2006
DOI: 10.1159/000090659
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Prolonged Clinical Remission of Patients with Severe Pemphigus upon Rapid Removal of Desmoglein-Reactive Autoantibodies by Immunoadsorption

Abstract: Background: In pemphigus, loss of epidermal adhesion is induced by binding of circulating autoantibodies to the desmosomal cadherins desmoglein 3 (Dsg3) in pemphigus vulgaris (PV) and desmoglein 1 (Dsg1) in pemphigus foliaceus (PF), respectively. Therapeutic removal of Dsg-reactive autoantibodies by immunoadsorption (IA) has been demonstrated to exert clinical remission of the disease. Objectives: The aim of this intervention study was to evaluate the efficacy and safety of the peptide-based Globaffin® Show more

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Cited by 55 publications
(56 citation statements)
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“…IA with the Globaffin adsorber system effectively reduced Dsg1/3-reactive IgG by an average of 50–70% per IA cycle accompanied by a rapid clinical improvement of cutaneous and mucosal lesions within 4–8 weeks. Within a follow-up period of up to 2 years, 3 patients still remained in complete remission on minimal therapy and 3 patients showed clinical relapses with residual cutaneous and mucosal erosions [19]. As previously shown by Amagai et al [23], IA with columns that were coated with recombinant Dsg3 effectively reduced pathogenic IgG in a passive transfer mouse model of pemphigus.…”
Section: Discussionmentioning
confidence: 84%
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“…IA with the Globaffin adsorber system effectively reduced Dsg1/3-reactive IgG by an average of 50–70% per IA cycle accompanied by a rapid clinical improvement of cutaneous and mucosal lesions within 4–8 weeks. Within a follow-up period of up to 2 years, 3 patients still remained in complete remission on minimal therapy and 3 patients showed clinical relapses with residual cutaneous and mucosal erosions [19]. As previously shown by Amagai et al [23], IA with columns that were coated with recombinant Dsg3 effectively reduced pathogenic IgG in a passive transfer mouse model of pemphigus.…”
Section: Discussionmentioning
confidence: 84%
“…Based on a recent study, showing that IA led to a rapid decrease in anti-Dsg1 and anti-Dsg3 autoantibodies [19], IA was administered in PV patients with high serum autoantibody titers. Treatment with IA reduced IgG titers against Dsg1 and Dsg3 faster than Rtx.…”
Section: Discussionmentioning
confidence: 99%
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“…Bullous diseases so far treated with IA had all been IgG mediated and mainly included pemphigus and a few patients with bullous pemphigoid, pemphigoid gestationis and epidermolysis bullosa acquisita [3,4,5,6,7]. Advantages of IA compared to plasmapheresis comprise the more specific reduction of immunoglobulin levels, the processing of larger plasma volumes and the avoidance of substitution with foreign proteins [7].…”
Section: Discussionmentioning
confidence: 99%
“…Immunoadsorption (IA) allows the rapid reduction of circulating autoantibodies. Following its successful adjuvant application in treatment-refractory pemphigus [3,4,5,6], IA has been increasingly used for various other IgG-mediated severe and/or treatment-resistant autoimmune bullous diseases including bullous pemphigoid, pemphigoid gestationis and epidermolysis bullosa acquisita [5,7]. However, IA has not been applied in IgA-mediated blistering autoimmune diseases so far.…”
Section: Introductionmentioning
confidence: 99%