| Primary CNS vasculitis |
- Evidence of vasculitis exclusively on angiography: Children, progressive angiography-positive childhood PACNS and nonprogressive angiography-positive childhood PACNS subtypes; adults, PACNS (51% in Mayo clinic cohort)
- Evidence of vasculitis exclusively on brain biopsy: Children, small-vessel childhood PACNS; adults, PACNS (25% in Mayo clinic cohort)
- Evidence of vasculitis on angiography and brain biopsy: Children, single cases of childhood PACNS; adults, PACNS (24% in Mayo clinic cohort)
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| Childhood secondary CNS vasculitis |
- Active infections or postinfectious states: Viral (VZV, HIV, EBV, CMV); bacterial (Mycobacterium tuberculosis, Mycoplasma pneumonia, Streptococcus pneumoniae); fungal (Aspergillus spp, Candida albicans, Actinomyces spp)
- Systemic rheumatic diseases: SLE, systemic vasculitis, scleroderma, dermatomyositis, plus others
- Systemic inflammatory diseases: Inflammatory bowel disease, hemophagocytic lymphohistiocytosis, Kawasaki syndrome
- Other systemic diseases or exposures: Graft-versus-host disease, radiation, malignancies
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| Adult secondary CNS vasculitis |
- Active infections or postinfectious states: Viral (VZV, HIV, CMV, HBV, HCV, HSV); bacterial (Borrelia burgdorferi [Lyme disease], Treponema pallidum [syphilis], M. tuberculosis, Salmonella typhi, Streptococcus pneumoniae); fungal (Aspergillus, Coccidoides, Mycomycoses, Histoplasma capsulatum, Toxoplasma); protozoa (Toxoplasma, Plasmodium [malaria])
- Systemic rheumatic diseases: Behçet syndrome, systemic vasculitis (giant cell arteritis, Takayasu arteritis, polyarteritis nodosa and ANCA-associated vasculitis), SLE, sarcoidosis or neurosarcoid, RA, Sjögren syndrome, scleroderma
- Systemic inflammatory diseases: Inflammatory bowel disease, hemophagocytic lymphohistiocytosis, cryoglobulinemia
- Other systemic diseases or exposures: Graft-versus-host diseases, radiation, drugs (cocaine, amphetamines, among others), malignancies (Hodgkin and non-Hodgkin lymphoma, among others)
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