Introduction Primary Central Nervous System Vasculitis (PCNSV) can be diagnosed using cerebral angiography and/or biopsy as gold-standard tests. Diagnostic criteria weight each equally but are unvalidated and limited sensitivity and specificity for both tests is recognised. A systematic literature review was undertaken to investigate the performance of diagnostic tests in cases of PCNSV.

Methods We searched the OVID Medline database for original cases of PCNSV. We recorded information on diagnostic tests used and assessed agreement between angiography and biopsy as well as ancillary tests.

Results 701 original cases of PCNSV were found. Only 248 cases (34%) had both angiography and brain biopsy performed in the same patient. Both tests showed classical vasculitis features in just 32 individual cases (5%). 74 cases with abnormal angiography had normal brain biopsy, while 99 patients with abnormal biopsies had normal cerebral angiography. Brain MRI and CSF were usually abnormal.

Conclusion Cerebral angiography and biopsy were performed together in only a minority of published cases of PCNSV. When both were performed, conflicting results from each gold-standard test were much more likely than agreement. The diagnostic criteria for PCNSV require revision in order to classify it correctly and to exclude mimics.