Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2020 Sep 9:16:80.
doi: 10.1186/s13223-020-00478-2. eCollection 2020.

Pediatric subset of primary immunodeficiency patients treated with SCIG: post hoc analysis of SHIFT and IBIS pooled data

Viviana Moschese  1 , Clementina Canessa  2 , Antonino Trizzino  3 , Baldassarre Martire  4 , Giorgio Maria Boggia  5 , Simona Graziani  1 ; SHIFT and IBIS Study Groups
Affiliations

Pediatric subset of primary immunodeficiency patients treated with SCIG: post hoc analysis of SHIFT and IBIS pooled data

Viviana Moschese et al. Allergy Asthma Clin Immunol. .

Abstract

Background: Primary immunodeficiencies (PID) constitute a heterogeneous group of more than 350 monogenetic diseases. PID patients with antibody impairment require lifelong administration of immunoglobulin G replacement therapy, administered either intravenously (IVIG) or subcutaneously (SCIG). Although the effectiveness of weekly and biweekly (every other week) SCIG administration has been shown in several trials, data on the viability of these two regimens in pediatric PID patients are sparse.

Methods: Data on the pediatric subsets of PID patients enrolled in SHIFT (weekly) and IBIS (biweekly) studies were pooled and analyzed to indirectly compare two different 20%-concentrated SCIG (Hizentra®) regimens. The primary endpoints were to evaluate trough IgG levels and cumulative monthly doses; the secondary endpoint was to analyze incidence of infections.

Results: Fifteen and 13 children from the SHIFT and IBIS studies were included, respectively. Cumulative 20%-concentrated SCIG monthly dose was slight lower for the biweekly regimen (Δ = - 2.04, 90% CI - 8.3 to 4.23). However, the trough IgG levels were similar between the two groups (Δ = 0.28, 90% CI - 0.51 to 1.07) and constantly above the threshold of 5 g/L. After adjusting for potential confounders, the annualized rate of infections was similar between SHIFT and IBIS patients (incidence rate ratio = 1.09, 90% CI 0.72-1.67); only 1 serious bacterial infection was experienced by a patient in the IBIS group.

Conclusion: In pediatric PID patients, weekly and biweekly Hizentra® administrations appeared equally effective treatment options.

Keywords: IBIS study; Immunoglobulin; Infection rate; Pediatric patients; Primary immunodeficiency; SHIFT study.

PubMed Disclaimer

Conflict of interest statement

Competing interestsVM has received honoraria by CSL Behring and has taken part to a board sponsored by Takeda. CC, AT and SG declare that they have no competing interests. BM has received honoraria from Takeda and Kedrion. GMB is an employee of CSL Behring.

Figures

Fig. 1
Fig. 1
Distribution of primary immunodeficiency conditions. APDS activated PI3K-delta syndrome, CVID common variable immunodeficiency, DGS DiGeorge syndrome, IgGSD IgG subclass deficiency, SCID severe combined immunodeficiency, UnPAD unclassified primary antibody deficiency, XLA X-linked agammaglobulinemia
Fig. 2
Fig. 2
Weight categories of 28 children from the SHIFT and IBIS groups. Q1W weekly administration, Q2W biweekly administration
Fig. 3
Fig. 3
Box plot of serum IgG trough concentrations (g/L) and distributions in the Q1W and Q2W dosage regimen groups. The bold lines represent the median values, the boxes indicate the interquartile range, and the whiskers represent the minimum and maximum values. Q1W weekly administration, i.e., data from the SHIFT study, Q2W biweekly administration, i.e., data from the IBIS study
Fig. 4
Fig. 4
Type and distribution of 8 non-SBI in 6/11 Q1W patients and 35 non-SBI in 10/13 Q2W patients. Q1W weekly administration, i.e., data from the SHIFT study, Q2W biweekly administration, i.e., data from the IBIS study
See this image and copyright information in PMC

Similar articles

See all similar articles

References

    1. Berger M. Principles of and advances in immunoglobulin replacement therapy for primary immunodeficiency. Immunol Allergy Clin North Am. 2008 - PMC - PubMed
    1. Food and Drug Administration (FDA). Safety, efficacy, and pharmacokinetic studies to support marketing of immune globulin intravenous (human) as replacement therapy for primary humoral immunodeficiency. Guidance for industry. 2008 https://www.fda.gov/media/124333/download.
    1. Stiehm ER. Human intravenous immunoglobulin in primary and secondary antibody deficiencies. Pediatr Infect Dis J. 1997;16:696–707. - PubMed
    1. Bonagura VR, Marchlewski R, Cox A, Rosenthal DW. Biologic IgG level in primary immunodeficiency disease: the IgG level that protects against recurrent infection. J Allergy Clin Immunol. 2008 - PubMed
    1. Chapel HM, Spickett GP, Ericson D, Engl W, Eibl MM, Bjorkander J. The comparison of the efficacy and safety of intravenous versus subcutaneous immunoglobulin replacement therapy. J Clin Immunol. 2000;20:94–100. - PubMed

LinkOut - more resources