Efficacy of Rituximab for Pemphigus: A Systematic Review and Meta-analysis of Different Regimens

Abstract

This meta-analysis examined the efficacy of different dosing regimens containing rituximab (RTX) in treating pemphigus. The analysis included 578 patients with pemphigus from 30 studies. Seventy-six percent of patients achieved complete remission (CR) after 1 cycle of RTX. Mean time to remission was 5.8 months, with a remission duration of 14.5 months and a 40% relapse rate. Eighteen patients (3.3%) developed serious adverse effects. The pooled estimate showed no significant differences in CR and relapse rates between patients treated with high-dose (near or ≥ 2,000 mg/cycle) vs. low-dose (< 1,500 mg/cycle) RTX. In the fully adjusted analysis, high-dose RTX was associated with longer duration of CR compared with low-dose RTX. No superiority of lymphoma protocol over rheumatoid arthritis or high-dose RTX over low-dose RTX was shown in other outcomes. RTX treatment is efficacious and well-tolerated in treating pemphigus. High-dose RTX treatment may lead to longer duration of remission. However, the choice of optimal regimen depends on the overall condition of the individual patient.

Pemphigus

“…A multicentre, prospective, randomized trial of rituximab as a first-line therapy for pemphigus was recently published 161 , resulting in designation of rituximab as a US FDA Breakthrough Therapy. Previously, prospective, open-label trials of rituximab (with or without intravenous immunoglobulin) 92,162–164 and meta-analyses 165,166 have supported the remarkable efficacy of rituximab in pemphigus, with 59–100% of patients achieving complete clinical remission after treatment, a mean time to clinical remission of 3–6 months and a median remission duration of 15–19 months. Rituximab therapy early in the disease course might be associated with better clinical response 92,167 .…”

“…Rituximab therapy early in the disease course might be associated with better clinical response 92,167 . Relapse rates after rituximab therapy range between 40% and 81% 92,166 and generally increase with the length of the follow-up. During long-term follow-up, 35–45% of patients with pemphigus treated with rituximab remain in complete clinical disease remission when off systemic therapy 92,165,166 , with rates as high as 100% in a small study of rituximab and intravenous immunoglobulin as adjunctive treatments 164 .…”

“…Dosing regimens used to treat lymphoma and rheumatoid arthritis have shown comparable rates of complete clinical remission, with the lymphoma protocol showing slightly higher rates of complete remission, shorter time to disease control and longer duration of remission 166 . High-dose rituximab was associated with a longer duration of complete clinical remission than low-dose rituximab (17 and 9 months, respectively) 166 .…”

Penfigoide Bolhoso Tratado com Sucesso com Rituximab

“…Tratamentos com doses maiores de rituximab estiveram associados a períodos maiores de remissão da doença. 7 Outro estudo comparou o outcome de doente tratados com PDN ou com PDN mais rituximab e observou-se melhor controlo da doença, menor dose de PDN para controlo da doença e menor mortalidade no grupo tratado com terapêutica combinada. 8 O rituximab tem um bom perfil de segurança e os seus efeitos secundários mais frequentes são náuseas, vómitos, urticária e tosse, que normalmente são ligeiros e autolimitados.…”

Untitled

“…1 It is used now for some autoimmune diseases such as rheumatologic diseases, [2][3][4] Pemphigus, 5 and acute antibody-mediated rejection (AMR) in kidney transplant recipients. 6 A recently recognized delayed adverse event of rituximab is late-onset neutropenia (LON).…”