ABSTRACT
The primary systemic vasculitides have an annual incidence of 40/million and are classified according to their association with anti-neutrophil cytoplasm autoantibodies (ANCAs). Those predominantly involving microscopic vessels without immune deposits include Wegener’s granulomatosis, microscopic polyangiitis and Churg-Strauss angiitis, and are usually ANCA-positive and represent 50-75% of the total. The ANCAnegative group include those with immune deposits, such as Henoch-Schönlein purpura and cryoglobulinemia or those restricted to muscular arteries, including polyarteritis nodosa and giant cell and Takayasu’s arteritis1,2. The treatment of primary systemic vasculitis with high-dose corticosteroids and immune-suppressive drugs is generally effective in controlling clinical evidence of disease activity in new presentations3,4. This approach is complicated by frequent, severe toxicity and infections, a high relapse rate and chronic morbidity with increasing incapacity. Following demonstration of the efficacy of intravenous immunoglobulin (IVIG) in reducing the incidence of coronary artery aneurysms in the childhood vasculitis, Kawasaki disease, there has been interest in treating other vasculitides with IVIG.
