Purpose: The presence of IgG auto-antibodies (auto-AB) plays an important role in the pathogenesis of pemphigus vulgaris (PV) and pemphigus foliaceus (PF). Since standard therapy often dose not result in a significant clinical benefit, the aim of the present study was to approve a new therapeutic strategy by immunoadsorption (IA). Methods: 5 patients (4 adults, age 43 to 67y. 1 child, 12y) were treated with regenerable staphylococcal protein A adsorber. Protocoll: day 1-3: daily IA. day 4-6: application of i.v. steroids (500mg/d prednisolon equivalent), day 4 i.v. cyclophosphamid 500mg (except the child), day 7-290: repeated lAs with prolonged intervals up to 4 weeks and decreasing low dose oral steroid application. Processed plasma volumes: 3-8 liters. Results: In all cases IA was tolerated well without severe side effects. Although 4 patients had been refractory to the standard therapies, in all patients a good clinical improvement by 1A could be observed. In 1 patient clinical remission remains meanwhile for over 1 year after stop of IA. Auto-ABs decreased in all cases by > 80%, in two of them auto-AB became negativ and skin lesions completely disappeared. Conclusions: PA-IA is an rational, effectiv and safe treatment modality for PV and PF. Our results encourage to further clinical studies for treatment of pemphigus by 1A.
|Zeitschrift||Infusionstherapie und Transfusionsmedizin|
|Seiten (von - bis)||34|
|Publikationsstatus||Veröffentlicht - 2001|
Strategische Forschungsbereiche und Zentren
- Forschungsschwerpunkt: Infektion und Entzündung - Zentrum für Infektions- und Entzündungsforschung Lübeck (ZIEL)