Clinical Rheumatology

, Volume 31, Issue 5, pp 801–806

Intravenous immunoglobulin therapy in adult patients with polymyositis/dermatomyositis: a systematic literature review

  • Dong Xue Wang
  • Xiao Ming Shu
  • Xiao Lan Tian
  • Fang Chen
  • Ning Zu
  • Li Ma
  • Guo Chun Wang
Original Article


The objectives of this study are to review and summarize published information on the use, effectiveness, and adverse effects of intravenous immunoglobulin (IVIG) in patients with polymyositis (PM) or dermatomyositis (DM) and to search MEDLINE and CNKI (Chinese) databases from 1985 to 2011 to retrieve clinical research articles concerning IVIG in adult patients with PM/DM. Of the 14 articles selected, two were randomized controlled trials, nine prospective open studies, and three retrospective studies with a total of 308 adult patients. IVIG has been used successfully in the treatment of PM/DM. The standard dose is 2 g/kg, given in two to five individual daily doses. The course of IVIG treatment is usually 3~6 months. IVIG therapy seemed rarely employed as first-line therapy in PM/DM. In a double-blind study conducted in patients with refractory DM, IVIG combined with corticosteroid significantly improved muscle strength and decreased serum creatine kinase level, compared with placebo. The beneficial effect of IVIG in refractory, flare-up, rapidly progressive, or severe PM/DM has been documented in many open-label trials. IVIG was shown to be effective in most of PM/DM patients with lung involvement and esophageal involvement. In some patients, IVIG can lower the corticosteroid dose required for maintenance, demonstrating the most effective steroid-sparing effect. Adverse effects were generally tolerable. IVIG is effective in the treatment of adult patients with PM/DM and appears to be relatively well tolerated and safe. IVIG may be a good choice especially in patients with refractory, flare-up, rapidly progressive, or severe PM/DM, and can be tried in patients with a contraindication for corticosteroid.


