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Use of Intravenous Immunoglobulin Therapy in a paediatric rheumatology service

Background

Intravenous Immunoglobulin (IVIG) is widely used in paediatric inflammatory disease. The Department of Health, UK recently produced guidelines for IVIG use and a demand management plan in response to global IVIG shortages, recommending use in "selected" patients with juvenile dermatomyositis (JDMS), as a "last resort" in systemic juvenile idiopathic arthritis (sJIA), and use not supported in juvenile systemic lupus erythematous (jSLE) and vasculitis.

In this context, we have retrospectively audited use of IVIG in a UK centre.

Materials and methods

A pilot analysis of children treated with IVIG in the previous 2 years.

Results

4 patients were male and 6 were female. The diseases were: sJIA (4), JDMS (3), jSLE (1) and polyarteritis nodosa (2). This represented approximately 50% of the total sJIA cohort and 75% of the JDMS cohort presenting during the study period.

9/10 had inadequate response to high dose pulse intravenous and oral steroid therapy along with first line disease modifying agents. The other patient received IVIG post stem cell transplant for sJIA.

7 children were able to taper their prednisolone dose < 0.5 mg/k/day at the end of 6 months. This steroid sparing effect is a surrogate marker for clinical response. One child did not tolerate IVIG.

Conclusion

IVIG is a valuable adjunct in a broad spectrum of paediatric inflammatory disease and we believe it should remain a treatment option in selected cases with partial response to standard first line therapies.

References

  1. 1.

    Uziel Y, Laxer RM, Schneider R, Silverman ED: Intravenous immunoglobulin therapy in systemic onset juvenile rheumatoid arthritis: a follow up study. Journal of Rheumatology. 1996, 23: 910-918.

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Author information

Correspondence to SM Saladi.

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Open Access This article is published under license to BioMed Central Ltd. This is an Open Access article is distributed under the terms of the Creative Commons Attribution 2.0 International License (https://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Keywords

  • Juvenile Idiopathic Arthritis
  • Intravenous Immunoglobulin
  • Dermatomyositis
  • Polyarteritis Nodosa
  • Paediatric Rheumatology