Adjuvant treatment of severe/ refractory bullous pemphigoid with protein a immunoadsorption

Background: While depletion of circulating autoantibodies using immunoadsorption (IA) is an established therapeutic approach in patients with pemphigus vulgaris, IA has only sporadically been used in other autoimmune bullous disorders. Although bullous pemphigoid (BP) usually responds well to topical and systemic corticosteroids, rapid depletion of serum autoantibodies may be an effective adjuvant treatment option in patients with severe and/or refractory disease. Patients and methods: Case series of 20 patients (13 women, 7 men; mean age 78.6 years; range 56–94 years) with severe or refractory BP. In addition to oral prednisolone (0.25–0.5 mg/kg/day), dapsone (1.0–1.5 mg/kg/day), and clobetasol propionate 0.05 % ointment (lesional application, twice daily), treatment consisted of protein A IA (three sessions on consecutive days). The mean follow-up period was 33.6 months (1–84 months). Results: The majority of patients showed a rapid and sustained response. One month after treatment, eight patients (42 %; 19 patients were included in the follow-up) were in complete remission; at the last follow-up visit (after 1 to 84 months), that number was 13 (68 %). Not only was there an initial drop in anti-BP180 autoantibodies (by 92 %), the effect also continued after one and three months, with mean autoantibody levels at 26 % and 13 % of baseline, respectively (p < 0.001). Both previously treated and treatment-naive patients showed a significant reduction in anti-BP180NC16A antibody levels throughout the observation period. Adverse events occurred in 13 of the 20 patients (65 %), Three were severe of which two were likely or probably related to IA. Conclusion: Immunoadsorption is an effective adjuvant treatment option for (the usually elderly) patients with severe and/or refractory BP.