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Review
. 2024 Sep;33(9):881-886.
doi: 10.1080/13543784.2024.2377323. Epub 2024 Jul 10.

Emerging treatment landscape for Guillain-Barré Syndrome (GBS): what's new?

Alina Sprenger-Svačina  1   2 , Martin K R Svačina  1   2 , Tong Gao  1 , Gang Zhang  1 , Kazim A Sheikh  1
Affiliations
Review

Emerging treatment landscape for Guillain-Barré Syndrome (GBS): what's new?

Alina Sprenger-Svačina et al. Expert Opin Investig Drugs. 2024 Sep.

Abstract

Introduction: Guillain-Barré syndrome (GBS) is a monophasic immune neuropathic disorder characterized by acute paralysis. A significant portion of patients are left with residual deficits, which presents a considerable global healthcare challenge. The precise mechanisms underlying GBS pathogenesis are not fully elucidated. Recent studies have focused on postinfectious molecular mimicry and identified involvement of IgG autoantibodies and innate immune effectors in GBS. Intravenous immunoglobulins (IVIg) and plasma exchange (PE) are two established evidence-based immunomodulatory treatments for GBS, but a significant proportion of GBS patients fails to respond adequately to either therapy. This emphasizes an urgent need for novel and more potent treatments.

Areas covered: We discuss novel immunomodulatory therapies presently at different phases of preclinical and clinical investigation. Some drugs in development target pathophysiologic mechanisms such as IgG autoantibody catabolism and complement activation, which are relevant to GBS.

Expert opinion: There is an unmet need for more effective immune therapies for GBS. New immunomodulatory therapies under development may provide more potent options for GBS patients who do not respond to IVIg or PE. Future directions may include incorporating neuroprotective interventions based on evolving understanding of mechanisms underlying nerve injury and axonal degeneration.

Keywords: Fc-gamma receptors; GBS; IVIg; IgG autoantibodies; Neonatal Fc receptor (FcRn); complement; immunomodulatory treatments; neuroprotective strategy.

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Conflict of interest statement

Declaration of Interests

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Figures

Figure 1.
Figure 1.
Pathophysiology and therapeutic targets in GBS. Both cellular and humoral immune mechanisms are involved in GBS. AIDP is characterized by lymphocytic infiltration and complement activation and macrophage-mediated demyelination. Autoantibody binding to Schwann cell/myelin antigens is implicated in AIDP due to the efficacy of plasma exchange (PE) and intravenous immunoglobulins (IVIg), but target antigens are largely unknown. The axonal variants, AMAN/AMSAN, are characterized by anti-ganglioside autoantibodies and complement deposition at nodal and internodal axolemma, resulting in subsequent macrophage-induced nodal lengthening and disruption and eventually axonal degeneration. Besides the established immunotherapies (IVIg and PE), there are other immune therapeutic approaches, with more specific mechanisms of action, which are in clinical trials in GBS (ANX005, eculizumab, imlifidase) or under clinical or preclinical stages of investigation (e.g. efgartigimod, hypersialylated IVIg (hsIVIg), recombinant Fc multimers (rFc)).
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