Since the effectiveness of high dose intravenous immunoglobulin (IVIg)
was first demonstrated in autoimmune thrombocytopenia, IVIg has been
investigated in the treatment of various autoimmune rheumatic disorder
s. Controlled randomised studies have established the efficacy of IVIg
in Kawasaki's syndrome, for which combined IVIg and aspirin (acetylsa
licylic acid) now constitutes the standard treatment. Another controll
ed study has demonstrated the benefit of IVIg in dermatomyositis. IVIg
treatment in juvenile rheumatoid arthritis has produced contradictory
results. Uncontrolled studies and case reports on the application of
IVIg in systemic lupus erythematosus, ANCA-associated vasculitides and
adult rheumatoid arthritis generally describe short term positive eff
ects. Various mechanisms are thought to underlie the effect of IVIg on
autoimmune rheumatic diseases, such as: (i) blockade of Fc receptorsr
(ii) immunomodulation via anti-idiotypic interactions: (iii) inhibiti
on of complement-mediated tissue damage; (iv) modulation of cytokine e
xpression by leucocytes and endothelial cells; and (v) inhibition of s
uperantigen-mediated T cell activation. IVIg is considered to be a low
-risk form of treatment. Reported adverse effects include headache, as
eptic meningitis and transient impairment of renal function. Haemolysi
s and anaphylactic reactions are rare. The effect profile of IVIg make
s it a relevant, although still experimental, form of treatment in aut
oimmune rheumatic disorders, but its high cost renders it unsuitable a
s a first-line treatment. Because IVIg does not weaken patients' resis
tance to infection, it might serve as a therapeutic option in bridging
clinical situations where immunosuppressive or cytotoxic approaches a
re contraindicated in patients with autoimmune disorders, such as inte
rcurrent infection or in the period immediately before and after surge
ry.