ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 3179

Diagnosing Childhood Small Vessel CNS Vasculitis

Marinka Twilt1, Maryam Nabavi Nouri2, Pascal N. Tyrrell3, Anastasia Dropol1, Shehla Sheikh4, Cynthia Hawkins5 and Susanne Benseler6,7, 1Rheumatology, Alberta Children's Hospital, Calgary, AB, Canada, 2Neurology, The Hospital for Sick Childern, Toronto, ON, Canada, 3Department of Medical Imaging, University of Toronto, Toronto, ON, Canada, 4Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, 5Paediatric Laboratory Medicine, The Hospital for Sick Children, Toronto, ON, Canada, 6Alberta Children's Hospital Research Institute/University of Calgary, Calgary, AB, Canada, 7Pediatrics/Alberta Children's Hospital, Department of Pediatrics/University of Calgary, Calgary, AB, Canada

Meeting: 2015 ACR/ARHP Annual Meeting

Date of first publication: September 29, 2015

Keywords: , ,

Session Information

Date: Tuesday, November 10, 2015

Title: Pediatric Rheumatology - Clinical and Therapeutic Aspects IV: Imaging and Novel Clinical Interventions

Session Type: ACR Concurrent Abstract Session

Session Time: 4:30PM-6:00PM

Background/Purpose:

Childhood primary small vessel CNS vasculitis (SVcPACNS) is an increasingly recognized inflammatory brain disease with high morbidity and mortality mandating an elective brain biopsy to confirm the diagnosis. The aim of the study was to systematically review biopsies of SVcPACNS patients and inflammatory and epilepsy controls and to determine characteristic features defining the diagnosis of SVcPACNS.

Methods:

A previously developed, standardized brain biopsy review instrument was applied to consecutive full thickness brain biopsies of pediatric cases and controls collected at a single center. Standardized stains including Hematoxyllin & Eosin, histochemistry of immune cell subsets plus electron microscopy. Nine North American expert neuropathologists were blinded reviewed to the patient’s presentation, diagnosis and therapy. All biopsies were de-identified and scored independently by two reviewers. Univariate analyses compared variable between groups; correspondence analysis determined the multi-dimensional relationship of histological variables and patient diagnoses. 

Results:

A total of 31 brain biopsy specimens of children with SVcPACNS, 12 with epilepsy and 11 with non-vasculitic inflammatory brain disease controls were included. Correspondence analyses revealed distinct clusters of the three diagnoses based on dimensions of location of infiltrate and subtype/ severity of inflammation.  Significant histological characteristics found to set apart SVcPACNS from controls included angiocentric (p<0.01) and/or perivascular infiltrates (p=0.04), evidence of endothelial cell activation (p<0.01) and inflammation in both grey and white matter (p<0.01). The infiltrate was found to be primarily T-cell mediated (CD3+ 86%, CD8+ 90%) only 27% of SVcPACNS biopsies had evidence of B cells. Features reported in adult PACNS including granulomas, necrosis or fibrin deposits were absent in all biopsies.  Leptomeningeal inflammation was non-diagnostic.

Conclusion:

Distinct histological features were identified on brain biopsies of SVcPACNS and may help defining the disease. These were absent in biopsies of children with epilepsy and non-vasculitic inflammatory brain diseases and allow for the development of diagnostic criteria.

Disclosure: M. Twilt, None; M. Nabavi Nouri, None; P. N. Tyrrell, None; A. Dropol, None; S. Sheikh, None; C. Hawkins, None; S. Benseler, None.

To cite this abstract in AMA style:

Twilt M, Nabavi Nouri M, Tyrrell PN, Dropol A, Sheikh S, Hawkins C, Benseler S. Diagnosing Childhood Small Vessel CNS Vasculitis [abstract]. Arthritis Rheumatol. 2015; 67 (suppl 10). https://acrabstracts.org/abstract/diagnosing-childhood-small-vessel-cns-vasculitis/. Accessed .

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