The evidence base for treatment of systemic vasculitis is reviewed in this
article. Accurate diagnosis and evaluation of disease status is crucial to
the appropriate management of these cases and disease status should be empl
oyed as a means of staging so that appropriate therapy can be offered tailo
red to the individual requirements of each case. Large vessel vasculitides
are almost exclusively managed with corticosteroids. Small vessel vasculiti
s with internal organ involvement usually requires more aggressive immune-s
uppression; by contrast, small vessel vasculitis without internal organ inv
olvement rarely requires intervention. Despite our current efforts, the lon
g-term morbidity of patients with systemic vasculitis remains high. We need
better therapeutic strategies in order to limit or control disease rapidly
and thereby prevent the accumulation of subsequent damage. Large-scale ran
domised trials comparing different therapeutic strategies offer the only ho
pe of establishing a firmer evidence base for the optimal management of the
se diseases.