Recommendations for the use of immunoapheresis in the treatment of autoimmune bullous diseases

Abstract

Despite the use of high-dose systemic corticosteroids in combination with other immunosuppressants, in some patients with autoimmune bullous diseases only insufficient improvement is achieved. In these cases and in acute severe disease, adjuvant immunoapheresis has been increasingly used. A consensus meeting was held in mid-2005 in Hamburg, aiming at developing guidelines for the use of immunoapheresis in the treatment of autoimmune bullous diseases. This paper summarizes the experts' recommendations.

Pemphigus. S2 Guideline for diagnosis and treatment – guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV)

“…Immunoadsorption is considered most effective in combination with systemic immunosuppressive drugs. 22,27,28 • Generally, four treatments of immunoadsorption are performed on four consecutive days (2.5-fold plasma volume/ day)…”

Clinical and Immunological Follow-Up of Pemphigus Patients on Adjuvant Treatment with Immunoadsorption or Rituximab

“…Recently, numerous case reports and small case series strongly suggest that IA as an adjuvant treatment is highly efficient in removing pathogenic autoantibodies in pemphigus and other autoimmune bullous skin disorders [12]. …”

Linear IgA Disease: Successful Application of Immunoadsorption and Review of the Literature

“…Bullous diseases so far treated with IA had all been IgG mediated and mainly included pemphigus and a few patients with bullous pemphigoid, pemphigoid gestationis and epidermolysis bullosa acquisita [3,4,5,6,7]. Advantages of IA compared to plasmapheresis comprise the more specific reduction of immunoglobulin levels, the processing of larger plasma volumes and the avoidance of substitution with foreign proteins [7].…”

“…Immunoadsorption (IA) allows the rapid reduction of circulating autoantibodies. Following its successful adjuvant application in treatment-refractory pemphigus [3,4,5,6], IA has been increasingly used for various other IgG-mediated severe and/or treatment-resistant autoimmune bullous diseases including bullous pemphigoid, pemphigoid gestationis and epidermolysis bullosa acquisita [5,7]. However, IA has not been applied in IgA-mediated blistering autoimmune diseases so far.…”