Arthritis & Rheumatism, Volume 62,
November 2010 Abstract Supplement

Abstracts of the American College of
Rheumatology/Association of Rheumatology Health Professionals
Annual Scientific Meeting
Atlanta, Georgia November 6-11, 2010.

Childhood Primary Angiitis of the CNS: Identifying Disease Trajectories and Early Risk Factors for Persistently Higher Disease Activity.

Cellucci,  Tania, N. Tyrrell,  Pascal, Laughlin,  Suzanne, Armstrong,  Derek, Halliday,  William, Sheikh,  Shehla, M. Benseler,  Susanne


Childhood Primary Angiitis of the CNS (cPACNS) is a devastating inflammatory brain disease. cPACNS comprises of distinct clinical subtypes at presentation. Disease courses of cPACNS have never been systematically studied. The aim of the study was to characterize and compare distinct disease activity trajectories in cPACNS and to identify early risk factors for persistently higher disease activity over time.


A single centre cohort study was performed including consecutive children diagnosed with cPACNS based on Calabrese criteria between December 1998 and June 2010. Patients had to be <18 years of age at diagnosis and have serial measures of disease activity as defined by physician global assessment (10 cm Visual Analogue Scale). Standardized clinical assessments, Pediatric Stroke Outcome Measures, inflammatory markers, and neuroimaging characteristics were collected serially. Longitudinal data were analyzed using mixed effects models accounting for repeated measures and linear regression of slope parameter estimates over time.


The study cohort consisted of 45 children: 21 males, 24 females, median age at diagnosis 9.8 years, and median follow-up 21 months. Diagnoses: 26 (57%) had angiography-negative cPACNS, and 19 (43%) had angiography-positive cPACNS. At diagnosis, children with angiography-negative cPACNS were more likely to be female (81% vs. 16%, p<0.001), present with seizures (85% vs. 10%, p<0.001), and have elevated levels of ESR (p=0.036) and CRP (p=0.046). Disease activity decreased significantly over time in all cPACNS patients (p<0.001). Distinct trajectories of disease activity over time were identified for each cPACNS subtype. Children with angiography-negative cPACNS had persistently higher disease activity and required a longer time to remission (p=0.046). Female sex and elevated inflammatory markers at diagnosis were not predictive of delayed remission. Seizures at diagnosis of cPACNS were found to be an early risk factor for persistently higher disease activity over time (p=0.016).


Distinct subtypes of cPACNS were found to have unique disease activity trajectories. In all children, disease activity improved significantly over time. However, children diagnosed with angiography-negative cPACNS and those presenting with seizures were at the highest risk for persistently higher disease activity. Early recognition of this high-risk cohort may enable physician to initiate targeted therapies and prevent long-term brain injury in children with cPACNS.

To cite this abstract, please use the following information:
Cellucci, Tania, N. Tyrrell, Pascal, Laughlin, Suzanne, Armstrong, Derek, Halliday, William, Sheikh, Shehla, et al; Childhood Primary Angiitis of the CNS: Identifying Disease Trajectories and Early Risk Factors for Persistently Higher Disease Activity. [abstract]. Arthritis Rheum 2010;62 Suppl 10 :2107
DOI: 10.1002/art.29872

Abstract Supplement

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