Subcutaneous immunoglobulin replacement therapy with Hizentra®, the first 20% SCIG preparation: a Practical approach

Advances in Therapy, Jul 2011

To reduce the risk of infection in adults and children with primary immunodeficiencies, replacement therapy with IgG, which can be administered to patients intravenously or subcutaneously, is required. Although intravenous administration of IgG (IVIG) has been the therapy of choice in the US and widely used in Europe for many years, subcutaneous administration of IgG (SCIG) has recently gained considerable acceptance among patients and doctors. SCIG therapy achieves high and stable serum IgG levels, is well tolerated, and can be self-administered. Hizentra® (IgPro20; CSL Behring, Berne, Switzerland) is the first, ready-to-use 20% liquid preparation of human IgG specifically formulated for subcutaneous infusions. The high concentration (20%) might allow shorter infusion times due to smaller infusion volumes, with potential improvement in the convenience of SCIG therapy. Hizentra is well tolerated and has been shown to protect adult and pediatric primary immunodeficiency patients against serious bacterial infections. In addition, it is easy to handle and can be stored at a temperature up to 25°C. In summary, Hizentra is an advance in the field of immunoglobulin replacement therapy, which might offer benefits for home therapy patients.

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Subcutaneous immunoglobulin replacement therapy with Hizentra®, the first 20% SCIG preparation: a Practical approach

S. Jolles J. W. Sleasman 0 0 S. Jolles Department of Medical Biochemistry and Immunology, University Hospital of Wales , Cardiff, UK 1 ) Department of Pediatrics, University of South Florida To reduce the risk of infection in adults and children with primary immunodeficiencies, replacement therapy with IgG, which can be administered to patients intravenously or subcutaneously, is required. Although intravenous administration of IgG (IVIG) has been the therapy of choice in the US and widely used in Europe for many years, subcutaneous administration of IgG (SCIG) has recently gained considerable acceptance among patients and doctors. SCIG therapy achieves high and stable serum IgG levels, is well tolerated, and can be self-administered. Hizentra (IgPro20; CSL Behring, Berne, Switzerland) is the first, ready-to-use 20% liquid preparation of human IgG specifically formulated for subcutaneous infusions. The high concentration (20%) might allow shorter infusion times due to smaller infusion volumes, with potential improvement in the convenience of SCIG therapy. Hizentra is well tolerated and has been shown to protect adult and pediatric primary immunodeficiency patients against serious bacterial infections. In addition, it is easy to handle and can be stored at a temperature up to 25C. In summary, Hizentra is an advance in the field of immunoglobulin replacement therapy, which might offer benefits for home therapy patients. - Patients with primary immunodeficiency (PI) disorders, such as common variable i m m u n o d e f i c i e n c y ( C V I D ) , X - l i n k e d agammaglobulinemia (XLA), and autosomal recessive agammaglobulinemia (ARAG) that are caused by B-cell dysfunction are prone to recurrent bacterial infections.1,2 Lifelong immunoglobulin (Ig) replacement therapy is the only effective treatment for these patients, and is thus the gold standard in the management of primary antibody deficiency.3 IgG can be administered subcutaneously (SCIG) or intravenously (IVIG) and was first used by Bruton in a child with agammaglobulinemia in 1952.3 SCIG preparations were introduced in the 1980s in the US and Europe. However, the slow infusion technique and the low concentration of the preparations available at the time made SCIG impractical and less attractive to patients and healthcare professionals. Therefore, IVIG, which allowed infusions of higher monthly doses, became the preferred route of administration. Despite its success, IVIG may not be suited to all patients, especially those with poor venous access. IVIG may be associated with systemic adverse events (AEs) and IVIG self-administration is technically more demanding and requires more training than SCIG self-administration. With recent technical advances in IgG formulation, pure and highly concentrated SCIG preparations that have relatively low viscosity, and can therefore be infused relatively rapidly, have been developed and are increasingly used worldwide.4-6 Usage of SCIG therapy varies greatly across countries and is predominant in Sweden, Germany, and the UK (Figure 1). While IVIG is infused every 3-4 weeks, SCIG is typically administered once a week, with the total IVIG monthly dose divided in smaller portions. The distribution of IgG between the vascular and extravascular compartments after subcutaneous administration can be described by a twocompartment model, which accurately describes the distribution of serum IgG in healthy individuals and PI patients (Figure 2).7 In this model, IgG enters the vascular compartment (blood) from the extravascular compartment (subcutaneous space) via the lymphatics at a defined rate, which integrates IgG catabolism and total IgG distribution. This model predicts a progressive release of IgG into the circulation that contributes to the more stable serum IgG levels achieved with SCIG. In comparison to IVIG, SCIG results in more sustained serum IgG levels, avoiding the peaks and troughs associated with IVIG.6,8 SCIG is associated with fewer systemic AEs than IVIG and requires no venous access.4,6,8 Finally, SCIG is easy to use and is easier to self-administer, providing patients with flexibility and improved quality of life.9 Patients treated with SCIG do not need to go to the hospital or infusion centers, avoiding unnecessary travel and their potential concerns for acquiring nosocomial infections. Patients require less assistance from healthcare professionals, reducing the cost associated with Ig replacement therapy, and can take greater control over their therapy. This review summarizes the available data on and practical considerations regarding the use of the subcutaneous 20% IgG preparation, IgPro20 (CSL Behring, Berne, Switzerland), currently marketed in the US under the brand name of Hizentra. Hizentra has a good safety profile and has been shown to effectively protect PI patients from serious and non-serious bacterial infections.10,11 INTRODUCING HIZENTRA Hizentra is a 20% (200 g/L) ready-to-use liquid pr (...truncated)


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S. Jolles, J. W. Sleasman. Subcutaneous immunoglobulin replacement therapy with Hizentra®, the first 20% SCIG preparation: a Practical approach, Advances in Therapy, 2011, pp. 521-533, Volume 28, Issue 7, DOI: 10.1007/s12325-011-0036-y