Medication Summary

Prednisone, the most commonly used first-line agent for Evans syndrome, often effectively controls acute episodes, though relapses may be frequent when patients are weaned off prednisone. Intravenous immune globulin (IVIg) may help patients who depend on steroids.

Class Summary

Steroids are the usual initial therapy for Evans syndrome. They possess anti-inflammatory properties and cause profound and varied metabolic effects. They modify the immune response of the body to diverse stimuli.

Prednisone (Rayos)

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Prednisone is a commonly used steroid that interferes with macrophage Fc gamma and C3b receptors responsible for destruction of red blood cells and platelets. It may work by decreasing abnormal immunoglobulin G (IgG) production that may be responsible for destruction of the cells. Prednisone can increase vascular stability and ameliorate endothelial abnormalities associated with thrombocytopenia; it also helps decrease clearance of opsonized platelets.

Class Summary

This is a purified preparation of gamma globulin. It is derived from large pools of human plasma and is composed of 4 subclasses of antibodies, approximating the distribution of human serum.

Immune globulin intravenous (Carimune NF, Bivigam, Hizentra, Gammagard, Octagam)

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IVIg neutralizes circulating myelin antibodies through anti-idiotypic antibodies. It down-regulates proinflammatory cytokines (eg, interferon gamma), blocks Fc receptors on macrophages, suppresses inducer T and B cells, and augments suppressor T cells. IVIg also blocks the complement cascade and promotes remyelination. In addition, it may increase IgG in cerebrospinal fluid.

Questions

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