Current issues of ACP Journal Club are published in Annals of Internal Medicine


Therapeutics

Review: Plasma exchange or intravenous immunoglobulin reduces disability in the Guillain-Barré syndrome

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ACP J Club. 2004 May-Jun;140:76. doi:10.7326/ACPJC-2004-140-3-076


Clinical Impact Ratings

Hospitalists: 5 stars

Critical Care: 6 stars

Neurology: 6 stars


Source Citation

Hughes RA, Wijdicks EF, Barohn R, et al. Practice parameter: Immunotherapy for Guillain-Barré syndrome: Report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2003;61:736-40. [PubMed ID: 14504313]


Abstract

Question

In patients with the Guillain-Barré syndrome (GBS) is immunotherapy consisting of plasma exchange, intravenous immunoglobulin (IgIV), or steroids effective for reducing GBS-related disability?

Data sources

Studies were identified by searching MEDLINE (1966 to March 2002), the Cochrane Library (March 2002), and reviewing bibliographies of relevant studies and personal reference lists of the members of the practice parameter group.

Study selection and assessment

Studies were selected if they were randomized controlled trials (RCTs) that evaluated immunotherapy including plasma exchange, immunoabsorption, IgIV, or steroids in patients with GBS.

Outcomes

The primary outcome measure used in most RCTs was a 0 to 6 disability scale (0 = normal, 1 = symptoms but able to run, 2 = unable to run, 3 = unable to walk unaided, 4 = bed-bound, 5 = needing ventilation, and 6 = dead).

Main results

Plasma exchange: At 4 weeks, more patients who received plasma exchange than supportive care improved by ≥ 1 disability grade (Table). Mean improvement in disability grade was greater in the plasma exchange group than in the supportive care group (4 RCTs, n = 585) (weighted mean difference [WMD] −0.89, 95% CI −1.14 to −0.63). The number of patients still on a ventilator 4 weeks after randomization was lower in the plasma exchange group than in the supportive care group (Table). At 1 year, more patients in the plasma exchange group than in the supportive care group had recovered full muscle strength (Table).

IgIV: Meta-analysis of 3 RCTs (n = 398) that compared IgIV with plasma exchange showed no difference between groups for improvement in GBS-related disability at 4 weeks (WMD 0.11, CI −0.14 to 0.37). Meta-analysis of 2 RCTs (n = 533) showed that fewer patients in the IgIV group than in the plasma exchange group discontinued the study (relative risk 0.11, CI 0.04 to 0.32).

Steroids: 6 RCTs compared any form of corticosteroid or adrenocorticotrophic hormone with no steroid or placebo. Meta-analysis of 3 RCTs (n = 296) showed that the groups did not differ for improvement in disability grade 4 weeks after randomization (WMD −0.06, CI −0.32 to 0.19).

Conclusion

In patients with the Guillain-Barré syndrome (GBS), immunotherapy consisting of plasma exchange or intravenous immunoglobulin reduces GBS-related disability.

Source of funding: No external funding.

For correspondence: Professor R.A. Hughes, Guy’s, King’s and St. Thomas’ School of Medicine, London, England, UK. E-mail richard.a.hughes@kcl.ac.uk.


Table. Plasma exchange (PLE) vs supportive care (SC) in the Guillain-Barré syndrome*

Outcomes Follow-up Number of RCTs (n) Weighted event rates RBI (95% CI) NNT (CI)
PLE SC
Improvement by ≥ 1 disability grade 4 wk 5 (623) 57% 35% 64% (37 to 96) 6 (4 to 7)
Recovery of full muscle strength 1 y 5 (404) 68% 55% 24% (7 to 45) 8 (5 to 25)
RRR (CI)
On ventilator 4 wk 5 (623) 14% 27% 47% (26 to 51) 8 (6 to 15)

*RCTs = randomized controlled trials. Other abbreviations defined in Glossary; RBI, RRR, NNT, and CI calculated from data in article using a fixed-effects model.


Commentary

The value of treating patients with GBS with plasma exchange or IgIV is beyond dispute. However, data from RCTs and Cochrane reviews included in the review by Hughes and colleagues do not help a clinician choose between the agents for individual patients. This choice should consider the probable adverse effects associated with each treatment. We usually favor plasma exchange for patients in whom hyperviscosity from IgIV is a potential problem. However, IgIV may be a better choice when central venous access for plasma exchange is problematic (e.g., in children or in patients who have received anticoagulants).

Some patients finish a course of treatment without improvement, or their disease continues to progress. It is tempting to apply another treatment in this circumstance, but clinical trials do not suggest any. No clear benefit was observed in a trial that evaluated plasma exchange followed by IgIV (1), although an insignificant difference between plasma exchange or IgIV alone and their combination sometimes leads to its use in desperation. The somewhat illogical sequence of IgIV followed by plasma exchange has not been tested.

The possibility of using corticosteroids in GBS refuses to die. A recent trial did not show any benefit from the combination of IgIV and methylprednisolone (2). No other treatments show clinical promise at this time. Addition of interferon β-1a to IgIV was not helpful (3).

Until new ideas to test are formulated, we must concentrate on early identification of GBS, rapid institution of plasma exchange or IgIV as appropriate for the patient, and prevention or management of complications while we hope that the patient responds to treatment.

Thomas P. Bleck, MD, FCCM
University of Virginia
Charlottesville, Virginia, USA


References

1. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Lancet. 1997;349:225-30. [PubMed ID: 9014908]

2. van Koningsveld R, Schmitz PI, Meche FG, et al. Effect of methylprednisolone when added to standard treatment with intravenous immunoglobulin for Guillain-Barre syndrome: randomised trial. Lancet. 2004;363:192-6. [PubMed ID: 14738791]

3. Pritchard J, Gray IA, Idrissova ZR, et al. A randomized controlled trial of recombinant interferon-beta 1a in Guillain-Barré syndrome. Neurology. 2003;61:1282-4. [PubMed ID: 14610140]