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Third-party-funded project

Title:
Immunoapheresis in cutaneous autoimmune diseases

Project management at the University of Würzburg:

Participating scientists:

Abstract:
Background: Pemphigus foliaceus (PF) and pemphigus vulgaris (PV) are autoimmune blistering skin diseases usually treated with high-dose systemic corticosteroids and other immunosuppressants that may cause severe side effects. Plasmapheresis has also been demonstrated to be of benefit in the treatment of pemphigus. In contrast to plasmapheresis, staphylococcal protein A immunoadsorption (PA-IA) specifically removes immunoglobulin from the circulation, allows treatment of larger plasma volumes, and does not require the substitution of plasma components.
Objectives: To determine effectiveness and side-effects of PA-IA in patients with severe pemphigus.
Methods: Five patients with severe pemphigus (PV, n=4; PF, n=1) were treated by PA-IA. Three of these patients had been refractory to various treatment regimens. In addition to PA-IA, methylprednisolone, 0.5 mg/kg body weight/d was given initially and subsequently tapered.
Results: In all patients, a dramatic clinical improvement was seen within two weeks after initiation of therapy. Patients were free of lesions after 3, 4, 4, 10, and 21 weeks of treatment, respectively. Concurrently, autoantibody levels decreased rapidly.
Conclusion: PA-IA is a rational, effective, and safe adjuvant therapy for severe pemphigus and warrants the wider use for this indication. A controlled study should compare side effects and effectiveness of PA-IA with other treatment options for pemphigus.

Key words:
    Pemphigus
    Desmoglein
    Immunoapheresis
    Desmoglein

Projekt period: from 01.2001 to 12.2001

Funding institution:
Wirtschaftsunternehmen ,Granting date: 15.12.2000

Publications: