Adult and childhood vasculitis

Handb Clin Neurol. 2023:195:653-705. doi: 10.1016/B978-0-323-98818-6.00008-X.


Vasculitis refers to heterogeneous clinicopathologic disorders that share the histopathology of inflammation of blood vessels. Unrecognized and therefore untreated, vasculitis of the nervous system leads to pervasive injury and disability, making this a disorder of paramount importance to all clinicians. There has been remarkable progress in the pathogenesis, diagnosis, and treatment of primary CNS and PNS vasculitides, predicated on achievement in primary systemic forms. Primary neurological vasculitides can be diagnosed with assurance after intensive evaluation that incudes tissue confirmation whenever possible. Clinicians must choose from among the available immune modulating, suppressive, and targeted immunotherapies to induce and maintain remission status and prevent relapse, unfortunately without the benefit of RCTs, and tempered by the recognition of anticipated medication side effects. It may be said that efforts to define a disease are attempts to understand the very concept of the disease. This has been especially evident in systemic and neurological disorders associated with vasculitis. For the past 100 years, since the first description of granulomatous angiitis of the brain, the CNS vasculitides have captured the attention of generations of clinical investigators around the globe to reach a better understanding of vasculitides involving the central and peripheral nervous system. Since that time it has become increasingly evident that this will necessitate an international collaborative effort.

Keywords: Adult; Children; Motor disorders; Vasculitis.

Publication types

  • Review

MeSH terms

  • Adult
  • Child
  • Humans
  • Nervous System Diseases* / complications
  • Peripheral Nervous System / pathology
  • Vasculitis* / complications
  • Vasculitis* / diagnosis
  • Vasculitis* / therapy