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<p>Autoimmune Blistering Diseases are characterized by autoantibody binding to skin components, causing blisters and erosions. Common subtypes include pemphigus vulgaris (PV), pemphigus foliaceus (PF), and bullous pemphigoid. Rituximab is the first-line treatment, particularly for pemphigus, but has rarely been linked to paradoxical exacerbations. We aim to investigate the characteristics of patients with pemphigus who experienced exacerbations following rituximab treatment. A systematic search was conducted using PubMed/Medline, Scopus, Web of Science, and Embase. Patients with unexplained disease exacerbations after rituximab treatment were included, while those with exacerbations due to treatment withdrawal and rapid steroid tapering were excluded. A total of 63 patients from 12 studies were analyzed, including 62 with PV and one with PF. Post-rituximab exacerbations are described as increased severity scores or the need for higher steroid doses. Across 76 rituximab cycles, 68 exacerbation episodes were reported, typically occurring within 5–15 days post-infusion. Management strategies included escalating prednisolone, intravenous immunoglobulin, and immunosuppressants. Among 25 patients with documented outcomes, 15 achieved complete remission, seven had partial remission, and one had persistent disease. Two patients died from sepsis. Post-rituximab exacerbations pose a significant challenge in pemphigus management, potentially driven by immune dysregulation and genetic factors. Identifying risk factors through further research and ensuring long-term follow-up is crucial for optimizing treatment outcomes.</p>

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Paradoxical reaction to rituximab in patients with pemphigus: a systematic review of 63 patients

  • 期刊论文

  • 发表日期：

    2026年1月7日

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  • Seyed Mohammad Vahabi,
  • Elnaz Pourgholi,
  • Mahshid Sadat Ansari,
  • Maryam Daneshpazhooh

摘要

Autoimmune Blistering Diseases are characterized by autoantibody binding to skin components, causing blisters and erosions. Common subtypes include pemphigus vulgaris (PV), pemphigus foliaceus (PF), and bullous pemphigoid. Rituximab is the first-line treatment, particularly for pemphigus, but has rarely been linked to paradoxical exacerbations. We aim to investigate the characteristics of patients with pemphigus who experienced exacerbations following rituximab treatment. A systematic search was conducted using PubMed/Medline, Scopus, Web of Science, and Embase. Patients with unexplained disease exacerbations after rituximab treatment were included, while those with exacerbations due to treatment withdrawal and rapid steroid tapering were excluded. A total of 63 patients from 12 studies were analyzed, including 62 with PV and one with PF. Post-rituximab exacerbations are described as increased severity scores or the need for higher steroid doses. Across 76 rituximab cycles, 68 exacerbation episodes were reported, typically occurring within 5–15 days post-infusion. Management strategies included escalating prednisolone, intravenous immunoglobulin, and immunosuppressants. Among 25 patients with documented outcomes, 15 achieved complete remission, seven had partial remission, and one had persistent disease. Two patients died from sepsis. Post-rituximab exacerbations pose a significant challenge in pemphigus management, potentially driven by immune dysregulation and genetic factors. Identifying risk factors through further research and ensuring long-term follow-up is crucial for optimizing treatment outcomes.