RESUMO
BACKGROUND: Mucous membrane pemphigoid (MMP) is an autoimmune disease that can lead to fibrosis of mucous membranes and functional impairment. Biologic agents should be explored as alternative treatment options to improve outcomes. OBJECTIVE: To conduct a systematic review of biologic treatment outcomes in patients with MMP. METHODS: A MEDLINE and Embase search was conducted on July 23, 2020, to include 63 studies using Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines. RESULTS: Use of intravenous immunoglobulin (n = 154), rituximab (n = 112), tumor necrosis factor α inhibitors (n = 7), and combination treatments (n = 58) were reported in 331 patients with MMP. Intravenous immunoglobulin led to complete resolution in 61.7% (n = 95/154) of patients within 26.0 months, with a recurrence rate of 22.7% (n = 35/154) and headache as the most common adverse effect (8.4%, n = 13/154). Rituximab led to complete resolution in 70.5% (n = 79/112) of patients within 8.7 months, with a recurrence rate of 35.7% (n = 40/112). The most commonly reported adverse effects were urinary tract infections (4.5%, n = 5/112), leukocytopenia (2.7%, n = 3/112), and death due to severe infections (1.8%, n = 2/112). Tumor necrosis factor α inhibitors led to complete resolution in 71.4% (n = 5/7) of patients within 3.9 months of treatment without reported adverse events. CONCLUSIONS: Randomized clinical trials with long-term follow-up are required to conclude the promising safety and efficacy of biologic agents in patients with MMP.