Abstract
Objective: In a large group of patients with primary immunodeficiency (PID), immunoglobulin replacement therapy is critical for infection control. There are two main methods of immunoglobulin replacement intravenous (IVIG) and subcutaneous (SCIG). The aim of this study was to determine the efficacy of SCIG by comparing IgG levels and frequency of infections obtained during SCIG replacements in patients with PID with those obtained during IVIG administration.
Method: Immunoglobulin levels of 28 patients who were followed up in our clinic with a diagnosis of PID and who started IVIG replacement and switched to SCIG replacement after follow-up, were evaluated retrospectively. Serum IgG levels and frequency of infections before starting immunoglobulin treatment, the previous year of IVIG before starting SCIG replacement, and during the first six months, second six months, and second year of SCIG replacement were compared.
Results: The mean age of all the patients that received SCIG was 10.5 years (min 15 months, max 23 years) and eleven of the patients were female. The mean serum IgG level of the patients before starting immunoglobulin replacement was 701±383 mg/dl, and for the final year they received IVIG replacement before switching to SCIG replacement it was calculated to be 900±342 mg/dl. The mean value was found to be 1082±312 mg/dl in the first six months after the initiation of SCIG, 1102±287 mg/dl in the second six months, and 1145±311 mg/dl in the second year. Serum IgG levels of the patients were significantly higher during IVIG and SCIG replacement than before treatment (p<0.05). Serum IgG levels during the first six months, second six months, and second year of SCIG treatment were significantly higher than levels during IVIG treatment (p=0.000, p=0.003, and p=0.002, respectively).
Conclusion: Compared to IVIG replacement, significantly higher and more stable serum IgG levels can be obtained with SCIG replacement. This is expected to ensure improved outcomes in the management of infections in PID patients.
Keywords: IgG levels, intravenous immunoglobulin replacement, subcutaneous immunoglobulin replacement
Copyright and license
Copyright © 2023 The author(s). This is an open-access article published by Aydın Pediatric Society under the terms of the Creative Commons Attribution License (CC BY) which permits unrestricted use, distribution, and reproduction in any medium or format, provided the original work is properly cited.
How to cite
References
- van Zelm MC, Condino-Neto A, Barbouche M-R. Editorial: Primary Immunodeficiencies Worldwide. Front Immunol. 2020;10:3148. https://doi.org/10.3389/fimmu.2019.03148
- Bousfiha A, Moundir A, Tangye SG, et al. The 2022 Update of IUIS Phenotypical Classification for Human Inborn Errors of Immunity. J Clin Immunol. 2022;42:1508-20. https://doi.org/10.1007/s10875-022-01352-z
- Buckley R, editor. Diagnostic and Clinical Care Guidelines for Primary Immunodeficiency Diseases. Towson, MD: Immune Deficiency Foundation; 2008.
- Eades-Perner AM, Gathmann B, Knerr V, et al. The European internet-based patient and research database for primary immunodeficiencies: results 2004-06. Clin Exp Immunol. 2007;147:306-12. https://doi.org/10.1111/j.1365-2249.2006.03292.x
- Bruton OC, Agammaglobulinemia. Pediatrics. 1952;9:722-8.
- Stiehm ER. Conventional therapy of primary immunodeficiency diseases. In: Ochs HD, Smith CE, Puck JM, editors. Primary immunodeficiency diseases a molecular and genetic approach (3rd ed). New York: Oxford University Press; 2014:833-53.
- Sriaroon P, Ballow M. Immunoglobulin Replacement Therapy for Primary Immunodeficiency. Immunol Allergy Clin North Am. 2015;35:713-30. https://doi.org/10.1016/j.iac.2015.07.006
- Ozen A, Karakoc-Aydiner E. Immunoglobulin replacement therapy in primary immunodeficiencies: Current status report 2019. Ankara: Buluş Publishing; 2019.
- Shrestha P, Karmacharya P, Wang Z, Donato A, Joshi AY. Impact of IVIG vs. SCIG on IgG trough level and infection incidence in primary immunodeficiency diseases: A systematic review and meta-analysis of clinical studies. World Allergy Organ J. 2019;12:100068. https://doi.org/10.1016/j.waojou.2019.100068
- Gardulf A. Immunoglobulin treatment for primary antibody deficiencies: advantages of the subcutaneous route. BioDrugs. 2007;21:105-16. https://doi.org/10.2165/00063030-200721020-00005
- Lemieux R, Bazin R, Néron S. Therapeutic intravenous immunoglobulins. Mol Immunol. 2005;42:839-48. https://doi.org/10.1016/j.molimm.2004.07.046
- Berger M. Subcutaneous immunoglobulin replacement in primary immunodeficiencies. Clin Immunol. 2004;112:1-7. https://doi.org/10.1016/j.clim.2004.02.002
- Perez EE, Orange JS, Bonilla F, et al. Update on the use of immunoglobulin in human disease: A review of evidence. J Allergy Clin Immunol. 2017;139:S1-46. https://doi.org/10.1016/j.jaci.2016.09.023
- Wasserman RL, Melamed I, Kobrynski L, et al. Efficacy, safety, and pharmacokinetics of a 10% liquid immune globulin preparation (GAMMAGARD LIQUID, 10%) administered subcutaneously in subjects with primary immunodeficiency disease. J Clin Immunol. 2011;31:323-31. https://doi.org/10.1007/s10875-011-9512-z
- Wasserman RL, Irani AM, Tracy J, et al. Pharmacokinetics and safety of subcutaneous immune globulin (human), 10% caprylate/chromatography purified in patients with primary immunodeficiency disease. Clin Exp Immunol. 2010;161:518-26. https://doi.org/10.1111/j.1365-2249.2010.04195.x
- Shabaninejad H, Asgharzadeh A, Rezaei N, Rezapoor A. A Comparative Study of Intravenous Immunoglobulin and Subcutaneous Immunoglobulin in Adult Patients with Primary Immunodeficiency Diseases: A Systematic Review and Meta-Analysis. Expert Rev Clin Immunol. 2016;12:595-602. https://doi.org/10.1586/1744666X.2016.1155452
- Bezrodnik L, Gómez Raccio A, Belardinelli G, et al. Comparative study of subcutaneous versus intravenous IgG replacement therapy in pediatric patients with primary immunodeficiency diseases: a multicenter study in Argentina. J Clin Immunol. 2013;33:1216-22. https://doi.org/10.1007/s10875-013-9916-z
- Bonilla FA, Khan DA, Ballas ZK, et al. Practice parameter for the diagnosis and management of primary immunodeficiency. J Allergy Clin Immunol. 2015;136:1186-205.e1-78. https://doi.org/10.1016/j.jaci.2015.04.049
- Roifman CM, Levison H, Gelfand EW. High-dose versus low-dose intravenous immunoglobulin in hypogammaglobulinaemia and chronic lung disease. Lancet. 1987;1:1075-7. https://doi.org/10.1016/s0140-6736(87)90494-6
- Orange JS, Belohradsky BH, Berger M, et al. Evaluation of correlation between dose and clinical outcomes in subcutaneous immunoglobulin replacement therapy. Clin Exp Immunol. 2012;169:172-81. https://doi.org/10.1111/j.1365-2249.2012.04594.x
- Ballow M. Optimizing immunoglobulin treatment for patients with primary immunodeficiency disease to prevent pneumonia and infection incidence: review of the current data. Ann Allergy Asthma Immunol. 2013;111:S2-5. https://doi.org/10.1016/j.anai.2013.06.013