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

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.

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.

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).

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