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Eming, Rüdiger; Rech, Jürgen; Barth, Sandra; Kalden, Joachim R.; Schuler, Gerold; Harrer, Thomas; Hertl, Michael

Prolonged Clinical Remission of Patients with Severe Pemphigus upon Rapid Removal of Desmoglein-Reactive Autoantibodies by Immunoadsorption

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  • Medientyp: E-Artikel
  • Titel: Prolonged Clinical Remission of Patients with Severe Pemphigus upon Rapid Removal of Desmoglein-Reactive Autoantibodies by Immunoadsorption
  • Beteiligte: Eming, Rüdiger; Rech, Jürgen; Barth, Sandra; Kalden, Joachim R.; Schuler, Gerold; Harrer, Thomas; Hertl, Michael
  • Erschienen: S. Karger AG, 2006
  • Erschienen in: Dermatology, 212 (2006) 2, Seite 177-187
  • Sprache: Englisch
  • DOI: 10.1159/000090659
  • ISSN: 1421-9832; 1018-8665
  • Entstehung:
  • Anmerkungen:
  • Beschreibung: <i>Background:</i> 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. <i>Objectives:</i> The aim of this intervention study was to evaluate the efficacy and safety of the peptide-based Globaffin<sup>®</sup> adsorber system in the treatment of severe pemphigus cases. <i>Patients and Methods:</i> We applied IA in 4 PV and 2 PF patients with severe chronic disease resistant to conventional immunosuppressive therapy. IA was performed on 4 consecutive days, representing 1 treatment cycle, followed by a 4-week treatment-free interval. Serum samples for determining serum IgG and anti-Dsg1/Dsg3 IgG autoantibodies were drawn daily before and after IA, respectively. During follow-up, patients were examined carefully, and laboratory parameters were controlled monthly for up to 1 year. <i>Results:</i> IA led to excellent clinical responses. Skin and mucosal lesions cleared almost completely within weeks. One IA cycle reduced anti-Dsg1 and anti-Dsg3 autoantibodies by an average of 50–70% as determined by ELISA. <i>Conclusions:</i> Using the Globaffin adsorber system, IA represents an effective and safe treatment opportunity in severe and therapy-resistant pemphigus.