal N., Laughlin,  Suzanne, deVeber,  Gabrielle, Benseler,  Susanne M.


Childhood non-progressive primary CNS vasculitis (NPcPACNS) accounts for the single most common cause of vascular strokes in children. The aims of the study were 1) to describe the cerebral vessel imaging at presentation in children with NPcPACNS and 2) to determine whether corticosteroid therapy had any impact on follow-up imaging.


A single-centre cohort study of consecutive children diagnosed with cPACNS based on Calabrese criteria between January 1990 and December 2009 was performed. Children were included if they 1) had non-progressive cPACNS (unilateral proximal stenoses and no evidence of progression >3 months) and 2) had serial cerebral vessel imaging, including either Magnetic Resonance Angiography (MRA) or conventional angiography. The study excluded progressive cPACNS and angiography negative cPACNS. Data collection: Serial MRA and conventional angiography were blindly reviewed by two independent neuroradiologists following a previous developed protocol. Parenchymal lesions were characterized by number, size, and location (laterality, arterial territory, grey/white matter involvement). Cerebral vessel involvement was characterized by location and features of stenoses, including artery name, number affected, severity, laterality, and appearance (beading, dilatation, smooth, irregular). Analysis: frequencies were compared using descriptive statistics.


A total of 44 children with NP-cPACNS were included. Initial cerebral vessel imaging included conventional angiography in 39 children (89%). Proximal middle cerebral artery (MCA) was involved in 35 children (80%), distal internal carotid artery (ICA) in 25 (57%), and proximal anterior cerebral artery (ACA) in 17 (39%). Left-sided vascular disease was detected in 28 children (64%). Sixteen children (36%) had a maximal stenosis of >75%, including 13 (30%) with complete occlusion. Children treated with corticosteroid therapy had a significantly higher degree of stenosis on initial imaging (p=0.02). At 3 month follow-up imaging, there was no statistical difference in the number of affected vessels, degree of stenosis, or maximum length of stenosis compared between children treated with and without corticosteroid therapy.


NPcPACNS likely represents an inflammatory attack on the arterial wall thus providing the rationale for considering immunosuppressive therapy on presentation. At our institution, corticosteroid therapy was not uniformly provided in children with NPcPACNS but was typically reserved for children with the most severe arteriographic abnormalities. Nonetheless, cerebral vessel imaging at 3 month follow-up was similar in children treated with and without corticosteroid therapy, thereby suggesting a possible role for immunosuppressive treatment of this disease.

To cite this abstract, please use the following information:
Soon, Gordon S., Yau, Ivanna, Armstrong, Derek, Tyrrell, Pascal N., Laughlin, Suzanne, deVeber, Gabrielle, et al; Vessel Remodeling in Childhood Primary CNS Vasculitis: Impact of Corticosteroid Therapy. [abstract]. Arthritis Rheum 2011;63 Suppl 10 :1534

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