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Secondary CNS Vasculitis

Children ≤18 years of age are diagnosed with secondary CNS vasculitis if they have evidence of 

  • Any newly acquired focal and/or diffuse neurological and/or psychiatric deficit plus 
  • Angiography and/or brain biopsy evidence of CNS vasculitis
  • In the presence of an underlying systemic condition such as an infection, rheumatic disease or other

Secondary CNS vasculitis can be caused by  a large number of underlying conditions. Infection is the most common cause for secondary CNS vasculitis.  A thorough evaluation for an underlying systemic condition is required when a child presents with newly acquired neurological and/or psychiatric deficit. 

Causes of Secondary CNS Vasculitis

The following is a list of causes of secondary CNS vasculitis in children:



  • Bacterial
    • Mycobacterium tuberculosis
    • Streptococcus pneumoniae
    • Salmonella species
    • Mycoplasma pneumoniae
    • Treponema pallidum
    • Other
  • Spiorochete
    • Borrelia burgdorferi
  • Viral
    • Varicella zoster virus
    • HIV
    • Hepatitis C virus
    • Cytomegalovirus
    • Epstein-Barr virus
    • Parvovirus B19
    • Enterovirus
    • West Nile virus
    • Other
  • Fungal
    • Actinomycosis
    • Candida albicans
    • Aspergillus
    • Other

Systemic rheumatic diseases

  • Systemic lupus erythematosus
  • Systemic vasculitis
    • Anti-neutrophil cytoplasmatic antibodies (ANCA) associated vasculitis
      • Granulomatosis with Polyangiitis (GPA, formerly known as Wegener's granulomatosis)
      • Microscopic polyangiitis (MPA)
      • Eosinophilic Granulomatosis with Polyangiitis (EGPA, formerly known as Churg–Strauss syndrome)
    • Polyarteritis nodosa (PAN)
    • Kawasaki disease
    • Takayasu's arteritis
    • Henoch-Schoenlein purpura (HSP)
  • Behçet's syndrome 
  • Sjögren's syndrome
  • Juvenile Dermatomyositis
  • Scleroderma

Systemic inflammatory diseases 

  • Inflammatory bowel disease
  • Hemophagocytic lymphohistiocytosis (HLH)

Other systemic diseases or exposures

  • Graft-versus-host diseases
  • Radiation
  • Drug-induced CNS vasculitis
  • Haemoglobinopathies
  • Malignancies
  • Other


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