Rituximab and intravenous immunoglobulin as alternatives to long-term systemic corticosteroids in the treatment of pemphigus: a single center case series of 63 patients

Abstract

Rituximab and intravenous immunoglobulin [IVIg] have recently emerged as effective treatments for pemphigus refractory to corticosteroids [CS]. This case series sought to compare the clinical, serologic,and adverse effects of CS, IVIg, and rituximab in patients with pemphigus. A retrospective review of 63 patients with pemphigus vulgaris (PV), pemphigus foliaceus (PF), or paraneoplastic pemphigus (PNP)was performed. Clinical remission (CR), serologic remission (SR), and adverse effects were evaluated. Three study groups were compared: patients treated with systemic CS, refractory patients treated withIVIg, and refractory patients treated with rituximab. The overall number of adverse effects was not significantly different between the groups but those observed in patients treated with systemic CS weremore severe. CR was less likely in the patients treated with systemic CS than in patients treated with IVIg or rituximab, P-value = 0.000467. SR was more likely in patients treated with systemic CS or rituximab thanin patients treated with IVIg, P-value = 0.002118. These results suggest that the clinical efficacy of IVIg is not correlated with an expected concomitant SR. Frequently reserved for refractory pemphigus,IVIg and rituximab are significantly more likely to produce clinical remission than systemic CS therapy, suggesting their utility as first-line treatments.