The systemic vasculitides are an uncommon group of autoimmune diseases capable of causing multi organ failure and death. Clinical evaluation tools have been developed and provide a comprehensive assessment of patients. Serological markers, especially anti-neutrophil cytoplasm antibody (ANCA), pathology and imaging investigations are a useful addition, but are more valuable in diagnosis rather than monitoring of disease activity.
Primary vasculitides: Kawasaki disease; Behçet's disease; Polyarteritis nodosa or PAN; Wegener's granulomatosis; Cryoglobulinemia ; Takayasu's arteritis or pulseless disease; Churg-Strauss syndrome; Giant cell arteritis or temporal arteritis; Henoch-Schönlein purpura.