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Abstract

Background:

Subcutaneous immunoglobulin G (SCIG) may be a better option than intravenous immunoglobulin G (IVIG) for patients with primary immunodeficiencies (PID) due to reduced systemic and serious adverse reactions and easier administration. The Infusione Bimensile di Immunoglobuline Sottocute (IBIS) study investigated the effects of Hizentra^®, a 20%-concentrated SCIG, administered biweekly in patients with PID. This subanalysis aimed to evaluate clinical and laboratory outcomes in the IBIS pediatric subcohort.

Methods:

Thirteen children with PID were observed for 12 months retrospectively (with previous IVIG/SCIG) and prospectively with biweekly Hizentra.

Results:

Mean ± standard deviation serum IG levels during the retrospective (833.8 ± 175.7 mg/dL) and the prospective (842.0 ± 188.0 mg/dL) phases were comparable; there were also no differences in the number of infections.

Conclusions:

Biweekly Hizentra is a noninferior option with respect to previous IVIG/SCIG-based treatment.

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References

Buckley

. Primary immunodeficiency diseases due to defects in lymphocytes. N Engl J Med, 2000; 343:1313–1324.

Berger

. Principles of and advances in immunoglobulin replacement therapy for primary immunodeficiency. Immunol Allergy Clin North Am, 2008; 28:413–437.

Bousfiha

, Jeddane

, Picard

, et al. The 2017 IUIS phenotypic classification for primary immunodeficiencies. J Clin Immunol, 2018; 38:129–143.

Radinsky

, Bonagura

. Subcutaneous immunoglobulin infusion as an alternative to intravenous immunoglobulin. J Allergy Clin Immunol, 2003; 112:630–633.

Gardulf

, Nicolay

, Asensio

, et al. Rapid subcutaneous IgG replacement therapy is effective and safe in children and adults with primary immunodeficiencies-a prospective, multi-national study. J Clin Immunol, 2006; 26:177–185.

Canessa

, Iacopelli

, Pecoraro

, et al. Shift from intravenous or 16% subcutaneous replacement therapy to 20% subcutaneous immunoglobulin in patients with primary antibody deficiencies. Int J Immunopathol Pharmacol, 2017; 30:73–82.

Misbah

, Chapel

. Adverse effects of intravenous immunoglobulin. Drug Saf, 1993; 9:254–262.

Borte

, Bernatowska

, Ochs

, et al. Efficacy and safety of home-based subcutaneous immunoglobulin replacement therapy in paediatric patients with primary immunodeficiencies. Clin Exp Immunol, 2011; 164:357–364.

Hamrock

. Adverse events associated with intravenous immunoglobulin therapy. Int Immunopharmacol, 2006; 6:535–542.

10.

CSL Behring. Hizentra^® 200 mg/ml solution for subcutaneous injection: summary of product characteristics. 2016. www.ema.europa.eu/docs/en_GB/document_library/EPAR_-_Product_Information/human/002127/WC500107057.pdf. Accessed February 5, 2018.

11.

CSL Behring. Hizentra^®, Immune globulin subcutaneous (human), 20% Liquid. Full prescribing information. 2018. http://labeling.cslbehring.com/PI/US/Hizentra/EN/Hizentra-Prescribing-Information.pdf. Accessed February 5, 2018.

12.

Vultaggio

, Azzari

, Ricci

, et al. Biweekly Hizentra^® in primary immunodeficiency: a multicenter, observational cohort study (IBIS). J Clin Immunol, 2018; 38:602–609.

13.

Perez

, Orange

, Bonilla

, et al. Update on the use of immunoglobulin in human disease: a review of evidence. J Allergy Clin Immunol, 2017; 139:S1–S46.

14.

Borte

, Pac

, Serban

, et al. Efficacy and safety of Hizentra^®, a new 20% immunoglobulin preparation for subcutaneous administration, in pediatric patients with primary immunodeficiency. J Clin Immunol, 2011; 31:752–761.

15.

Gustafson

, Gardulf

, Hansen

, et al. Rapid subcutaneous immunoglobulin administration every second week results in high and stable serum immunoglobulin G levels in patients with primary antibody deficiencies. Clin Exp Immunol, 2008; 152:274–279.

16.

Jolles

, Bernatowska

, de Gracia

, et al. Efficacy and safety of Hizentra^® in patients with primary immunodeficiency after a dose-equivalent switch from intravenous or subcutaneous replacement therapy. Clin Immunol, 2011; 141:90–102.

17.

U.S. Food and Drug Administration. Guidance for industry: safety, efficacy and pharmacokinetic studies to support marketing of immune globulin intravenous (human) as replacement therapy for primary humoral immunodeficiency. 2008. www.fda.gov/downloads/BiologicsBloodVaccines/GuidanceComplianceRegulatoryInformation/Guidances/Blood/UCM078526.pdf. Accessed February 7, 2018.