Skip to main content
PMC home page
Archives of Disease in Childhood logoLink to Archives of Disease in Childhood
. 1995 Jan;72(1):25–28. doi: 10.1136/adc.72.1.25

Intravenous immunoglobulin in juvenile dermatomyositis--four year review of nine cases.

A Sansome 1, V Dubowitz 1
PMCID: PMC1510958  PMID: 7717731

Abstract

Juvenile dermatomyositis is difficult to treat, compounded by complications of the disease itself as well as side effects of treatment. The mainstay of pharmacological management is corticosteroids, to which the disease is usually very responsive, but steroids have well established short and long term side effects. Refractory cases may be resistant to steroids or patients may become dependent on high doses, with relapse in clinical disease precipitated by reduction. Overtreatment with steroids and too rapid a reduction are common errors. Various second line agents have been used with success in complex or refractory cases, including cyclophosphamide, methotrexate, and cyclosporin. Intravenous immunoglobulin has been tried in the management of autoimmune diseases, either as an alternative to cytotoxic treatment or as a steroid sparing agent. The reported benefit in a number of childhood illnesses, including juvenile dermatomyositis, prompted this clinical trial in some of our more refractory cases. Over the past four years, nine children attending a dermatomyositis clinic have been treated with intravenous immunoglobulin. The two main indications were failure of conventional treatment and severe side effects from previous treatment, principally corticosteroids. All nine were still on treatment with various permutations of prednisolone, azathioprine, and cyclosporin. All nine showed clinical improvement at some point in their treatment with intravenous immunoglobulin. Of eight children on concurrent prednisolone, the dose could be reduced in six and kept the same in two.

