Abstract
Intravenous immunoglobulin (IVIg) is an effective tool for the treatment of diseases with immune pathogenesis. This article reviews the current knowledge of the benefits of treating with IVIg patients with myasthenia gravis (MG), Lambert Eaton myasthenic syndrome (LEMS), dermatomyositis (DM), polymyositis (PM) and inclusion body myositis (IBM).
Myasthenia gravis
Treatment of MG with IVIg was reported to be beneficial in a number of case series and two randomised controlled trials, in which efficacy was measured by clinical improvement using myasthenic muscle score and decrease in anti-acetylcholine receptor antibodies (AchRAb). According to the results, IVIg could be recommended for crisis and severe exacerbation. In many other clinical conditions, such as response to treatment of mild or moderate exacerbation, changes in steroid dosage and before thymectomy, IVIg has also been reported to be helpful, but no controlled trials to confirm its efficacy have been performed.
Lambert-Eaton myasthenic syndrome
A placebo-controlled crossover study reported a significant clinical improvement in the amplitude of the resting CMAP following IVIg treatment. Further experience from case reports also indicates that IVIg is useful in patients with LEMS, both as a short- and long-term treatment, especially when immunosuppressive drugs are not fully effective.
Inflammatory myopathies/Dermatomyositis:
In a double-blind placebo-controlled crossover trial in patients with DM resistant to other treatments, IVIg was shown to produce a significant increase of muscle strength as well as a marked improvement in immunopathological parameters in repeated muscle biopsies (before and after IVIg). Thus, IVIg is an important therapy in patients with DM resistant to other conventional therapies.
Polymyositis:
No randomised trials have been undertaken. One study showed clinical improvement and a reduction in the need of prednisone in patients with chronic refractory PM.
Inclusion body myositis:
Three controlled trials showed some muscle strength improvement, although the changes did not reach statistical significance. However improvement in swallowing was repeatedly observed, suggesting that some patients with severe dysphagia may derive a modest benefit from IVIg therapy.
Conclusion
Controlled trials indicate that in MG, LEMS, and DM, IVIg at a total dose of 2 g/kg is a highly useful therapy. Uncontrolled trials and case reports indicate benefit in many different clinical situations, but further clinical investigation is required.
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
References
Achiron A, Barak Y, Miron S, Sarova-Pinhas I (2000) Immunoglobulin treatment in refractory Myasthenia gravis. Muscle Nerve 23:551–555
Al-Mayouf SM, Laxer RM, Schneider R, Silverman ED, Feldman BM (2000) Intravenous immunoglobulin therapy for juvenile dermatomyositis: efficacy and safety. J Rheumatol 27:2498–2503
Arsura EL, Bick A, Brunner NG, Grob D (1988) Effects of repeated doses of intravenous immunoglobulin in myasthenia gravis. Am J Med Sci 295:438–443
Bain PG, Motomura M, Newsom-Davis J, Misbah SA, Chapel HM, Lee ML, Vincent A, Lang B (1996) Effects of intravenous immunoglobulin on muscle weakness and calcium-channel autoantibodies in the Lambert-Eaton myasthenic syndrome. Neurology 47:678–683
Bird SJ (1992) Clinical and electrophysiologic improvement in Lambert-Eaton syndrome with intravenous immunoglobulin therapy. Neurology 42:1422–1423
