IVIg TREATMENT OF PEMPHIGUS: How it Works and How to Use it

Improvement of Intravenous Immunoglobulin Therapy for Bullous Pemphigoid by Adding Immunosuppressive Agents

“…8 These findings are consistent with those of our group's prior studies, which have shown that this rebound can be reduced in animals 11 and in humans 12 by using immunosuppressive agents that inhibit antibody synthesis. Our results are nearly identical to those reported previously that the rebound in antibody levels that follows pemphigus antibody depletion by plasmapheresis was suppressed by the concurrent administration of an immunosuppressive agent.…”

“…We have speculated that the coadministration of an immunosuppressive agent will similarly improve the effectiveness of IVIg in pemphigus and other autoantibody-mediated blistering diseases. 8 This hypothesis is supported by our observation herein of a patient with autoantibody-mediated bullous pemphigoid who was repeatedly treated with IVIg given with or without an immunosuppressive drug. This permitted the relative effectiveness of these 2 approaches of using IVIg to be compared in the same patient.…”

“…A variety of mechanisms have been proposed, 7 but the most likely is that it rapidly and selectively lowers serum levels of the autoantibodies that mediate the disease. 8 Our research group has found that 2 weeks after a single cycle of IVIg treatment, serum levels of the autoantibodies that mediatepemphigusvulgariscandecreasebyover 60%comparedwith16%after3weeksofcon-ventional treatment with high doses of steroids and immunosuppressive drugs. 9,10 However, a physiologic regulatory feedback mechanism maintains a constant level of individual antibodies in serum.…”

Effect of Intravenous Immunoglobulin Therapy on Serum Levels of IgG1 and IgG4 Antidesmoglein 1 and Antidesmoglein 3 Antibodies in Pemphigus Vulgaris

“…Intravenous immunoglobulin seems to be effective in the treatment of PV by increasing the catabolism of all immunoglobulin molecules, which results in a specific decrease in only pathogenic antibodies, inasmuch as serum levels of normal antibodies are maintained by those present in the IVIG preparation. 10 If this explanation is correct, the rate of catabolism of individual classes and subclasses of antibodies depends on their relative concentration in patient serum and in the IVIG preparation. Subclasses in which serum levels are increased to a relatively greater extent, either because they are present to a larger amount in the IVIG preparation or are particularly low in the patient, will be degraded more rapidly, resulting in a more rapid decrease in that IgG subclass.…”

Effect of intravenous immunoglobulin with or without cytotoxic drugs on pemphigus intercellular antibodies

“…6,7 It results in a rebound in the serum levels of the depleted antibody which can exceed that present before depletion. This rebound may limit the effectiveness of IVIg and suppressing it with cytotoxic drugs could improve the effectiveness of this treatment.…”