Rituximab in childhood and juvenile autoimmune bullous diseases as first-line and second-line treatment: a case series of 13 patients

Background Little data are available concerning the outcome of rituximab (RTX) therapy in pediatric patients with autoimmune bullous diseases (AIBDs). Objective We sought to evaluate safety and efficacy of RTX administration in pediatric patients with AIBDs and to assess first-line RTX therapy in pemphigus patients. Methods AIBD patients consisting of 12 pemphigus patients and a patient with bullous pemphigoid who received RTX before the age of 18 were enrolled. Detailed information regarding patients' outcome after the first RTX cycle was assessed. Results The mean age of the patients at RTX infusion was 15 +/- 2 years. Six patients in the pemphigus group received RTX as first-line therapy. In pemphigus patients: complete remission (on minimal therapy) was achieved by seven patients, partial remission (on minimal therapy) and complete remission (off therapy) were achieved by three patients and one, respectively. Relapse occurred in nine patients, which were mostly mild. Likewise, the BP patient received RTX with a good clinical response. The observed adverse events were mostly mild infusion reactions and a case of sepsis. Conclusion Rituximab is safe and effective in childhood/juvenile patients with AIBDs. Furthermore, RTX can be used as first-line treatment in pediatric patients with pemphigus.