Dermatomyositis Intravenous immunoglobulin Polymyositis 


  1. 1.
    Roifman CM, Schaffer FM, Wachsmuth SE, Murphy G, Gelfand EW (1987) Reversal of chronic polymyositis following intravenous immune serum globulin therapy. JAMA 258:513–515PubMedCrossRefGoogle Scholar
  2. 2.
    Phillips B, Ball C, Sacker D, Badenoch D, Straus S, Haynes B, Dawes M (2009) Oxford Centre for Evidence-based Medicine Levels of Evidence (March). Accessed 11 Oct 2011
  3. 3.
    Suzuki Y, Hayakawa H, Miwa S, Shirai M, Fujii M, Gemma H, Suda T, Chida K (2009) Intravenous immunoglobulin therapy for refractory interstitial lung disease associated with polymyositis/dermatomyositis. Lung 187:201–206PubMedCrossRefGoogle Scholar
  4. 4.
    Marie I, Menard JF, Hatron PY, Hachulla E, Mouthon L, Tiev K, Ducrotte P, Cherin P (2010) Intravenous immunoglobulins for steroid-refractory esophageal involvement related to polymyositis and dermatomyositis: a series of 73 patients. Arthritis Care Res 62:1748–1755CrossRefGoogle Scholar
  5. 5.
    Tian J, Gao JS, Chen JW, Li F, Xie X, Du JF (2008) Efficacy and safety of the combined treatment with intravenous immunoglobulin and oral glucocorticoid in the elderly with dermatomyositis. Chin J Geriatr 27:588–590Google Scholar
  6. 6.
    Cherin P, Piette JC, Wechsler B, Bletry O, Ziza JM, Laraki R, Godeau P, Herson S (1994) Intravenous gamma globulin as first line therapy in polymyositis and dermatomyositis: an open study in 11 adult patients. J Rheumatol 21:1092–1097PubMedGoogle Scholar
  7. 7.
    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 329:1993–2000PubMedCrossRefGoogle Scholar
  8. 8.
    Danieli MG, Calcabrini L, Calabrese V, Marchetti A, Logullo F, Gabrielli A (2009) Intravenous immunoglobulin as add on treatment with mycophenolate mofetil in severe myositis. Autoimmun Rev 9:124–127PubMedCrossRefGoogle Scholar
  9. 9.
    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 46:467–474PubMedCrossRefGoogle Scholar
  10. 10.
    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 61:37–41PubMedCrossRefGoogle Scholar
  11. 11.
    Saito E, Koike T, Hashimoto H, Miyasaka N, Ikeda Y, Hara M, Yamada H, Yoshida T, Harigai M, Ichikawa Y (2008) Efficacy of high-dose intravenous immunoglobulin therapy in Japanese patients with steroid-resistant polymyositis and dermatomyositis. Mod Rheumatol 18:34–44PubMedCrossRefGoogle Scholar
  12. 12.
    Quick A, Tandan R (2011) Mechanisms of action of intravenous immunoglobulin in inflammatory muscle disease. Curr Rheumatol Rep 13:192–198PubMedCrossRefGoogle Scholar
  13. 13.
    Chung L, Genovese MC, Fiorentino DF (2007) A pilot trial of rituximab in the treatment of patients with dermatomyositis. Arch Dermatol 143:763–767PubMedCrossRefGoogle Scholar
  14. 14.
    Dalakas MC (2011) Immunotherapy of inflammatory myopathies: practical approach and future prospects. Curr Treat Options Neurol 13:311–323PubMedCrossRefGoogle Scholar
  15. 15.
    Mastaglia FL, Phillips BA, Zilko PJ (1998) Immunoglobulin therapy in inflammatory myopathies. J Neurol Neurosurg Psychiatry 65:107–110PubMedCrossRefGoogle Scholar
  16. 16.
    Wittstock M, Benecke R, Zettl UK (2003) Therapy with intravenous immunoglobulins: complications and side-effects. Eur Neurol 50:172–175PubMedCrossRefGoogle Scholar
  17. 17.
    Nozaki Y, Ikoma S, Funauchi M, Kinoshita K (2008) Respiratory muscle weakness with dermatomyositis during pregnancy: successful treatment with intravenous immunoglobulin therapy. J Rheumatol 35:2289PubMedCrossRefGoogle Scholar
  18. 18.
    Williams L, Chang PY, Park E, Gorson KC, Bayer-Zwirello L (2007) Successful treatment of dermatomyositis during pregnancy with intravenous immunoglobulin monotherapy. Obstet Gynecol 109:561–563PubMedCrossRefGoogle Scholar
  19. 19.
    Mosca M, Strigini F, Carmignani A, D'ascanio A, Genazzani AR, Bombardieri S (2005) Pregnant patient with dermatomyositis successfully treated with intravenous immunoglobulin therapy. Arthritis Rheum 53:119–121PubMedCrossRefGoogle Scholar
  20. 20.
    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 91:162–168PubMedCrossRefGoogle Scholar
  21. 21.
    Göttfried I, Seeber A, Anegg B, Rieger A, Stingl G, Volc-Platzer B (2000) High dose intravenous immunoglobulin (IVIG) in dermatomyositis: clinical responses and effect on sIL-2R levels. Eur J Dermatol 10:29–35PubMedGoogle Scholar
  22. 22.
    Kamali S, Cefle A, Sayarlioglu M, Gul A, Inanc M, Ocal L, Konice OAM (2005) Experience with monthly, high-dose, intravenous immunoglobulin therapy in patients with different connective tissue diseases. Rheumatol Int 25:211–214PubMedCrossRefGoogle Scholar
  23. 23.
    Helmers SB, Dastmalchi M, Alexanderson H, Nennesmo I, Esbjörnsson M, Lindvall B, Lundberg IE (2007) Limited effects of high-dose intravenous immunoglobulin (IVIG) treatment on molecular expression in muscle tissue of patients with inflammatory myopathies. Ann Rheum Dis 66:1276–1283CrossRefGoogle Scholar

Copyright information

© Clinical Rheumatology 2012

Authors and Affiliations

  • Dong Xue Wang
    • 1
  • Xiao Ming Shu
    • 1
  • Xiao Lan Tian
    • 1
  • Fang Chen
    • 1
  • Ning Zu
    • 1
  • Li Ma
    • 1
  • Guo Chun Wang
    • 1
  1. 1.Department of RheumatologyChina-Japan Friendship HospitalBeijingChina

Personalised recommendations