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Intravenous immunoglobulin in patients with bullous pemphigoid insufficient response to corticosteroids: Nationwide post-marketing surveillance in Japan
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DOI：10.1016/j.jdermsci.2023.01.004.png)
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Abstract
En 中文
Background: In Japan, intravenous immunoglobulin (IVIG) has been approved for corticosteroid-un-responsive bullous pemphigoid (BP); however, its usage, efficacy, and safety in clinical settings remain unclear.Objective: To elucidate IVIG efficacy, we examined the improvement in disease severity based on the Bullous Pemphigoid Disease Area Index (BPDAI).Methods: In this 3-year (April 2016-March 2019), prospective, post-marketing, observational surveillance study, we enrolled 379 patients (51.3 % men; mean age, 74.5 years) with corticosteroid-unresponsive BP treated with IVIG from 143 institutions in Japan (720 treatment cycles). The percentage of patients who improved by at least one severity stage or whose symptoms completely resolved based on the BPDAI score was evaluated at 15, 30, and 60-90 days.Results: The improvement rates at 15, 30, and 60-90 days after initial treatment in the 328 IVIG-naive patients were 70.7 %, 83.5 %, and 84.3 %, respectively. The BPDAI score decreased rapidly and significantly by 15 days compared with that observed during pre-treatment. Further improvement was observed at 30 and 60-90 days. The corticosteroid dose and anti-BP180 antibody titers decreased significantly post-treatment (both, p < .001). Approximately 25 % of IVIG-naive patients underwent multiple treatment cycles. The improvement rate at 30 days after the final dose was 88 %, and the symptoms completely resolved in 44 % of patients. The incidence of adverse drug reactions per cycle was 8.34 %; the most common reaction was transient thrombocytopenia.Conclusion: Most patients showed improvement in severity and decrease in corticosteroid dose and anti-BP180 antibody levels post-treatment, indicating that IVIG is useful for corticosteroid-unresponsive BP treatment.(c) 2023 The Authors. Published by Elsevier B.V. on behalf of Japanese Society for Investigative Dermatology. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Keywords:
Corticosteroid-unresponsive bullous pemphigoid
Intravenous immunoglobulin
Bullous Pemphigoid Disease Area Index
Nationwide surveillance
Dipeptidyl peptidase-4 inhibitor
Thrombocytopenia
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