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. Primary systemic vasculitis has an incidence of more than 100 new cases per million [1]. Pathogenic mechanisms remain uncertain, although understanding the viral aetiology of some

forms of polyarteritis nodosa (linked to hepatitis B) and cryoglobulinaemic vasculitis (linked to hepatitis C) has allowed a more tailored management approach [2,3]. Despite a significant

Peptide 17 reduction in mortality as a result of standard immunosuppression, most patients experience poor quality of life, characterized by relapse, persisting low-grade disease activity and increasing burden of drug toxicity [4–6]. Factors influencing remission, relapse and survival include type of immunosuppressive therapy, type of organ involvement, presence of anti-neutrophil cytoplasm antibodies (ANCA), older age and male gender [7]. A structured approach, based on careful disease staging and evaluation, is the cornerstone of good disease management [8]. The relationship GSK126 in vitro between ANCA and Wegener’s granulomatosis and microscopic polyangiitis suggests a pathogenic role [9]. Targeting ANCA or monitoring levels to assess disease activity have both been attempted as treatment strategies, but with limited success [10–12]. Initial evaluation includes a comprehensive clinical assessment, serological tests, histology and radiology. For subsequent evaluations, it is effective and practical to measure clinical disease status for most patients with small

and medium vessel vasculitis [8]. For large vessel disease such as Takayasu’s arteritis, while radiological assessment of vascular C-X-C chemokine receptor type 7 (CXCR-7) anatomy is possible, the correlation of imaging findings may be poor [13]. Therapy is based on the pattern of vasculitis and on careful evaluation of the extent and activity of disease. We will review the evidence for treatment including glucocorticoids and immunosuppressive agents in different forms of vasculitis. There is increasing experience in the use of more specific biological therapies in patients with vasculitis which will also be discussed. The subtlety and diversity of symptoms in the initial phase of vasculitis can be a real diagnostic problem, and thus early recognition of a vasculitic condition relies on the experience of a team of dedicated professionals from several different subspecialties, including laboratory medicine.