Adjuvant therapy of severe and/or refractory bullous pemphigoid with immunoadsorption - A prospective monocenter pilot study.

BACKGROUND AND

OBJECTIVES

Bullous pemphigoid (BP) is the most common autoimmune blistering disease in the Western world. While remission is achieved in the majority of BP patients by long-term use of corticosteroids with or without immunomodulants/immunosuppressants, national and international guidelines recommend adjuvant immunoadsorption (IA) in refractory patients. Here we investigated the safety and efficacy of IA in severe and/or refractory BP patients.

PATIENTS AND

METHODS

10 BP patients (3 women, 7 men; mean age 69.7 years; range 52-81 years) were treated with IA on three consecutive days (LigaSorb®, Fresenius Medical Care) combined with oral prednisolone (0.5 mg/kg BW per day, tapered), dapsone (1.5 mg/kg BW per day), and lesional mometasone furoate ointment.

RESULTS

Within 2 and 6 months after IA, 50% and 90% of patients showed complete remission on therapy, respectively. In all patients, serum anti-BP180 IgG levels decreased significantly by an average of 89% and 73% immediately after the third IA and 4 weeks later, respectively. A total of 56 adverse events (AE) occurred during the 12-month follow-up. The majority of AE were of severity grade 2 (50%), 15 AE were classified as severe (grade 3-4).

CONCLUSIONS

IA can be considered as a relatively safe and effective adjuvant therapy for patients with severe or refractory BP.