What is central nervous system vasculitis?

What is central nervous system vasculitis?

Central nervous system (CNS) vasculitis is inflammation of blood vessel walls in the brain or spine. (The brain and the spine make up the central nervous system.) CNS vasculitis often occurs in the following situations:

  • Accompanied by other autoimmune diseases such as systemic lupus erythematosus, dermatomyositis and rarely, sarcoidosis and rheumatoid arthritis.
  • Infection, such as viral or bacterial.
  • Systemic (affecting the whole body) vasculitic disorders, which include granulomatosis with polyangiitis GPA), eosinophilic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis, cryoglobulinemic vasculitis and Behçet's syndrome).

It can also occur without any associated systemic disorder. In this case, the vasculitis is only confined to the brain or the spinal cord and it’s referred to as primary angiitis of the CNS (PACNS).

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