Subcutaneous immunoglobulin therapy: A new option for patients with primary immunodeficiency diseases

Biologics: Targets and Therapy

Volumn 6, Issue , 2012, Pages 277-287

  Kobrynski, Lisa ^a  

^a EMORY UNIVERSITY SCHOOL OF MEDICINE   (United States)

Author keywords

Immunoglobulin g; Primary immunodeficiency diseases; Subcutaneous immunoglobulin

Indexed keywords

GAMMAKED; GAMUNEX C; HUMAN IMMUNOGLOBULIN; IMMUNOGLOBULIN; IMMUNOGLOBULIN G; UNCLASSIFIED DRUG;

AREA UNDER THE CURVE; BACTERIAL INFECTION; BRONCHITIS; CHILL; DIZZINESS; DOSE RESPONSE; DRUG ABSORPTION; DRUG BLOOD LEVEL; DRUG DISTRIBUTION; DRUG EFFICACY; DRUG FEVER; DRUG HALF LIFE; DRUG INDUCED HEADACHE; DRUG SAFETY; DRUG SYNTHESIS; DRUG WITHDRAWAL; EDEMA; ERYTHEMA; FATIGUE; HEMOLYSIS; HUMAN; IMMUNE DEFICIENCY; INFECTION PREVENTION; MALAISE; OTITIS MEDIA; PATIENT SELECTION; QUALITY OF LIFE; RECOMMENDED DRUG DOSE; REVIEW; SINUSITIS; THROMBOSIS; TRANSFUSION RELATED ACUTE LUNG INJURY; URTICARIA; VIRUS INFECTION; VOMITING;

EID: 84866765312     PISSN: 11775475     EISSN: 11775491     Source Type: Journal    
DOI: 10.2147/BTT.S25188     Document Type: Review

References (93)

