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The systemic vasculitides are a complex and often serious group of disorders which, while uncommon, require careful management in order to ensure optimal outcome. In most cases there is no known cause. Multi-system disease is likely to be fatal without judicious use of immunosuppression. A prompt diagnosis is necessary to preserve organ function. Comprehensive and repeated disease assessment is a necessary basis for planning therapy and modification of treatment protocols according to response. Therapies typically include glucocorticoids and, especially for small and medium vessel vasculitis, an effective immunosuppressive agent. Cyclophosphamide is currently the standard therapy for small vessel multi-system vasculitis, but other agents are now being evaluated in large randomized trials. Comorbidity is common in patients with vasculitis, including the cumulative effects of potentially toxic therapy. Long-term evaluation of patients is important in order to detect and manage relapses.

Original publication

DOI

10.1111/j.1365-2249.2009.04078.x

Type

Journal article

Journal

Clinical and experimental immunology

Publication Date

05/2010

Volume

160

Pages

143 - 160

Addresses

Nuffield Orthopaedic Centre, Oxford, UK.

Keywords

Animals, Mice, Knockout, Humans, Mice, Inflammation Mediators, Immunosuppressive Agents, Antibodies, Antineutrophil Cytoplasmic, Autoantibodies, Diagnostic Imaging, Biopsy, Physical Examination, Early Diagnosis, Incidence, Interdisciplinary Communication, Drug Design, Patient Care Team, Clinical Trials as Topic, Multicenter Studies as Topic, Systemic Vasculitis