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The gold standard of care for severe cases of autoimmune disease includes corticosteroids and immunosuppressive agents. The major disadvantage of cytotoxic drugs is their serious toxicity profile, leading to an increased risk of severe infection, bone marrow suppression and ovarian failure. Steroid resistance is found in patients with inflammatory and autoimmune diseases, and can be evaluated by testing for lymphocyte activation in the presence of steroids. Furthermore, the glucocorticoid-resistant isoform of the receptor, which is induced in unresponsive patients, can be measured. In addition, an IL-4 polymorphism is found in steroid-resistant patients. Intravenous immunoglobulin (IVIg) significantly reduces IL-2/IL-4 induction of glucocorticoid-resistant receptor binding affinity to corticosteroids [1]. Thus, IVIg may have a steroid-sparing effect. IVIg therapy has less serious adverse effects (AEs) than steroid and cytotoxic treatment and usually, if present, they are mild and transient [2,3]. Treatment with IVIg is indicated in patients with concomitant infection [4], those where treatment with steroids or cytotoxic agents is contraindicated or refused, and those who are resistant to conventional therapy. Thus, IVIg provides an additional good therapeutic method to treat difficult cases [5,6].
This review covers the major advances in the therapeutic potential of IVIg as a steroid-sparing agent in autoimmune diseases published on Medline (1992-2007). The inclusion criteria were articles published in English on Medline, containing the terms steroid-sparing effect and intravenous immunoglobulin. Owing to a relative sparsity of relevant articles, we further extended our search terms: steroid-sparing effect and IVIg, and autoimmune disease, systemic sclerosis, vasculitis, antiphospholipid syndrome, polymyositis, dermatomyositis, idiopathic thrombocytopenic purpura, Guillain-Barre syndrome, systemic lupus erythematosus, pemphigus, myasthenia gravis, multiple sclerosis. We then reviewed articles on IVIg as an empiric therapy in autoimmune diseases that addressed the steroid-sparing effects if mentioned. We included all published articles, including anecdotal reports, small case series and open trials. Exclusion criteria were articles not written in English and studies that investigated the beneficial effects of IVIg alone. Studies on nonautoimmune diseases were also not included. We briefly describe the indications, mechanisms and AEs of IVIg.
A total of 17 articles were retrieved and the relevant ones are discussed below by disease. For autoimmune diseases, such as systemic sclerosis, vasculitis, antiphospholipid syndrome (APS), polymyositis, dermatomyositis, idiopathic thrombocytopenic purpura (ITP) and Guillain-Barre syndrome, no articles were found that...