1. Al-Herz W, Bousfiha A, Casanova JL, et al. Primary immunodeficiency diseases: an update on the classification from the International Union of Immunological Societies Expert Committee for Primary Immunodeficiency. Front Immunol. 2011;2(54):1-26.
2. Bruton OC. Agammaglobulinemia. Pediatrics. 1952;9:722-728.
3. Berger M, Cupps TR, Fauci AS. Immunoglobulin replacement therapy by slow subcutaneous infusion. Ann Intern Med. 1980;93(1):55-56.
4. Roord JJ, van der Meer JW, Kuis M, et al. Home treatment in patients with antibody deficiency by slow subcutaneous infusion of gammaglobulin. Lancet. 1982;1(8273):689-690.
5. Ugazio AG, Duse M, Re R, Mangili G, Burgio GR. Subcutaneous infusions of gammaglobulins in management of agammaglobulinaemia. Lancet. 1982;319(8265):226.
6. Welch MJ, Stiehm ER. Slow subcutaneous immunoglobulin therapy in a patient with reactions to intramuscular immunoglobulin. J Clin Immunol. 1983;3(3):285-286.
7. Gardulf A, Hammarstrom L, Smith CL. Home treatment of hypogammaglobulinemia with subcutaneous gammaglobulin by rapid infusion. Lancet. 1991;338(8760):162-166.
8. fda.gov [homepage on the Internet]. January 9, 2006 approval letter for Vivaglobin. Silver Spring, MD: US Food and Drug Administration [updated 2011 April 8]. Available from: http://www.fda.gov/BiologicsBloodVaccines/BloodBloodProducts/Approvedproducts/licensedproductsBLAs/fractionatedplasmaproducts/UCM070367.htm. Accessed April 24, 2012.
9. Gammagard liquid® (immune globulin infusion, human, 10%) [prescribing information]. West Lake Village, CA: Baxter Healthcare Corporation; 2012.
10. Gammaked™ (immune globulin injection, human, 10%) [prescribing information]. Research Triangle Park, NC: Talecris Biotherapeutics, Inc.; 2011.
11. Gamunex-C (immune globulin injection, human, 10%) [prescribing information]. Research Triangle Park, NC: Talecris Biotherapeutics, Inc.; 2010.
12. Hizentra (immune globulin subcutaneous, human, 20% liquid) [prescribing information]. Kankakee, IL: CSL Behring LLC; 2011.
13. Lee DC, Stenland CJ, Hartwell RC, et al. Monitoring plasma processing steps with a sensitive Western Blot assay for the detection of the prion protein. J Virol Methods. 2000;84(1):77-89.
14. Stenland CJ, Lee DC, Brown P, et al. Partitioning of human and sheep forms of the pathogenic prion protein during the purification of therapeutic proteins from human plasma. Transfusion. 2002;42:1497-500.
15. Strucki M, Boschetti N, Schafer W, et al. Investigations of prion and virus safety of a new liquid IVIG product. Biologicals. 2008;36(4):239-247.
16. Mankarious S, Lee M, Fischer S, et al. The half-lives of IgG subclasses and specific antibodies in patients with primary immunodeficiency who are receiving intravenously administered immunoglobulin. J Lab Clin Med. 1988;112(5):634-640.
17. Waldman TA, Strober W, Blaese RM. Metabolism of immunoglobulins. Prog Allergy. 1969:13:1-110.
18. Waniewski I, Gardulf A, Hammarström L. Bioavailability of gamma-globulin after subcutaneous infusions in patients with common variable immunodeficiency. J Clin Immunol. 1994;14(2):90-97.
19. Borte M, Pac M, Serban M, 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.
20. 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;161:518-526.
21. Ochs HD, Gupta S, Kiessling P, Nicolay U, Berger M. Safety and efficacy of self-administered subcutaneous immunoglobulin in patients with primary immunodeficiency diseases. J Clin Immunol. 2006;26(3):265-273.
22. Gardulf A, Nicolay U, Asensio O, 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(2):177-185.
23. Desai SH, Chouksey A, Poll J, Berger M. A pilot study of equal doses of 10% IGIV given intravenously or subcutaneously. J Allergy Clin Immunol. 2009;124:854-856.
24. Vivaglobin® (immune globulin subcutaneous, human, 16%) [prescribing information]. Kankakee, IL: CSL Behring, LLC; 2010.
25. Ochs HD, Pinciaro PJ. The Octogam study group, Octogam 5% an intravenous IgG product is efficacious and well tolerated in subjects with primary immunodeficneicy diseases. J Clin Immunol. 2004;24:309-314.
26. Ballow M. Safety of IGIV therapy and infusion related adverse events. Immunol Res. 2007;38:122-132.
27. Gardulf A, Nicolay U, Math D, et al. Children and adults with primary antibody deficiencies gain quality of life by subcutaneous IgG self-infusions at home. J Allergy Clin Immunol. 2004;114:936-942.
