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Home arrow Environment arrow Inflammatory Disorders of the Nervous System: Pathogenesis, Immunology, and Clinical Management
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Short-Term Immunomodulating Therapies

Plasma exchange (PE) and intravenous immunoglobulin (IVIg) have rapid onset of action with improvement within days, but this is a transient effect. They are used in certain situations such as myasthenic crisis and preoperatively before thymectomy or other surgical procedures. They can be used intermittently to maintain remission in patients with MG who are not well controlled despite the use of chronic immunomodulating drugs.

Plasma Exchange (PE)

PE improves strength in most patients with MG by directly removing AChR from the circulation [48]. Typically one exchange is done every other day for a total of four to six times. Adverse effects of PE include hypotension, paresthesias, infections, thrombotic complications related to venous access, and bleeding tendencies due to decreased coagulation factors [50].

Intravenous Immunoglobulin Therapy (IVIg)

IVIg are preparations of immunoglobulins isolated from pooled human plasma by ethanol cryoprecipitation. IVIg is usually administered for 5 days at a dose of 0.4 g/ kg/day. Different doses and schedules involving fewer infusions at higher doses are also used. The mechanism of action of IVIg is complex. Therapeutic mechanisms include inhibition of cytokines, competition with autoantibodies, and inhibition of complement deposition. Interference with the binding of Fc receptor on macrophages, Ig receptor on B cells, and interference with antigen recognition by sensitized T cells are other mechanisms [50]. More specific techniques to remove pathogenic anti-AChR antibodies utilizing immunoadsorption have been developed recently and offer a more targeted approach to MG treatment. Clinical trials showed significant reduction of blocking antibodies with concomitant clinical improvement in patients treated with immunoadsorption techniques [41].

IVIg is considered to be relatively safe, but rare cases of severe complications such as thrombosis, renal insufficiency, volume overload, and hemolytic anemia are reported [42].

Compared to plasma exchange, IVIg is similar in terms of efficacy and complication rates [43]. However, plasma exchange (PE) has considerable cost advantages over IVIg with a cost-benefit ratio of 2:1 for treatment of myasthenia gravis [44].

Long-Term Immunotherapies

The goal of immune-directed therapy of MG is to induce a remission or near remission of the disease.

 
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