The diagnosis of vasculitis is first and foremost a clinical one. Corr
ect diagnosis requires a high index of suspicion coupled with knowledg
e of the manifestations of other disorders that may masquerade as vasc
ulitis. Treatment of vasculitis requires prolonged use of drugs with t
he potential for serious side effects. Whereas the prompt initiation o
f definitive treatment is a very high priority, there is also substant
ial risk of inappropriately treating self-limited and more benign diso
rders mimicking vasculitis. This has been a particular problem with pr
imary angiitis of the central nervous system. Laboratory studies, part
icularly tissue biopsy, provide a crucial adjunct to clinical diagnosi
s.