28. Immune Deficiency Foundation. Treatment Experiences and Preferences of Patients with Primary Immune Deficiency Diseases: First National Survey 2002. June 20, 2003. Available from: http://primaryimmune.org/about-primary-immunodeficiency-diseases/idf-publications?aid=1266&pid=275&sa=1. Accessed April 2, 2012.
29. Nicolay U, Kiessling P, Berger M, et al. Health-related quality of life and treatment satisfaction in North American patients with Primary Immunedefiency Diseases receiving subcutaneous IgG self-infusions at home. J Clin Immunol. 2006;26:65-72.
30. Bayrakci B, Ersoy F, Sanal O, et al. The efficacy of immunoglobulin replacement therapy in the long-term follow-up of the B-cell deficiencies. Turk J Pediatr. 2005;47:239-246.
31. 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;109:1001-1004.
32. Liese JG, Wintergerst U, Tympner KD, Belohradsky BH. High-vs lowdose immunoglobulin therapy in the long-term treatment of X-linked agammaglobulinemia. Am J Dis Child. 1992;146:335-339.
33. Nolte MT, Pirofsky B, Gerritz GA, Golding B. Intravenous immunoglobulin therapy for antibody deficiency. Clin Exp Immunol. 1979;36:237-243.
34. Pourpak Z, Aghamohammadi A, Sedighipour L, et al. Effect of regular intravenous immunoglobulin therapy on prevention of pneumonia in patients with common variable immunodeficiency. J Microbiol Immunol Infect. 2006;39:114-120.
35. Roifman CM, Lederman HM, Lavi S, Stein LD, Levison H, Gelfand EW. Benefit of intravenous IgG replacement in hypogammaglobulinemic patients with chronic sinopulmonary disease. Am J Med. 1985;79:171-174.
36. 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.
37. Berger M, Murphy E, Riley P, Bergman GE; VIRTUE Trial Investigators. Improved quality of life, immunoglobulin G levels, and infection rates in patients with primary immunodeficiency disease during self-treatment with subcutaneous immunoglobulin G. South Med J. 2010;103(9):856-863.
38. Hagan JB, Fasano MB, Spector S, et al. Efficacy and safety of a new 20% immunoglobulin preparation for subcutaneous administration, IgPro20, in patients with primary immunodeficiency. J Clin Immunol. 2010;30:734-745.
39. Jolles S, Bernatowska E, de Gracia J, 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.
40. 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;31:323-331.
41. 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;137:21-30.
42. 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;125:1354-1360.
43. Eijkhout HW, van Der Meer JW, Kallenberg CG, et al. The effect of two different dosages of intravenous immunoglobulin on the incidence of recurrent infections in patients with primary hypogammaglobulinemia. A randomized, double-blind, multicenter crossover trial. Ann Intern Med. 2001;135(3):165-174.
44. Roifman CM, Levison H, Gelfand EW. High-dose versus low-dose intravenous immunoglobulin in hypogammaglobulinemia and chronic lung disease. Lancet. 1987;1(8541):1075-1077.
45. Shehata N, Palda V, Bowen T, et al. The use of immunoglobulin therapy for patients with primary immune deficiency: an evidence-based practice guideline. Transfus Med Rev. 2010;24:S28-S50.
46. Quartier P, Debré M, De Blic J, et al. Early and prolonged intravenous immunoglobulin replacement therapy in childhood agammaglobulinemia: a retrospective survey of 31 patients. J Pediatr. 1999;134:589-596.
47. Roifman CM, Schroeder H, Berger M, et al. Comparison of the efficacy of IGIV-C, 10% (caprylate/chromatography) and IGIV-SD, 10% as replacement therapy in primary immune deficiency. A randomized doubleblind trial. Int Immunopharmacol. 2003;3:1325-1333.
48. Berger M. Principles of and advances in immunoglobulin replacement therapy for primary immunodeficiency. Immunol Allergy Clin North Am. 2008;28(2):413-437.
49. 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(1):210-212.
50. Cunningham-Rundles C. How I treat common variable immune deficiency. Blood. 2010;116:7-15.
51. 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;31:924-926.
52. Khan S, Grimbacher B, Boecking C, et al. Serum trough IgG level and annual intravenous immunoglobulin dose are not related to body size in patients on regular replacement therapy. Drug Metab Lett. 2011;5:132-136.
53. Berger M, Rojavin M, Kiessling P, Zenker O. Pharmacokinetics of subcutaneous immunoglobulin and their use in dosing of replacement therapy in patients with primary immunodeficiencies. Clin Immunol. 2011;139:133-141.
