In this study, we examined antibody titers of desmoglein (Dsg) in serum, waste fluid, and saliva detected by enzyme-linked immunosorbent assay (ELISA) and serum IgG values during double filtration plasmapheresis (DFPP) treatment of three patients with persistant pemphigus. The first patient suffered from mucosal dominant pemphigus vulgaris, second one from pemphigus vegetans, and third from mucocutaneous pemphigus vulgaris; all were unresponsive to standard managements such as systemic steroid therapy and had to stop immunosuppressive drug because of the side effects. The DFPP treatment resulted in a highly successful removal rate of both anti-Dsg1 and anti-Dsg3 autoantibodies, 41.1±8.5% and 40.2±12.6%, respectively. The removal rate of serum IgG was lower (34.4±10.7%) than that of anti-Dsg autoantibodies. In order to avoid a rebound flare of the circulating anti-Dsg autoantibodies, three intravenous injections (i.v.) of methyl prednisolone sodium succinate (m-PSL) (40 mg/day) and three treatments either i.v. of m-PSL (1,000 mg/day) or per os (p.o.) of betamethasone were given after DFPP. The three treatments with m-PSL (1,000 mg/day) and the increased dose of betamethasone were very effective. The antibody titers of Dsg in their sera and waste fluids closely paralleled each other during DFPP treatment; thus we could check the effectiveness of removal of anti-Dsg autoantibodies. There were no anti-Dsg autoantibodies in the saliva. Our results suggest that various approaches are necessary in the high quality treatment of patients with pemphigus during DFPP.
View full abstract