Immunotherapy in Pediatric Guillain-Barré Syndrome: Intravenous Immunoglobulin, Plasmapheresis, Both or Something Else?

Abstract

Guillain-Barré syndrome (GBS) is an autoimmune disease of the peripheral nervous system that is clinically characterized by rapidly progressing and symmetric muscle weakness, loss (or decrease) of deep tendon reflexes and respiratory distress, leading in some cases to the need for artificial ventilation. This is a clinical diagnosis that can be supported by the integration of several results, coming from cerebrospinal fluid examination, neuroimaging, nerve conduction studies and serum analysis. Plasma exchange and intravenous immunoglobulin (IgIV) are both treatments that have proven to be effective in improving motor recovery and reducing the need for mechanic ventilation. While their efficacy is comparable, IgIV is the first line treatment and plasma exchange is not used as the primary approach due to the need for specialized personnel and specific equipment. However, some long-term results with intravenous monotherapy are not always the most favorable and, therefore, studies combining the two interventions have begun to be developed. One of them, defining the zipper method, proved that intercalating both techniques may improve the outcome when compared to each therapy on its own. Nevertheless, approaches with monoclonal antibodies, such as eculizumab, seem interesting, but only in adults, so far. In this article, we aim to review existing evidence on the immune therapeutic approach to GBS in children.