54. Quinti I, Soresina A, Guerra A, et al. Effectiveness of immunoglobulin replacement therapy on clinical outcome in patients with primary antibody deficiencies: results from a multicenter prospective cohort study. J Clin Immunol. 2011;31:315-322.
55. Chapel H, Cunningham-Rundles C. Update in understanding common variable immunodeficiency disorders (CVIDs) and the management of patients with these conditions. Br J Haematol. 2009;145:709-727.
56. Roifman CM, Berger M, Notarangelo LD. Management of primary antibody deficiency with replacement therapy: summary of guidelines. Immunol Allergy Clin North Am. 2008;28(4):875-876.
57. Yong PI, Boyle J, Ballow M, et al. Use of intravenous immunoglobulin and adjunctive therapies in the treatment of primary immunodeficiencies. A working group report of and study by the Primary Immunodeficiency Committee of the American Academy of Allergy, Asthma and Immunology. Clin Immunol. 2010;135(2):255-263.
58. Gardulf A, Bjorvell H, Gustafson R, et al. Safety of rapid subcutaneous gamma-globulin infusions in patients with primary antibody deficiency. Immunodeficiency. 1993;4:81-84.
59. Gaspar J, Gerritsen B, Jones A. Immunoglobulin replacement treatment by rapid subcutaneous infusion. Arch Dis Child. 1998;79:48-51.
60. Hansen S, Gustafson R, Smith CI, Gardulf A. Express subcutaneous IgG infusions: decreased time of delivery with maintained safety. Clin Immunol. 2002;104:237-241.
61. Shapiro R. Subcutaneous immunoglobulin therapy by rapid push is preferred to infusion by pump: a retrospective analysis. J Clin Immunol. 2010;30:301-307.
62. Berger M. Subcutaneous administration of IgG. Immunol Allergy Clin North Amer. 2008;28(4):779-802.
63. Gamunex-c.com [homepage on the Internet]. Gamunex-C PI SubQ Infusion Video. USA: Talecris Biotherapeutics; 2010. Available from: http://www.gamunex-c.com/patient-connexions-support-patient-education-PI.htm. Accessed April 30, 2012.
64. Gammagard.com [homepage on the Internet]. How to infuse: subcutaneous administration of Gammagard liquid. Westlake Village, CA: Baxter Healthcare Corporation; 2011. Available from: http://www.gammagard.com/patients-and-families/help-for-patients/patient-education.html. Accessed April 30, 2012.
65. hizentra.com [homepage on the Internet]. Self-Administration Video. Kankakee, IL: CSL Behring, LLC; 2010. Available from: www.hizentra.com/consumer/about-hizentra/sub-q-self-administration-video.aspx. Accessed April 30, 2012.
66. primaryimmune.org [homepage on the Internet]. Online accredited course for nurses about primary immunodeficiency and immunoglobulin therapy. Towson, MD: Immune Deficiency Foundation; 2011. Available from: http://primaryimmune.org/2011/09/idf-launches-free-accredited-course-for-nurses-about-primary-immunodeficiency-and-immunoglobulin-therapy. Accessed April 30, 2012.
67. hizentra.com [homepage on the Internet]. SHARE nurse training. Kankakee, IL: CSL Behring, LLC; 2012. Available from: http://www.hizentra.com/share/default.aspx. Accessed April 30, 2012.
68. Berger M, Pinciaro PJ. Safety, efficacy, and pharmacokinetics of Flebogamma 5% immune globulin intravenous (human) for replacement therapy in primary immunodeficiency diseases. J Clin Immunol. 2004;24(4):389-396.
69. Church JA, Leibl H, Stein MR, et al. Efficacy, safety and tolerability of a new 10% liquid intravenous immune globulin (IGIV 10%) in patients with primary immunodeficiency. J Clin Immunol. 2006;26:388-395.
70. Stein M, Nelson R, Church J, et al. Safety and efficacy of Privigen, a novel 10% liquid immunoglobulin preparation for intravenous use, in patients with primary immunodeficiencies. J Clin Immunol. 2009;29:137-144.
71. Wasserman R, Church J, Stein M, et al. Safety, efficacy and pharmacokinetics of a new 10% liquid intravenous immunoglobulin (IVIG) in patients with primary immunodeficiency. J Clin Immunol. March 6, 2012. [Epub ahead of print.]
72. Brennan VM, Salome-Bentley NJ, Chapel HM. Prospective audit of adverse reactions occurring in 459 primary antibody-deficient patients receiving intravenous immunoglobulin. Clin Exp Immunol. 2003;133:247-251.
73. Gardulf A, Anderson V, Bjorkander J, et al. Subcutaneous immunoglobulin replacement in patients with primary antibody deficiencies: safety and costs. Lancet. 1995;345(8946):365-369.
74. Radinsky S, Bonagura VR. Subcutaneous immunoglobulin infusion as an alternative to intravenous immunoglobulin. J Allergy Clin Immunol. 2003;112(3):630-633.
