Intravenous immunoglobulin in paediatric neurology: safety, adherence to guidelines, and long-term outcome

Margherita Nosadini, Shekeeb S. Mohammad, Agnese Suppiej, Stefano Sartori, Russell C. Dale*, IVIG in Neurology Study Group

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

28 Citations (Scopus)

Abstract

Aim: Intravenous immunoglobulin (IVIG) is an expensive therapy used in immunodeficiency and autoimmune disorders. Increasing demands and consequent shortages result in a need for usage to conform to guidelines. 

Method: We retrospectively evaluated IVIG use for neuroimmunological indications and adherence to existing guidelines in a major Australian paediatric hospital between 2000 and 2014. 

Results: One-hundred and ninety-six children (96 male, 100 female; mean age at disease onset 6y 5mo [range 3mo–15y 10mo], mean age at first IVIG dose 7y 2mo [range 3mo–16y 5mo]) received IVIG for neuroimmunological indications during the study period (28.1% had Guillain–Barré syndrome), representing 15.5% of all hospital indications. In total, 1669 IVIG courses were administered (total 57 221g, median 78g/patient, range 12–5748g). The highest median numbers of courses were in chronic inflammatory demyelinating polyneuropathies, opsoclonus-myoclonus ataxia syndrome, suspected immune-mediated epilepsies, and Rasmussen's encephalitis. Adverse reactions occurred in 25.5% of patients, but these were mostly minor. Outcome at follow-up was best in anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis, Guillain–Barré syndrome, and myasthenia gravis, and worst in Rasmussen's encephalitis and epilepsies. The total cost of IVIG was US$2 595 907 (median $3538/patient, range $544–260 766). Of patients receiving IVIG, 45.4% to 57.1% were given the therapy for ‘weak’ indications or indications ‘not listed’ in international guidelines. Some entities commonly treated with IVIG in current practice, such as anti-NMDAR encephalitis and transverse myelitis, are not listed in most guidelines. 

Interpretation: Our study demonstrates that IVIG is generally well tolerated but expensive, and discloses discrepancies between guidelines and clinical practice in paediatric neurology, suggesting both the need for greater adherence to current recommendations, and for recommendations to be updated to accommodate emerging indications.

Original languageEnglish
Pages (from-to)1180-1192
Number of pages13
JournalDevelopmental Medicine and Child Neurology
Volume58
Issue number11
DOIs
Publication statusPublished - 1 Nov 2016
Externally publishedYes

Fingerprint

Dive into the research topics of 'Intravenous immunoglobulin in paediatric neurology: safety, adherence to guidelines, and long-term outcome'. Together they form a unique fingerprint.

Cite this