Cerebral vasculitis as a clinical manifestation of neurosarcoidosis: A scoping review

Autoimmun Rev. 2024 Apr;23(4):103528. doi: 10.1016/j.autrev.2024.103528. Epub 2024 Mar 16.

Abstract

The occurrence of cerebral vasculitis in individuals with neurosarcoidosis (NS) is considered to be rare. Although the number of relevant publications has increased in recent years, evidence is mostly limited to case reports. To obtain a better understanding of this rare and severe manifestation of disease, we carried out a scoping review on cerebral vasculitis in patients diagnosed with NS. The results of the review indicate that the diagnosis of cerebral vasculitis in patients with NS is made especially in patients with systemic sarcoidosis. However, recurrent strokes in patients with NS remains the main indicator of cerebral vasculitis. A tissue biopsy is considered the gold standard to confirm the diagnosis despite occasional false-negative results. Glucocorticoids and steroid-sparing agents are the most successful current treatments. Favorable outcomes were observed with strategies targeting TNFα and B cells. The goal of this review is to summarize the current literature and treatment options for cerebral vasculitis in patients with NS.

Keywords: Neurosarcoidosis; Stroke; Vasculitis.

Publication types

  • Review

MeSH terms

  • Central Nervous System Diseases* / diagnosis
  • Central Nervous System Diseases* / etiology
  • Glucocorticoids / therapeutic use
  • Humans
  • Sarcoidosis* / complications
  • Sarcoidosis* / diagnosis
  • Vasculitis, Central Nervous System* / diagnosis
  • Vasculitis, Central Nervous System* / drug therapy
  • Vasculitis, Central Nervous System* / etiology

Substances

  • Glucocorticoids

Supplementary concepts

  • Neurosarcoidosis