Abstract

Abstract Background IVIG is a therapeutic preparation comprising of pooled blood donated from large numbers of healthy people. There are different preparations of IVIG available. All should have high levels of IgG (>95%) but vary in concentrations, pH, stabilizing agents and IgA content. In inflammatory conditions, IVIG is often used as an adjunct therapy in patients with poor response to first line therapy and in general well tolerated. However adverse reactions can occur and vary in severity from mild constitutional symptoms to aseptic meningitis. The aim of this study was to identify patients who required IVIG within the paediatric rheumatology department at Great Ormond Street Hospital, the underlying diagnosis of these patients and the indication for starting treatment. The study also looked at the number of adverse drug reactions of patients requiring IVIG, the symptoms they presented with and the management of these symptoms. Methods A retrospective case review, of patients under the care of the paediatric rheumatology team who received IVIG between July 2013 to August 2018. We collected demographics including, age, sex, diagnosis of patients and indication for commencing IVIG, symptoms of any adverse drug reaction and management of symptoms. Results We identified 43 patients who required IVIG over the last 5 years as part of their treatment plan, of which 23 patients had a diagnosis of Juvenile dermatomyositis (JDM). Of the remaining patients 5 had a diagnosis of SLE, 4 with SRP positive mysositis, 2 with necrotising myositis and 2 with atypical systemic onset JIA. The main indication for starting treatment was for active or worsening skin disease, muscle weakness or both. From the 43 patients that required IVIG, 21 patients had an adverse drug reaction and 15/21 patients had a diagnosis of JDM. 5/21 patients had symptoms associated with aseptic meningitis (headache, photophobia and neck stiffness), 2 of which were unable to continue treatment. The remaining 16 patients had milder constitutional symptoms. Symptoms were managed by premedicating patients prior to subsequent IVIG treatment with a combination of paracetamol, chlorphenamine, ondansetron or by reducing the rate of IVIG infusion or reducing the dose of IVIG from 2g/kg to 1g/kg. Conclusion We identified from the study, that half the patients requiring IVIG as part of their treatment plan had an adverse drug reaction of which half had a diagnosis of JDM. However, the majority of patients who had an adverse drug reaction had mild constitutional symptoms, relieved by premedication or by adjusting IVIG rate of infusion or dose. Only 2 patients were unable to continue IVIG treatment. Disclosures S.H.A. Wildman None. M. Al-Obaidi None.

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