Full text

PDF

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Ballow M., White W., Desbonnet C. Modulation of in vitro synthesis of immunoglobulin and the induction of suppressor activity by therapy with intravenous immune globulin. J Allergy Clin Immunol. 1989 Oct;84(4 Pt 2):595–602. doi: 10.1016/0091-6749(89)90196-6. [DOI] [PubMed] [Google Scholar]
  2. Barron K. S., Murphy D. J., Jr, Silverman E. D., Ruttenberg H. D., Wright G. B., Franklin W., Goldberg S. J., Higashino S. M., Cox D. G., Lee M. Treatment of Kawasaki syndrome: a comparison of two dosage regimens of intravenously administered immune globulin. J Pediatr. 1990 Oct;117(4):638–644. doi: 10.1016/s0022-3476(05)80707-3. [DOI] [PubMed] [Google Scholar]
  3. Bowles N. E., Dubowitz V., Sewry C. A., Archard L. C. Dermatomyositis, polymyositis, and Coxsackie-B-virus infection. Lancet. 1987 May 2;1(8540):1004–1007. doi: 10.1016/s0140-6736(87)92271-9. [DOI] [PubMed] [Google Scholar]
  4. Bunch T. W., Worthington J. W., Combs J. J., Ilstrup D. M., Engel A. G. Azathioprine with prednisone for polymyositis. A controlled, clinical trial. Ann Intern Med. 1980 Mar;92(3):365–369. doi: 10.7326/0003-4819-92-3-365. [DOI] [PubMed] [Google Scholar]
  5. Dalakas M. C., Illa I., Dambrosia J. M., Soueidan S. A., Stein D. P., Otero C., Dinsmore S. T., McCrosky S. A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis. N Engl J Med. 1993 Dec 30;329(27):1993–2000. doi: 10.1056/NEJM199312303292704. [DOI] [PubMed] [Google Scholar]
  6. Dubowitz V. Treatment of dermatomyositis in childhood. Arch Dis Child. 1976 Jul;51(7):494–500. doi: 10.1136/adc.51.7.494. [DOI] [PMC free article] [PubMed] [Google Scholar]
  7. Furusho K., Kamiya T., Nakano H., Kiyosawa N., Shinomiya K., Hayashidera T., Tamura T., Hirose O., Manabe Y., Yokoyama T. High-dose intravenous gammaglobulin for Kawasaki disease. Lancet. 1984 Nov 10;2(8411):1055–1058. doi: 10.1016/s0140-6736(84)91504-6. [DOI] [PubMed] [Google Scholar]
  8. Heckmatt J., Hasson N., Saunders C., Thompson N., Peters A. M., Cambridge G., Rose M., Hyde S. A., Dubowitz V. Cyclosporin in juvenile dermatomyositis. Lancet. 1989 May 13;1(8646):1063–1066. doi: 10.1016/s0140-6736(89)92456-2. [DOI] [PubMed] [Google Scholar]
  9. Jacobs J. C. Methotrexate and azathioprine treatment of childhood dermatomyositis. Pediatrics. 1977 Feb;59(2):212–218. [PubMed] [Google Scholar]
  10. Kissel J. T., Halterman R. K., Rammohan K. W., Mendell J. R. The relationship of complement-mediated microvasculopathy to the histologic features and clinical duration of disease in dermatomyositis. Arch Neurol. 1991 Jan;48(1):26–30. doi: 10.1001/archneur.1991.00530130034016. [DOI] [PubMed] [Google Scholar]
  11. Miller F. W., Leitman S. F., Cronin M. E., Hicks J. E., Leff R. L., Wesley R., Fraser D. D., Dalakas M., Plotz P. H. Controlled trial of plasma exchange and leukapheresis in polymyositis and dermatomyositis. N Engl J Med. 1992 May 21;326(21):1380–1384. doi: 10.1056/NEJM199205213262102. [DOI] [PubMed] [Google Scholar]
  12. Miller G., Heckmatt J. Z., Dubowitz V. Drug treatment of juvenile dermatomyositis. Arch Dis Child. 1983 Jun;58(6):445–450. doi: 10.1136/adc.58.6.445. [DOI] [PMC free article] [PubMed] [Google Scholar]
  13. Morell A., Schürch B., Ryser D., Hofer F., Skvaril F., Barandun S. In vivo behaviour of gamma globulin preparations. Vox Sang. 1980;38(5):272–283. doi: 10.1111/j.1423-0410.1980.tb02367.x. [DOI] [PubMed] [Google Scholar]
  14. Newburger J. W., Takahashi M., Burns J. C., Beiser A. S., Chung K. J., Duffy C. E., Glode M. P., Mason W. H., Reddy V., Sanders S. P. The treatment of Kawasaki syndrome with intravenous gamma globulin. N Engl J Med. 1986 Aug 7;315(6):341–347. doi: 10.1056/NEJM198608073150601. [DOI] [PubMed] [Google Scholar]
  15. Ronda N., Hurez V., Kazatchkine M. D. Intravenous immunoglobulin therapy of autoimmune and systemic inflammatory diseases. Vox Sang. 1993;64(2):65–72. doi: 10.1111/j.1423-0410.1993.tb02521.x. [DOI] [PubMed] [Google Scholar]
  16. Rossi F., Kazatchkine M. D. Antiidiotypes against autoantibodies in pooled normal human polyspecific Ig. J Immunol. 1989 Dec 15;143(12):4104–4109. [PubMed] [Google Scholar]
  17. Scott O. M., Hyde S. A., Goddard C., Dubowitz V. Quantitation of muscle function in children: a prospective study in Duchenne muscular dystrophy. Muscle Nerve. 1982 Apr;5(4):291–301. doi: 10.1002/mus.880050405. [DOI] [PubMed] [Google Scholar]
  18. Silverman E. D., Laxer R. M., Greenwald M., Gelfand E., Shore A., Stein L. D., Roifman C. M. Intravenous gamma globulin therapy in systemic juvenile rheumatoid arthritis. Arthritis Rheum. 1990 Jul;33(7):1015–1022. doi: 10.1002/art.1780330714. [DOI] [PubMed] [Google Scholar]

Articles from Archives of Disease in Childhood are provided here courtesy of BMJ Publishing Group

RESOURCES