Vasculitides comprise a heterogeneous group of inflammatory diseases of the blood vessels, which are characterized both by their diversity and an overlap of clinical and pathological findings and manifestations. The etiology of most vasculitides is unknown. Based on pathological and immunological features, vasculitides are subdivided into major categories affecting large, medium-sized and small vessels. Early diagnosis and prompt institution of immunosuppressive therapy may be delayed due to the rarity of vasculitides and their complex clinical presentation, rapid and unpredictable courses, and lack of unambiguous diagnostic laboratory tests. Moreover, access to an appropriate tissue for histological examination may not always be feasible and distinctive clinical features therefore serve as surrogate criterions. Prior to the introduction of immunosuppressive therapy, systemic vasculitis used to be an inevitably fatal condition. With the advent of corticosteroids and establishment of immunosuppressive treatment protocols through randomized trials, outcomes and survival rates have improved. Studies using biologicals have been shown to further improve therapy, e.g. rituximab for the maintenance of remission in ANCA-associated vasculitis. Based on trials and other reports, national and international recommendations for the treatment of the different categories of vasculitides have been compiled. Modern immunosuppressive treatment options have considerably improved the prognosis of vascultides, so that the major challenges are now chronicity, relapses and complicating comorbidities. Thus, a need for safer and more effective targeted therapies remains.
Disclosure of Interest None declared
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