75. Epstein JS, Zoon KC. Dear doctor letter -important drug warning: immune globulin intravenous (human). FDA Med Watch. US Food and Drug Administration; 1998. Available from: http://www.fda.gov/biologicsbloodvaccines/safetyavailability/ucm105914.htm. Accessed July 14, 2012.
76. Pierce LR, Jain N. Risks associated with the use of intravenous immunoglobulin. Transfusion Med Rev. 2003;17(4):241-251.
77. Dalakas MC. High-dose intravenous immunoglobulin and serum viscosity: risk of precipitating thromboembolic events. Neurology. 1994;44:223-226.
78. Daniel GW, Menis M, Sridhar G, et al. Immune globulins and thrombotic adverse events as recorded in a large administrative database in 2008 through 2010. Transfusion. March 12, 2012. [Epub ahead of print.]
79. Ovanesov MV. Laboratory investigations of products associated with thrombotic events. In: FDA Workshop: Risk Mitigation Strategies to Address Procoagulant Activity in Immune Globulin Products. Bethesda, MD; US Food and Drug Administration: 2011. Available from: http://www.fda.gov/downloads/BiologicsBloodVaccines/NewsEvents/WorkshopsMeetingsConferences/UCM260788.pdf. Accessed.
80. Nicolay U, Haag S, Eichmann F, Herget S, Spruck D, Gardulf A. Measuring treatment satisfaction in patients with primary immunodeficiency diseases receiving lifelong immunoglobulin replacement therapy. Qual Life Res. 2005;14:1683-1691.
81. Fasth A, Nystrom J. Quality of life and health-care resource utilization among children with primary immunodeficiency receiving home treatment with subcutaneous human immunoglobulin. J Clin Immunol. 2008;28:370-378.
82. Kittner JM, Grimbacher B, Wulff W, Jäger B, Schmidt RE. Patients' attitude to subcutaneous immunoglobulin substitution as home therapy. J Clin Immunol. 2006;26:400-405.
83. Hoffmann F, Grimbacher B, Thiel J, Peter HH, Belohradsky BH; Vivaglobin Study Group. Home-based subcutaneous immunoglobulin G replacement therapy under real-life conditions in children and adults with antibody deficiency. Eur J Med Res. 2010;15(6):238-245.
84. Fasth A, Nystrom J. Safety and efficacy of subcutaneous human immunoglobulin in children with primary immunodeficiency. Acta Paediatrica. 2007;96:1474-1478.
85. Gardulf A, Andersson E, Lindqvist M, et al. Rapid subcutaneous IgG replacement therapy at home for pregnant immunodeficient women. J Clin Immunol. 2001;21:150-154.
86. Sundin U, Nava S, Hammarstrom L. Induction of unresponsiveness against IgA in IgA-deficient patients on subcutaneous immunoglobulin infusion therapy. Clin Exp Immunol. 1998;112:341-346.
87. Koller H, Schroeter M, Feischen H, Hartung HP, Kieseier BC. Subcutaneous self-infusions of immunoglobulins as a potential therapeutic regimen in immune-mediated neuropathies. J Neurol. 2006;253(11):1505-1506.
88. Misbah S, Kuijpers T, van der Heijden J, Grimbacher B, Guzman D, Orange J. Bringing immunoglobulin knowledge up to date: how should we treat today? Clin Exp Immunol. 2011;166:16-25.
89. Knight E, Carne E, Novak B, et al. Self-administered hyaluronidase-facilitated subcutaneous immunoglobulin home therapy in a patient with primary immunodeficiency. J Clin Pathol. 2010;63:846-847.
90. Schiff RI, Wasserman R, Stein M, et al. Recombinant human hyaluronidase facilitates dispersion of subcutaneously administered gammagard liquid, enabling administration of full monthly dose in single site with improved bioavailability in immunodeficient patients [abstract]. Clin Exp Immunol. 2008;154(Suppl 1):121.
91. Melamed IR, Stein MR, Wasserman RL, et al. Recombinant human hyaluronidase facilitates dispersion of subcutaneously administered Gammagard liquid and enables administration of a full monthly dose in a single site to patients with immunodeficiency diseases [abstract]. J Allergy Clin Immunol. 2008;121(2 Suppl 1):S83.
92. Stein M, Wasserman RL, Melamed I, et al. Pharmacokinetics (PK) of human immunoglobulin 10% (IgG) administered intravenously (IGIV), subcutaneously (IGSC) or facilitated subcutaneously with recombinant human hyaluronidase (IGHy) in a subset of patients with Primary Immunodeficiency Disease (PIDD). J Allergy Clin Immunol. 2012;29:AB14.
93. Wasserman RL, Melamed I, Stein M, et al. Tolerability and efficacy of facilitated-subcutaneous infusion of immune globulin (Human), 10% and recombinant human hyaluronidase (IGHy) in a subset of study patients with Primary Immunodeficiency Disease (PIDD). J Allergy Clin Immunol. 2012;29:AB15.