Intravenous Immunoglobulin and Plasma Exchange

PE is a process in which blood is removed from a patient, the plasma and cellular components separated mechanically, and the cellular components then reintroduced to the patient. PE works by simply removing circulating auto-antibodies.

IVIg is the infusion of human immunoglobulin pooled from 3000 to 10 000 donors.36 IVIg is thought to act by several different mechanisms, varying according to the disease. It is thought to negatively impact autoantibody levels by supplying anti-idiotypic antibodies that can potentially neutralize pathogenic autoantibodies, and bind to and negatively regulate antibody production by B cells. In addition, IVIg could theoretically accelerate the catabolism of autoantibodies by saturating the protective transport receptors (FcRn) in endocytotic vesicles. IVIg also appears to act by inhibition of the complement pathway - by inhibiting complement uptake and formation and deposition of membranolytic attack complex. IVIg modulates Fc receptors on macrophages - either by blockade of the receptors or by increasing the ratio of receptors to favor inhibition over the activation of phagocytosis. IVIg also neutralizes superantigens, and could prevent the activation and clonal expansion of superantigen-triggered cytotoxic Tcells; this effect may be relevant in controlling relapses triggered by infections - as seen in MG and CIDP, for example. IVIg might also inhibit Tcell function; indeed, IVIg induces transient lymphopenia.

The onset for both IVIg and PE can vary greatly; for CIDP, it is generally 24-48 h, but can take up to 4 weeks, or a patient may see no effect. The duration of action is short - generally 1-2 months. Further, both IVIg and PE are associated with adverse events, although complications seem to be less with IVIg. Further, both take several hours to administer. IVIg is expensive and sometimes associated with recalls and shortages; in addition, there are many products on the market, although there is no evidence of differences in biological action. While all indications for IVIg are off-label, IVIg or PE are considered first-line therapy for GBS and CIDP, including MMN, and as a second-line therapy for MG, SLE, and MS. Currently, clinical trials for IVIg or PE for MG, CIDP, GBS, and SLE are ongoing.

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