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Published on: November 9, 2017
[Guillain-Barré syndrome]
1Service de réanimation médicale, hôpital Raymond-Poincaré, 92380 Garches. jean-claude.raphael@rpc.ap-hop-paris.fr
Insights
Guillain-Barré Syndrome (GBS) treatment guidelines recommend plasma exchange (PE) as a first-line therapy. Intravenous immunoglobulin (IVIg) is equally effective for severe GBS, with treatment choice depending on patient factors and availability.
Area of Science:
- Neurology
- Immunology
Context:
- Guillain-Barré Syndrome (GBS) is a rare autoimmune disorder affecting the peripheral nervous system.
- GBS has a significant mortality rate (5%) and can lead to severe disability.
Purpose:
- To clarify treatment indications and optimize management strategies for Guillain-Barré Syndrome.
- To provide evidence-based recommendations for plasma exchange (PE) and intravenous immunoglobulin (IVIg) therapy.
Summary:
- Plasma exchange (PE) is the recommended first-line treatment for GBS, with specific numbers of exchanges based on disease severity (mild, moderate, severe).
- High-dose IVIg is as effective as PE for intermediate and severe GBS.
- Treatment decisions between PE and IVIg should consider contraindications and local availability.
Impact:
- Optimized treatment protocols for GBS can improve patient outcomes and reduce long-term disability.
- Standardized treatment approaches enhance the effectiveness of specialist care and rehabilitation efforts.
Abstract:
The annual incidence of Guillain-Barré Syndrome is 1.5 per 100000 people. It has an associated mortality of about 5%, with ten percent of patients severely disabled one year after the first onset of neurological signs. To treat this condition, specialist teams, access to intensive care and rehabilitation are essential and should be provided in appropriate hospital units. Neither oral nor intravenous corticosteroids are beneficial. Plasma exchange (PE) is the first line treatment having been found to improve outcomes in several randomised, controlled clinical trials. The indications for treatment have now been clarified. Two PE are recommended in patients who are able to walk (mild) with two additional PE if they deteriorate. In patients unable to walk unaided (moderate), four plasma exchanges are sufficient, likewise in those who require mechanically ventilation (severe form). It is not useful to add further PE in more severe disease or if there is no response. High-dose of intravenous immunoglobulin (0.4 g/kg daily for 5 days) and PE are equally effective in intermediate and severe forms. The choice between the two treatments depends on their respective contra-indications and local availability. A trial is underway to assess the indications and optimal dose of IVIg and number of PE to be used in different severities of disease.
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