# Primary central nervous system vasculitis: an update

## TL;DR

This paper updates the understanding of primary CNS vasculitis, a rare and serious condition affecting the brain's blood vessels.

## Contribution

The paper highlights recent advances in diagnosing and treating primary CNS vasculitis and suggests areas for future research.

## Key findings

- Biopsy and histopathological confirmation are crucial for diagnosing PCNSV.
- PCNSV is potentially treatable, though clinical trials are still needed.
- Collaborative studies could help advance treatment for this challenging disease.

## Abstract

Primary CNS vasculitis (PCNSV) is uncommon, difficult to diagnose, and potentially fatal or at least likely to cause major and persistent neurological deficits, and yet appears highly treatable (although controlled clinical trials remain to be done). Here, we summarize recent advances in the recognition and diagnosis of PCNSV, the crucial role of biopsy and histopathological confirmation of the diagnosis, and currently recommended approaches to treatment. We also sign post areas of potentially fruitful future collaborative study that might advance this difficult disease.