The immunotherapy of Guillain-Barré syndrome

ABSTRACT

Introduction: Guillain-Barré syndrome is the most common cause of acute flaccid paralysis worldwide. Microorganisms such as Campylobacter jejuni, Cytomegalovirus, Epstein-Barr virus, Mycoplasma pneumoniae, Haemophilus influenzae and Zika virus have been linked to the disease. The most common clinical variants are acute inflammatory demyelinating polyneuropathy and acute motor axonal neuropathy. Plasma exchange and intravenous immunoglobulins are the standard therapy for the disease.

Areas covered: research to elucidate the pathophysiology of Guillain-Barré syndrome has led to the development of drugs directed towards new potential therapeutic targets. This review offers a comprehensive view of the current treatment based upon the physiopathology.

Expert opinion: patients with Guillain-Barré syndrome need a multidisciplinary approach, limitation to walk unaided and disability score are indicators for treatment as well as the presence of autonomic dysfunction and pain. Admission to intensive care units should be considered for those patients presenting with respiratory failure, bulbar involvement and progression of the disease. Research aimed to deciphering the pathophysiology of the disease, discovering new biomarkers and establishing algorithms of prediction of both the disease and its outcomes is warranted.

KEYWORDS:

• Polyneuropathy
• Guillain-Barré syndrome
• immunotherapy
• complement
• biological therapy
• intravenous immunoglobulins
• plasma exchange

Article highlights

• IVIg and PE have similar efficacy and safety profile as treatment for GBS.

• Not all patients respond equally to an initial standard dose; a second cycle of IVIg has shown positive results.

• The use of corticosteroids has shown no benefit when added to IVIg or PE, or when used alone.

• Complement-targeted therapy seems to be a promising area of investigation. Eculizumab appears to be safe and well tolerated. New drugs are under study.

• Patients with limitation to walk unaided, dysautonomia, pain and Hughes score equal or greater than 3 should be treated promptly

This box summarizes key points contained in the article.

Acknowledgments

Authors would like to express their gratitude to all the colleagues at the Center for Autoimmune Diseases Research (CREA) for fruitful discussions and collaboration. Funding

This paper is supported by Universidad del Rosario (ABN011), and Colciencias (747-2016), Bogotá, Colombia

Declaration of interest

The authors have no relevant affiliation 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, patents received or pending, or royalties.

Supplementary material

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Additional information

Funding

This work was supported by the Departamento Administrativo de Ciencia, Tecnología e Innovación, Bogota, Colombia[747-2016];