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Immune globulin therapy in primary immunodeficiency

Author
Jordan S Orange, MD, PhD
Section Editor
E Richard Stiehm, MD
Deputy Editor
Anna M Feldweg, MD

INTRODUCTION

Polyclonal immune globulin consists of immunoglobulins (mostly immunoglobulin G [IgG]) purified from pooled human plasma. Immune globulin is used to treat a wide variety of diseases, including primary and secondary immunodeficiency states and hematologic and autoimmune disorders [1].

Terminology — Immune globulin, intravenous (human) will be referred to as "IVIG" in this review, because this term is commonly used by clinicians, although the abbreviation preferred by various regulatory agencies is "IGIV." Immune globulin can also be administered subcutaneously. Immune globulin, subcutaneous (human) will be referred to as "SCIG."

The use of immune globulin in primary immunodeficiency will be reviewed here. General principles in the use of IVIG and SCIG and the role of these agents in the treatment of various hematologic and autoimmune disorders are presented separately. (See "Overview of intravenous immune globulin (IVIG) therapy".)

Comparisons of intravenous and subcutaneous therapy, both traditional and hyaluronidase-facilitated, are discussed elsewhere. (See "Subcutaneous and intramuscular immune globulin therapy".)

INDICATIONS

Immune globulin therapy is the mainstay of treatment for a variety of primary immunodeficiency states [2].

                          

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Literature review current through: Nov 2016. | This topic last updated: Fri Nov 13 00:00:00 GMT+00:00 2015.
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References
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  1. Orange JS, Hossny EM, Weiler CR, et al. Use of intravenous immunoglobulin in human disease: a review of evidence by members of the Primary Immunodeficiency Committee of the American Academy of Allergy, Asthma and Immunology. J Allergy Clin Immunol 2006; 117:S525.
  2. Guidelines for IVIG administration in the United Kingdom. www.ivig.nhs.uk/documents/Clinical%20Guidelines%20SECOND%20EDITION%20(3).pdf (Accessed on January 07, 2009).
  3. Schroeder HW Jr. Primary antibody deficiencies. In: Clinical immunology: Principles and practice, 2nd ed, Rich RR, Fleisher TA, Shearer WT, et al (Eds), Mosby International Ltd, 2001.
  4. Conley ME, Notarangelo LD, Etzioni A. Diagnostic criteria for primary immunodeficiencies. Representing PAGID (Pan-American Group for Immunodeficiency) and ESID (European Society for Immunodeficiencies). Clin Immunol 1999; 93:190.
  5. Durandy A, Revy P, Imai K, Fischer A. Hyper-immunoglobulin M syndromes caused by intrinsic B-lymphocyte defects. Immunol Rev 2005; 203:67.
  6. 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.
  7. Picard C, Al-Herz W, Bousfiha A, et al. Primary Immunodeficiency Diseases: an Update on the Classification from the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency 2015. J Clin Immunol 2015; 35:696.
  8. US FDA: Guidance for industry: Safety, efficacy and pharmacokinetic studies to support marketing of IGIV (human) as replacement therapy for primary humoral immunodeficiency. www.fda.gov.cber/gdlns/igivimmuno.pdf (Accessed on October 10, 2008).
  9. Busse PJ, Razvi S, Cunningham-Rundles C. Efficacy of intravenous immunoglobulin in the prevention of pneumonia in patients with common variable immunodeficiency. J Allergy Clin Immunol 2002; 109:1001.
  10. Lucas M, Lee M, Lortan J, et al. Infection outcomes in patients with common variable immunodeficiency disorders: relationship to immunoglobulin therapy over 22 years. J Allergy Clin Immunol 2010; 125:1354.
  11. Berger M. Incidence of infection is inversely related to steady-state (trough) serum IgG level in studies of subcutaneous IgG in PIDD. J Clin Immunol 2011; 31:924.
  12. Orange JS, Grossman WJ, Navickis RJ, Wilkes MM. Impact of trough IgG on pneumonia incidence in primary immunodeficiency: A meta-analysis of clinical studies. Clin Immunol 2010; 137:21.
  13. de Gracia J, Vendrell M, Alvarez A, et al. Immunoglobulin therapy to control lung damage in patients with common variable immunodeficiency. Int Immunopharmacol 2004; 4:745.
  14. ONCLEY JL, MELIN M. The separation of the antibodies, isoagglutinins, prothrombin, plasminogen and beta1-lipoprotein into subfractions of human plasma. J Am Chem Soc 1949; 71:541.
  15. KISTLER P, NITSCHMANN H. Large scale production of human plasma fractions. Eight years experience with the alcohol fractionation procedure of Nitschmann, Kistler and Lergier. Vox Sang 1962; 7:414.
  16. Hachulla E. [IgIV at home:experience of a center--economic aspects]. Rev Med Interne 2007; 28 Spec No. 1:7.
  17. Hayakawa F, Imada K, Towatari M, Saito H. Life-threatening human parvovirus B19 infection transmitted by intravenous immune globulin. Br J Haematol 2002; 118:1187.
  18. 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.
  19. Hernandez-Trujillo HS, Chapel H, Lo Re V 3rd, et al. Comparison of American and European practices in the management of patients with primary immunodeficiencies. Clin Exp Immunol 2012; 169:57.
  20. 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; 122:210.
  21. Misbah SA. Effective dosing strategies for therapeutic immunoglobulin: managing wear-off effects in antibody replacement to immunomodulation. Clin Exp Immunol 2014; 178 Suppl 1:70.
  22. Jolles S, Orange JS, Gardulf A, et al. Current treatment options with immunoglobulin G for the individualization of care in patients with primary immunodeficiency disease. Clin Exp Immunol 2015; 179:146.
  23. Kurecová B, Janků P, Litzman J. [Common variable immunodeficiency in pregnancy (set of case reports)]. Ceska Gynekol 2009; 74:197.
  24. Williams PE, Leen CL, Heppleston AD, Yap PL. IgG replacement therapy for primary hypogammaglobulinaemia during pregnancy: report of 9 pregnancies in 4 patients. Blut 1990; 60:198.
  25. Berger M, Cupps TR, Fauci AS. High-dose immunoglobulin replacement therapy by slow subcutaneous infusion during pregnancy. JAMA 1982; 247:2824.
  26. Gardulf A, Andersson E, Lindqvist M, et al. Rapid subcutaneous IgG replacement therapy at home for pregnant immunodeficient women. J Clin Immunol 2001; 21:150.
  27. Palmeira P, Costa-Carvalho BT, Arslanian C, et al. Transfer of antibodies across the placenta and in breast milk from mothers on intravenous immunoglobulin. Pediatr Allergy Immunol 2009; 20:528.
  28. Hansen S, Gardulf A, Andersson E, et al. Women with primary antibody deficiencies requiring IgG replacement therapy: their perception of prenatal care during pregnancy. J Obstet Gynecol Neonatal Nurs 2004; 33:604.