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Review
. 2010 May;30 Suppl 1(Suppl 1):S74-8.
doi: 10.1007/s10875-010-9407-4.

IVIG treatment and prognosis in Guillain-Barré syndrome

Pieter A van Doorn  1 , Krista Kuitwaard, Christa Walgaard, Rinske van Koningsveld, Liselotte Ruts, Bart C Jacobs
Affiliations
Review

IVIG treatment and prognosis in Guillain-Barré syndrome

Pieter A van Doorn et al. J Clin Immunol. 2010 May.

Abstract

Introduction: Guillain-Barré syndrome (GBS) is an acute, immune-mediated polyneuropathy that often leads to severe weakness. Intravenous immunoglobulin (IVIG) is a proven effective treatment for GBS (class 1 evidence). However, about 25% of patients need artificial ventilation and 20% are still unable to walk unaided after 6 months. Important clinical factors associated with poor outcome are age, presence of preceding diarrhea and the severity of disability in the early course of disease. These clinical factors were combined in a clinical prognostic scoring scale, the Erasmus GBS Outcome Scale (EGOS).

Materials and methods: GBS patients being unable to walk unaided are currently treated with a standard single IVIg dose (0.4 g/kg bodyweight for 5 days). A recent retrospective study in 174 GBS patients enrolled in one of our randomized controlled clinical trials showed that patients with a minor increase of serum IgG level after standard single IVIg dose recovered significantly slower. Additionally, fewer patients reached the ability to walk unaided at six months after correction for the known clinical prognostic factors (multivariate analysis; P < 0.022).

Discussion: It is yet unknown why some GBS patients only have a minor increase after standard IVIg treatment. By using the EGOS it is possible to select GBS patients with a poor prognosis. These patients potentially may benefit from a second IVIg dose.

Conclusion: A standard dose of IVIG is not sufficiently effective in many GBS patients. Whether these patients might benefit from a second IVIg dose needs further investigation.

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Conflict of interest statement

B.C. Jacobs received an honorarium from Baxter for consultancy.

P.A. van Doorn received an unrestricted departmental research grant from Baxter to conduct a randomized controlled trial comparing Gammagard S/D with Kiovig in CIDP, and to conduct an RCT comparing Gammagard S/D with or without methylprednisolone in GBS. He also has received personal and departmental payments for consultancy/RCT board participation from Talecris Biotherapeutics, ZLB Plasma Germany, Baxter, and Octapharma AG.

The other authors have no conflicts of interest to disclose.

Figures

Fig. 1
Fig. 1
Variations in course of disease in GBS. Indicated are the courses of disease in mildly and severely affected GBS patients. The effect of IVIG in GBS has only been investigated in randomized controlled trials in patients unable to walk unaided at nadir (severely affected patients) and not in mildly affected patients (able to walk unaided at nadir). Whether mildly affected GBS patients may also benefit from IVIG is yet unknown. GBS patients who initially improve or stabilize after IVIG and subsequently deteriorate again have a “treatment-related fluctuation” (GBS-TRF): a condition that usually responds to an additional IVIG dose. Some GBS patients have a severe course of disease and a slow recovery phase. The prognosis of GBS patients can be determined using the Erasmus GBS Outcome Scale. Whether a second IVIG dose is effective in patients with a poor prognosis is not known yet
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