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

Abstract

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.

Keywords: autoimmune bullous diseases; childhood pemphigus vulgaris; juvenile pemphigus vulgaris; rituximab.