Effectiveness of Intravenous Immunoglobulin (IVIG) Therapy in Treatment of CIDP

Researchers believe that intravenous immunoglobulin (IVIG) is longer lasting & provides patients with CIDP a safer, more effective alternative to standard therapies for the disease. IVIG is a drug that has been used successfully to treat other immune-related diseases of the nervous system.

IVIG and plasmapheresis seem to be equally effective. IVIG is generally preferred to plasmapheresis because it is safer, more accessible and less invasive . Long term treatment with steroids can have serious side-effects. IVIG is effective in 70% to 90% of cases; however, most patients with initial improvement need long-term periodic doses of IVIG to maintain clinical stability[1].

At least five small randomized controlled studies have demonstrated the benefit of IVIG in the majority of patients with CIDP[2-7].  The ICE study is the largest and most recent trial of IVIG to treat CIDP[7]. The study not only confirmed the short-term efficacy of IVIG, but also demonstrated that a maintenance therapy can sustain improvement, increase quality of life over 12 months, and prevent further axonal degeneration[7-10]. The ICE study advocates IVIG as a first-line therapy, and has led to FDA approval of one brand of IVIG. The study has also shown that repeated therapy is usually needed to improve symptoms and then to maintain that improvement. Typical doses recommended repeat every 3 to 6 weeks. Interestingly large part of the IVIG treatment responsive patients were able to wean off of therapy after 24 weeks without showing a relapse before the study period ended. So, the amount of medication and the frequency of the need for repeated doses can vary widely from patient to patient and is usually determined by the patients response to the drug and their physicians experience with other similar patients in the past.

What is the IVIG dose for treatment of CIDP?

IVIG is usually given initially at a dose of 0.4 g/kg per day for 5 days. The response is assessed after 1 to 2 months. In patients who continue to worsen or develop periodic relapses, IVIG pulses should be given at 1.0 g/kg or less as a single infusion in monthly or bimonthly intervals[11]. A weekly dosing schedule may also be useful for maintenance therapy in select cases[12].

How is IVIG treatment given?

If you have been diagnosed with CIDP and your physician recommends IVIG therapy, you will receive IVIG therapy on regualar basis. The infusion is usually given intravenously, which means through a needle directly into a vein at a doctor's office, hospital, or infusion center. You may also be able to arrange to have your infusion at home.

What are adverse reactions of IVIG treatment?

Tolerability of IVIG is usually very good and adverse reactions are usually minor. The most common side effects are headache, nausea, chills, flushing, myalgia, hypotension, hypertension, chest discomfort, and fatigue. Infrequent adverse reactions include thromboembolic events, skin reactions, aseptic meningitis, renal tubular necrosis, and severe anaphylactic reaction.

References

1. Van Doorn PA. Treatment of patients with chronic inflammatory demyelinating polyneuropathy. Rev Neurol (Paris). 1996;152:383-386.
2.Mendell, J. R. et al. Randomized controlled trial of IVIg in untreated chronic inflammatory demyelinating polyradiculoneuropathy. Neurology 56, 445-449 (2001).
3.Hahn, A. F. Treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulin. Neurology 51 (6 Suppl. 5), S16-S21 (1998).
4.Vermeulen M. et al. Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating polyneuropathy: a double blind, placebo controlled study. J. Neurol. Neurosurg. Psychiatry 56, 36-39 (1993).
5.Dyck, P. J. et al. A plasma exchange versus immune globulin infusion trial in chronic inflamatory demyelinating polyradiculoneuropathy. Ann. Neurol. 36, 838-845 (1994).
6.van Doorn, P A., Brand, A., Strengers, P F., Meulstee, J. & Vermeulen, M. High-dose intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy: a double-blind, placebo-controlled, crossover study. Neurology 40, 209-212 (1990).
7.Hughes, R. A. C. et al. Intravenous immune globulin (10% caprylate-chromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE study): a randomised placebo-controlled trial. Lancet Neurol. 7, 136-144 (2008).
8.Merkies, I. S. et al. Health-related quality-of-life improvements in CIDP with immune globulin IV 10%: the ICE Study. Neurology 72, 1337-1344 (2009).
9.Bril, V. et al. Electrophysiologic correlations with clinical outcomes in CIDP. Muscle Nerve 42, 492-497 (2010).
10.Latov, N. et al. Timing and course of clinical response to intravenous immunoglobulin in chronic inflammatory demyelinating polyradiculoneuropathy. Arch. Neurol. 67, 802-807 (2010).
11. Hahn AF, Bolton CF, Zochodne D, Feasby TE. Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy: a double-blind, placebo-controlled, cross-over study. Brain. 1996;119(pt 4):1067-1077
12. Dyck PJ, Litchy WJ, Kratz KM, et al. A plasma exchange versus immune globulin infusion trial in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 1994;36:838-845.

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