Literature Review Supports Efficacy of IVIG in Mucocutaneous Blistering Disease

Noting that intravenous immunoglobulin (IVIG)is increasingly used off-label in the treatment of autoimmune and chronic inflammatory disorders, these U.S. investigators searched the PubMed database to determine if there is evidence to support the efficacy of IVIG therapy specifically in autoimmune mucocutaneous blistering diseases (AMBDs).

Twenty-three English-language studies, published between May 1999 and April 2010, were identified that met the following criteria: 1) minimum of five patients, 2) diagnosis based on histology and immunopathology and 3) statistical analysis of data for comparison of efficacy provided. One randomized trial was found and all other studies were case series. Data on 260 patients treated with IVIG were analyzed; these included 191 patients with pemphigus and 69 patients with pemphigoid disorders.

Overall, 245 patients showed improvement with IVIG therapy, without a significant incidence of serious adverse effects. IVIG also demonstrated a corticosteroid-sparing effect. The reviewers concluded that “the best available evidence in the literature indicates that IVIG is efficacious and has a good safety profile in the treatment of AMBDs.”