Diagnostic test results in primary CNS vasculitis: a systematic review of published cases

McVerry, F., McCluskey, G., McCarron, P., Muir, K. W. and McCarron, M. O. (2017) Diagnostic test results in primary CNS vasculitis: a systematic review of published cases. Neurology: Clinical Practice, 7(3), pp. 256-265. (doi: 10.1212/CPJ.0000000000000359)

Abstract

Background: Primary central nervous system vasculitis (PCNSV) can be diagnosed using cerebral angiography and/or histopathology combined with clinical features. The original diagnostic criteria, which weight each test equally, have not been validated. Limited sensitivity and specificity for biopsy and angiography are recognized. We systematically reviewed results of diagnostic tests performed in patients with an ultimate diagnosis of PCNSV. Methods: We searched the OVID Medline database and bibliographies for original cases of PCNSV. We recorded demographics, diagnostic tests used, and assessed agreement between angiography and biopsy when both tests were performed. We also recorded MRI and cerebrospinal fluid (CSF) results. Results: We found 701 original cases with PCNSV diagnosed with angiography and / or pathology. 269 patients (38.4%) had both cerebral angiography and histopathological testing (biopsy/ post mortem). Classic angiographic features of vasculitis were associated with pathological confirmation in just 32 patients (4.6%). 74 patients (10.6%) with any abnormality on angiography had a normal biopsy, and 99 patients (14.1%) with abnormal biopsies had normal angiography. Brain MRI was abnormal in 505/541 patients (93.3%) and CSF was abnormal in 360/484 patients (74.4%). Increasing use of angiography and decreasing histopathological testing were found over time. Conclusion: Cerebral angiography and pathological tissue examination were undertaken in a minority of published cases with a diagnosis of PCNSV. When both diagnostic tests were performed, disagreement between them was more than five times more likely than agreement. Diagnostic criteria for PCNSV may require revision to classify the clinical, pathological and radiological features of this condition more accurately.

• Item Type: Articles
• Status: Published
• Refereed: Yes
• Glasgow Author(s) Enlighten ID: Muir, Professor Keith and McVerry, Dr Ferghal
• Authors: McVerry, F., McCluskey, G., McCarron, P., Muir, K. W., and McCarron, M. O.
• College/School: College of Medical Veterinary and Life Sciences > School of Psychology & Neuroscience
• Journal Name: Neurology: Clinical Practice
• Publisher: American Academy of Neurology
• ISSN: 2163-0402
• ISSN (Online): 2163-0933
• Published Online: 28 April 2017
• Copyright Holders: Copyright © 2017 American Academy of Neurology
• First Published: First published in Neurology: Clinical Practice 7(3): 256-265
• Publisher Policy: Reproduced in accordance with the copyright policy of the publisher