Therapeutic issues in Guillain–Barré syndrome

ABSTRACT

Introduction

Guillain–Barré syndrome or acute inflammatory polyradiculoneuropathy is an inflammatory disease of peripheral nerves, which usually leads to tetraparesis of acute onset. It can lead to major residual disability in some patients despite current treatment options that have shown a favorable impact on the natural course of the disease.

Areas covered

This review focuses on the disease-modifying treatments that have been approved or are currently investigated for the treatment of Guillain–Barré syndrome. The authors also give their expert perspectives.

Expert opinion

Current treatment options, albeit efficacious, are not always able to stop the disease course of Guillain–Barré syndrome. It is admitted that patients with a benign course should be carefully monitored but don’t necessarily require specific treatment. In all other cases, specialized care and use of plasma exchange or intravenous immunoglobulin is required as soon as possible. The sequential use of both treatments has not shown any particular benefit, and it has recently been demonstrated that two courses of intravenous immunoglobulin do not perform better than one. There is therefore an urgent need to develop new therapeutic strategies for this sometimes-devastating disease, with promising options targeting the complement or autoantibodies. It remains important to discover biomarkers of disease activity, to select patients for intensive treatment and to identify if different treatment options should be used in different variants of Guillain–Barré syndrome.

KEYWORDS:

• Guillain-Barré syndrome
• treatment
• Intravenous immunoglobulin
• plasma exchange
• complement

Article highlights

• Guillain–Barré syndrome (GBS) is a potentially devastating inflammatory disease of the peripheral nervous system.

• Advances in intensive care, combined with disease-modifying therapies, such as intravenous immunoglobulin and plasma exchange, have improved the prognosis and mortality risk of patients with GBS.

• Early diagnosis of GBS is essential, particularly by testing proximal nerve conduction.

• The overall functional prognosis of GBS remains poor in severe forms, justifying the need for new therapeutic approaches.

• Advances in understanding the pathophysiology of the different forms of GBS have led to the emergence of promising therapeutic approaches, such as complement inhibitors.

• Imlifidase, an enzyme from Streptococcus pyogenes, is being investigated as an add-on therapy and may be an excellent option due to its ability to rapidly cleave IgG from the serum of affected patients.

Declaration of interest

Laurent Magy has received travel expenses, speaking fees and honoraria for consulting from Baxter, CSL Behring, Grifols, LFB and is or has been an investigator in clinical studies for CSL Behring, Hansa Biopharma and LFB. Simon Frachet has also served as an investigator on clinical studies for CSL Behring and Hansa Biopharma. The authors have no other 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 apart from those disclosed. He is otherwise an investigator in clinical studies for CSL Behring and Hansa Biopharma.

Reviewer disclosures

One referee declares that they consult for bioscience and pharmaceutical companies, which are currently involved in research and development pertaining to Guillain-Barré syndrome. The names of the companies have been removed to preserve the anonymity of the referee, but the full details were provided to the editorial team for review. Peer reviewers on this manuscript have no other relevant financial relationships or otherwise to disclose.

Additional information

Funding

This manuscript was not funded.