Cherin P, Herson S, Wechsler B, Piette JC, Bletry O, Coutellier A, Ziza JM, Godeau P (1991) Efficacy of intravenous gammaglobulin therapy in chronic refractory polymyositis and dermatomyositis: an open study with 20 adult patients. Am J Med 91:162–168
Cherin P, Pelletier S, Teixeira A, Laforet P, Genereau T, Simon A, Maisonobe T, Eymard B, Herson S (2002) Results and long-term follow-up of intravenous immunoglobulin infusions in chronic, refractory polymyositis: an open study with thirty-five adult patients. Arthritis Rheum 46:467–474
Cosi V, Lombardi M, Piccolo G, Erbetta A (1991) Treatment of myasthenia gravis with high-dose intravenous immunoglobulin. Acta Neurol Scand 84:81–84
Dalakas MC (1991) Polymyositis, dermatomyositis and inclusion-body myositis. N Engl J Med 325:1487–1498
Dalakas MC (1998) Controlled studies with high-dose intravenous immunoglobulin in the treatment of dermatomyositis, inclusion body myositis, and polymyositis. Neurology 51:S37–S45
Dalakas MC (1999) Intravenous immunoglobulin in the treatment of autoimmune neuromuscular diseases: present status and practical therapeutic guidelines. Muscle Nerve 22:1479–1497
Dalakas MC (2003) Therapeutic approaches in patients with inflammatory myopathies. Semin Neurol 23:199–206
Dalakas MC (2004) The molecular pathophysiology in inflammatory myopathies. Rev Med Interne 25(Suppl 1):S14–S16
Dalakas MC, Hohlfeld R (2003) Polymyositis and dermatomyositis. Lancet 362:971–982
Dalakas MC, Illa I, Dambrosia JM, Soueidan SA, Stein DP, Otero C, Dinsmore ST, McCrosky S (1993) A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis. N Engl J Med 329:1993–2000
Dalakas MC, Koffman B, Fujii M, Spector S, Sivakumar K, Cupler E (2001) A controlled study of intravenous immunoglobulin combined with prednisone in the treatment of IBM. Neurology 56:323–327
Dalakas MC, Sonies B, Dambrosia J, Sekul E, Cupler E, Sivakumar K (1997) Treatment of inclusion-body myositis with IVIg: a double-blind, placebo-controlled study. Neurology 48:712–716
Danieli MG, Malcangi G, Palmieri C, Logullo F, Salvi A, Piani M, Danieli G (2002) Cyclosporin A and intravenous immunoglobulin treatment in polymyositis/dermatomyositis. Ann Rheum Dis 61:37–41
Drachman DB (1994) Myasthenia gravis. N Engl J Med 330:1797–1810
Drachman DB, Jones RJ, Brodsky RA (2003) Treatment of refractory myasthenia: “rebooting” with high-dose cyclophosphamide. Ann Neurol 53:29–34
Fateh-Moghadam A, Wick M, Besinger U, Geursen RG (1984) High-dose intravenous gammaglobulin for myasthenia gravis. Lancet 1:848–849
Gajdos P, Chevret S, Clair B, Tranchant C, Chastang C (1997) Clinical trial of plasma exchange and high-dose intravenous immunoglobulin in myasthenia gravis. Myasthenia Gravis Clinical Study Group. Ann Neurol 41:789–796
Gajdos P, Chevret S, Toyka K (2003) Intravenous immunoglobulin for myasthenia gravis. Cochrane Database Syst Rev:CD002277
Gronseth GS, Barohn RJ (2000) Practice parameter: thymectomy for autoimmune myasthenia gravis (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 55:7–15
Hoch W, McConville J, Helms S, Newsom-Davis J, Melms A, Vincent A (2001) Auto-antibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies. Nat Med 7:365–368
Hughes BW, Moro De Casillas ML, Kaminski HJ (2004) Pathophysiology of myasthenia gravis. Semin Neurol 24:21–30
Lang B, Newsom-Davis J, Wray D, Vincent A, Murray N (1981) Autoimmune aetiology for myasthenic (Eaton-Lambert) syndrome. Lancet 2:224–226
