Clinical and Experimental Immunology

Intravenous immunoglobulin (IVIG) is a blood product prepared from the serum of between 1000 and 15 000 donors per batch. It is the treatment of choice for patients with antibody deficiencies. For this indication, IVIG is used at a ‘replacement dose’ of 200–400 mg/kg body weight, given approximately 3-weekly. In contrast, ‘high dose’ IVIG (hdIVIG), given most frequently at 2 g/kg/month, is used as an ‘immunomodulatory’ agent in an increasing number of immune and inflammatory disorders. Initial use of hdIVIG was for immune thrombocytopenic purpura (ITP) in children [1]. The clinical specialities using the largest amounts of IVIG are neurology, haematology, immunology, nephrology, rheumatology and dermatology. IVIG has had a major impact on the treatment of neurological disorders including dermatomyositis, Guillain–Barre syndrome, chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy (MMN), myasthenia gravis and stiff person syndrome. In haematology it is used to treat immune cytopenias, parvovirus B19 associated red cell aplasia, hypogammaglobulinaemia secondary to myeloma and chronic lymphatic leukaemia and post-bone marrow transplantation. In immunology IVIG is used in the treatment of primary antibody deficiency (PAD), in nephrology, rheumatology and ophthalmology it has been used to treat vasculitis, systemic lupus erythematosis (SLE), mucous membrane pemphigoid and uveitis and in dermatology it is used most commonly to treat Kawasaki syndrome, dermatomyositis, toxic epidermal necrolysis and the blistering diseases (Table 1). In this paper, we review recent developments in the understanding of mechanisms of action of IVIG, the major current clinical areas of use concentrating on conditions with a better established evidence base and discuss emerging areas of use of IVIG.