Rituximab therapy in pediatric pemphigus patients: A retrospective analysis of five Turkish patients and review of the literature.

BACKGROUND/OBJECTIVES: There is inadequate knowledge regarding rituximab (RTX) administration in autoimmune bullous diseases (AIBDs), disease prevalence, clinical characteristics, and treatment outcomes within pediatric populations due to the rarity of AIBDs affecting the pediatric age group. The aim of this retrospective analysis was to evaluate the effectiveness, safety of RTX, and treatment outcomes in Turkish pediatric patients with pemphigus vulgaris (PV) and to review the literature. METHODS: Five patients under 18 years of age and diagnosed with PV received RTX treatment and were identified in four dermatology departments of Turkey. RESULTS: The mean age of the patients at the time of RTX therapy initiation was 15 years (range: 11-17 years), and the total duration of follow-up after RTX therapy was 42.6 months (range: 19-60 months). All patients showed a clinical response. At the last visit, complete remission off therapy was achieved in three patients. The remaining two patients achieved partial remission off therapy. No adverse events were observed. CONCLUSIONS: This retrospective case series of five pediatric patients showed that RTX treatment can be effective and safe for the treatment of recalcitrant PV in pediatric patients. With increasing evidence, RTX is a good treatment choice in adults and pediatric patients with pemphigus.

Successful use of rituximab in the treatment of childhood and juvenile pemphigus.

BACKGROUND: There is a lack of data on outcomes of management of pemphigus in children. OBJECTIVE: We sought to evaluate rituximab treatment in childhood and juvenile pemphigus. METHOD: All cases of pemphigus treated with rituximab in patients younger than 18 years were included. Clinical and epidemiologic data and details of rituximab administration were recorded. Response to treatment was assessed as control of disease activity, partial remission, complete remission, and relapse/flare. RESULTS: Ten patients aged 9 to 17 years received rituximab treatment. After therapy, they were followed up for a median period of 16 months (range 8-36 months). Complete remission without concomitant therapy was achieved in 7 patients by a mean of 21 weeks. One patient each achieved complete remission (on immunosuppressant therapy), control of disease activity, and partial remission (on immunosuppressant therapy) by 15, 8, and 14 weeks, respectively. Relapse/flare occurred in 6 patients by a mean period of 13 months. Two patients received a second cycle of rituximab infusions with good clinical response. Infusion reactions were the most common adverse event. There were no long-term complications. LIMITATION: Small sample size and retrospective study design are limitations. CONCLUSION: The current data suggest that rituximab is useful in treating childhood and juvenile pemphigus.