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 |
| Dermatomyositis |
| Systemic sclerosis |
| Mixed connective tissue disease |
| Sarcoidosis |
| Drugs |
| Cocaine |
| Amphetamine |
| Epinephrine/mimics |
| Infections/immune complexes |
| Viral |
| Varicella zoster, HIV |
| Bacteria |
| Syphilis, tuberculosis, mycoplasma, rickettsia |
| Fungi |
| Aspergillosis, mucormycosis, histoplasma |
| Coccidioidomycosis, candidosis |
| Parasites |
| Cysticercosis, toxoplasma |
| Secondary cryoglobulins, immune complexes |
| Hepatitis C, hepatitis B, cytomegalovirus, Epstein-Barr virus, parvovirus B19 |
| Lyme disease, malaria |
| Malignancy |
| Hodgkin's and non-Hodgkin lymphomas |
| Paraneoplasia |
| 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.