Practice patterns in the management of pemphigus – An international survey study

Introduction: Anti-CD20 therapy (rituximab) has emerged as an effective and disease modifying treatment for pemphigus, especially in early disease. However, significant variability exists around the clinical application of this therapy. Methods: In this survey-based study among bullous disease experts, we investigated practice patterns surrounding the use of rituximab in the management of pemphigus. Anonymized surveys were sent to physician members of the internation Pemphigus and Pemphigoid Foundation. Topics of inquiry included the use of rituximab first line in newly diagnosed pemphigus, rituximab dosing regimen, use of concomitant steroid sparing agents, criteria for retreatment, and the use of antimicrobial prophylaxis. Results: Among specialists who responded to the survey, there was a strong consensus in the utilization of rheumatoid arthritis rituximab dosing regimen. There was variability in the thresholds for re-treatment with rituximab for patients in clinical remission and in the use prophylaxis for opportunistic infections. Additionally, there was a high level of dispersion in responses related to the use of concomitant immunosuppressive medications as well as the clinical and serologic markers used to monitor patients after treatment. Conclusion: This study provides valuable insight into real-world practice patterns in the treatment of pemphigus. While rituximab is being increasingly utilized as first line therapy in the management of pemphigus, there is a high degree of variability in its application, particularly with regards to retreatment parameters following clinical remission as well as in the use of antimicrobial prophylaxis. Further clinical data will be needed to establish best practices and consensus-based guidelines for the use of CD20 depleting therapy in pemphigus.