Protein A immunoadsorption: a novel and effective adjuvant treatment of severe pemphigus

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Abstract
Summary 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 also has 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 the 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−1 body weight day−1 was given initially and subsequently tapered. Results In all patients, a dramatic clinical improvement was seen within 2 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. Conclusions PA–IA is a rational, effective, and safe adjuvant therapy for severe pemphigus and warrants wider use for this indication. A controlled study should compare side-effects and effectiveness of PA–IA with other treatment options for pemphigus.

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