Initially,
intravenous immunoglobulins (IVIgs) were used as replacement
therapy in primary and secondary
antibody-deficiency syndromes. The clinical use of
IVIg has been extended during the past decade to a wide variety of clinical conditions, such as infectious processes, neuroimmunological diseases, and different systemic
autoimmune diseases. The mode of action of
IVIg is complex, involving modulation of the
Fc receptors, interference with the
complement and
cytokine network, and effects on the activation and differentiation of T and B-cells.
Kawasaki disease (KD) was one of the first diseases within the group of primary
vasculitides in which
IVIg were used. Today, there is a clear evidence of benefit for
IVIg in the treatment of coronary artery abnormalities related to KD. Subsequently, various reports have suggested a beneficial effect in other
vasculitides; however, there are few data from controlled studies. For
antineutrophil cytoplasmic antibody-associated
vasculitis (AAV) one placebo-controlled and several open-label studies have shown a beneficial effect on the disease activity in patients with
Wegener's granulomatosis or
microscopic polyangiitis refractory to standard
therapy with
prednisone and
cyclophosphamide. For other
vasculitides, such as
polyarteritis nodosa or
Henoch-Schonlein purpura, only case reports have described an inhibition of a
disease progression by
IVIg so far. However, the effect was partly only temporary. In conclusion, KD and AAV are the only
vasculitides with a definite beneficial use of
IVIg. For other
vasculitides, the efficacy of
IVIg has not been proven properly but may be useful in single cases.