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Improved protocol for treatment of pemphigus vulgaris with protein A immunoadsorption

Authors

  • I. Shimanovich,

    1. Department of Dermatology, University of Lübeck, Germany; Departments of *Dermatology and †Transfusion Medicine, University of Würzburg, Germany; and ‡Department of Dermatology, University Hospital Mannheim, University of Heidelberg, Germany
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  • S. Herzog,

    1. Department of Dermatology, University of Lübeck, Germany; Departments of *Dermatology and †Transfusion Medicine, University of Würzburg, Germany; and ‡Department of Dermatology, University Hospital Mannheim, University of Heidelberg, Germany
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  • E. Schmidt,

    1. Department of Dermatology, University of Lübeck, Germany; Departments of *Dermatology and †Transfusion Medicine, University of Würzburg, Germany; and ‡Department of Dermatology, University Hospital Mannheim, University of Heidelberg, Germany
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  • A. Opitz,

    1. Department of Dermatology, University of Lübeck, Germany; Departments of *Dermatology and †Transfusion Medicine, University of Würzburg, Germany; and ‡Department of Dermatology, University Hospital Mannheim, University of Heidelberg, Germany
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  • E. Klinker,

    1. Department of Dermatology, University of Lübeck, Germany; Departments of *Dermatology and †Transfusion Medicine, University of Würzburg, Germany; and ‡Department of Dermatology, University Hospital Mannheim, University of Heidelberg, Germany
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  • E-B. Bröcker,

    1. Department of Dermatology, University of Lübeck, Germany; Departments of *Dermatology and †Transfusion Medicine, University of Würzburg, Germany; and ‡Department of Dermatology, University Hospital Mannheim, University of Heidelberg, Germany
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  • M. Goebeler,

    1. Department of Dermatology, University of Lübeck, Germany; Departments of *Dermatology and †Transfusion Medicine, University of Würzburg, Germany; and ‡Department of Dermatology, University Hospital Mannheim, University of Heidelberg, Germany
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  • D. Zillikens

    1. Department of Dermatology, University of Lübeck, Germany; Departments of *Dermatology and †Transfusion Medicine, University of Würzburg, Germany; and ‡Department of Dermatology, University Hospital Mannheim, University of Heidelberg, Germany
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  • Conflict of interest: none declared.

Dr Detlef Zillikens, Department of Dermatolgy, University of Lübeck, Ratzeburger Allee 160, 23538 Lübeck, Germany.
E-mail: detlef.zillikens@uk-sh.de
The first two authors contributed equally to this work.

Summary

Background.  Pemphigus vulgaris is a life-threatening autoimmune blistering skin disease, usually treated with high-dose corticosteroids in combination with other immunosuppressants. However, this regimen may prove inadequate in severe cases and can cause dangerous side-effects. We have recently reported protein A immunoadsorption (PAIA) to be an effective adjuvant treatment for induction of remission in severe pemphigus. However, in a significant number of cases, the disease rapidly recurred once PAIA and immunosuppressive medication were tapered.

Aims.  The aim of the present study was to develop a PAIA-based therapeutic regimen that would result in a more prolonged remission of pemphigus.

Methods.  Nine patients with pemphigus vulgaris were treated with a modified protocol characterized by a combination of PAIA with a higher initial dose of systemic methylprednisolone (2 mg/kg). In addition, azathioprine or mycophenolate mofetil was administered as a steroid-sparing agent.

Results.  In all nine patients treated with this regimen, we observed a sharp decline of circulating autoantibody levels and dramatic improvement of cutaneous and mucosal lesions within 4 weeks of therapy. The patients remained free of clinical disease for up to 26 months after PAIA treatment was discontinued.

Conclusion. The improved treatment protocol appears to combine highly effective induction of clinical remission in severe or treatment-resistant pemphigus with a prolonged subsequent symptom-free interval.

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