Mareska M, Gutmann L (2004) Lambert-Eaton myasthenic syndrome. Semin Neurol 24:149–153
Mastaglia FL, Phillips BA, Zilko PJ (1998) Immunoglobulin therapy in inflammatory myopathies. J Neurol Neurosurg Psychiatry 65:107–110
McEvoy KM, Windebank AJ, Daube JR, Low PA (1989) 3.4-Diaminopyridine in the treatment of Lambert-Eaton myasthenic syndrome. N Engl J Med 321:1567–1571
Meriggioli MN, Ciafaloni E, Al-Hayk KA, Rowin J, Tucker-Lipscomb B, Massey JM, Sanders DB (2003) Mycophenolate mofetil for myasthenia gravis: an analysis of efficacy, safety, and tolerability. Neurology 61:1438–1440
Muchnik S, Losavio AS, Vidal A, Cura L, Mazia C (1997) Long-term follow-up of Lambert-Eaton syndrome treated with intravenous immunoglobulin. Muscle Nerve 20:674–678
Newsom-Davis J (2004) Lambert-Eaton myasthenic syndrome. Rev Neurol (Paris) 160:177–180
Palace J, Newsom-Davis J, Lecky B (1998) A randomized double-blind trial of prednisolone alone or with azathioprine in myasthenia gravis. Myasthenia Gravis Study Group. Neurology 50:1778–1783
Perez Nellar J, Dominguez AM, Llorens-Figueroa JA, Ferra-Betancourt A, Pardo A, Quiala M, Gali Z (2001) A comparative study of intravenous immunoglobulin and plasmapheresis preoperatively in myasthenia. Rev Neurol 33:413–416
Qureshi AI, Choudhry MA, Akbar MS, Mohammad Y, Chua HC, Yahia AM, Ulatowski JA, Krendel DA, Leshner RT (1999) Plasma exchange versus intravenous immunoglobulin treatment in myasthenic crisis. Neurology 52:629–632
Richman DP, Agius MA (2003) Treatment of autoimmune myasthenia gravis. Neurology 61:1652–1661
Ronager J, Ravnborg M, Hermansen I, Vorstrup S (2001) Immunoglobulin treatment versus plasma exchange in patients with chronic moderate to severe myasthenia gravis. Artif Organs 25:967–973
Sanders DB, Massey JM, Sanders LL, Edwards LJ (2000) A randomized trial of 3,4-diaminopyridine in Lambert-Eaton myasthenic syndrome. Neurology 54:603–607
Takano H, Tanaka M, Koike R, Nagai H, Arakawa M, Tsuji S (1994) Effect of intravenous immunoglobulin in Lambert-Eaton myasthenic syndrome with small-cell lung cancer: correlation with the titer of anti-voltage-gated calcium channel antibody. Muscle Nerve 17:1073–1075
Vincent A, Bowen J, Newsom-Davis J, McConville J (2003) Seronegative generalised myasthenia gravis: clinical features, antibodies, and their targets. Lancet Neurol 2:99–106
Vincent A, McConville J, Farrugia ME, Bowen J, Plested P, Tang T, Evoli A, Matthews I, Sims G, Dalton P, Jacobson L, Polizzi A, Blaes F, Lang B, Beeson D, Willcox N, Newsom-Davis J, Hoch W (2003) Antibodies in myasthenia gravis and related disorders. Ann N Y Acad Sci 998:324–335
Walter MC, Lochmuller H, Toepfer M, Schlotter B, Reilich P, Schroder M, Muller-Felber W, Pongratz D (2000) High-dose immunoglobulin therapy in sporadic inclusion body myositis: a double-blind, placebo-controlled study. J Neurol 247:22–28
Wolfe GI, Barohn RJ, Foster BM, Jackson CE, Kissel JT, Day JW, Thornton CA, Nations SP, Bryan WW, Amato AA, Freimer ML, Parry GJ (2002) Randomized, controlled trial of intravenous immunoglobulin in myasthenia gravis. Muscle Nerve 26:549–552
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Illa, I. IVIg in myasthenia gravis, Lambert Eaton myasthenic syndrome and inflammatory myopathies: current status. J Neurol 252 (Suppl 1), i14–i18 (2005). https://doi.org/10.1007/s00415-005-1104-5
Issue Date:
DOI: https://doi.org/10.1007/s00415-005-1104-5