Table 1

Conditions associated with CNS vasculitis

Idiopathic/isolated/primary cerebral/CNS vasculitis
 Amyloid-β-related angiitis
 (Eale’s disease and
 Cogan’s syndrome*)
Secondary CNS vasculitis
 Systemic vasculitides
 Granulomatosis with polyangiitis
 Churg-Strauss syndrome
 Behçet's disease
 Microscopic polyarteritis nodosa
 Classical polyarteritis nodosa
 Small-vessel vasculitis (including Henoch-Schönlein purpura)
 Kawasaki disease
 Giant cell arteritis
 Takayasu's arteritis
 Connective tissue diseases
 Systemic lupus erythematosus
 Antiphospholipid antibody syndrome
 Rheumatoid arthritis
 Sjögren's syndrome
 Systemic sclerosis
 Mixed connective tissue disease
Infections/immune complexes
  Varicella zoster, HIV
  Syphilis, tuberculosis, mycoplasma, rickettsia
  Aspergillosis, mucormycosis, histoplasma
  Coccidioidomycosis, candidosis
  Cysticercosis, toxoplasma
 Secondary cryoglobulins, immune complexes
  Hepatitis C, hepatitis B, cytomegalovirus, Epstein-Barr virus, parvovirus B19
  Lyme disease, malaria
 Hodgkin's and non-Hodgkin lymphomas
 Lymphomatoid granulomatosis
 Malignant angioendotheliomatosis
  • *Eales’ and Cogan’s syndromes are idiopathic disorders characterised histopathologically by vasculitis. Eales’ disease mainly involves the retina and Cogan’s syndrome involves mainly the eye and the inner ear; both can (uncommonly) involve brain parenchyma. Since the inner ear and the eye may both arguably be considered parts of the nervous system, they could be said to be primary CNS vasculitides, hence their guarded inclusion.

  • CNS, central nervous system.