Immunoglobulin treatment in primary antibody deficiency

Abstract

The primary antibody deficiency syndromes are characterised by recurrent respiratory tract infections and the inability to produce effective immunoglobulin (Ig) responses. The best known primary antibody deficiencies are common variable immunodeficiency (CVID), X-linked agammaglobulinaemia (XLA), immunoglobulin G (IgG) subclass deficiency, and selective antibody deficiency with normal immunoglobulins (SADNI).Therapy in these patients consists of prophylactic antibiotics and/or Ig replacement therapy. Diagnostic delay remains common owing to limited awareness of the presenting features and may result in increased morbidity and mortality. Replacement therapy with immunoglobulins increases life expectancy and reduces the frequency and severity of infections, but the effect on end-organ damage is still unknown. Both intravenous immunoglobulin (IVIg) and subcutaneous immunoglobulin (SCIg) treatment appear to be safe, with comparable efficacy. A starting dose of 300-400 mg/kg/month in IVIg and 100 mg/week for SCIg is recommended. IgG trough levels should be >5 g/L for patients with agammaglobulinaemia and 3 g/L greater than the initial IgG level for patients with CVID; however, the clinical response should be foremost in choosing the dose and trough level. Infusion-related adverse reactions are generally mild owing to improved manufacturing processes. In this paper, aspects of Ig replacement therapy in primary antibody-deficient patients will be addressed.

Home Care Use of Intravenous and Subcutaneous Immunoglobulin for Primary Immunodeficiency in the United States

“…The prescribed doses, calculated with the number of grams of Ig ordered, the patient's weight, and the frequency of administration, were generally in accordance with the recommended dose of 400 to 600 mg/kg/month [8,[14][15][16][17]. The most common frequency of IV Ig dosing was every 4 weeks, consistent with the survey by Yong et al, which reported that 87 % of surveyed allergist/immunologists initially prescribed this frequency [18].…”

“…The intravenous (IV) and subcutaneous (SQ) routes are both effective methods of administration of Ig for PIDD [6][7][8]. While earlier reports suggested that lower doses were satisfactory, the current trend is to give increased amounts, with attention paid to trough IgG levels as well as the health of the patient [4,5,9].…”

Biopharmaceutical Products from Animal Cell Culture

“…What has also been observed is that serum half-life can vary greatly with humanized and fully human antibody drugs compared to natural antibodies such as IgG which has a mean half-life of 25-32 days (Maarschalk-Ellerbroek et al 2011). These engineered therapeutic antibodies have a serum half-life that varies greatly from a low of 7.5 days to a range similar to natural antibodies (Yoon et al 2010).…”

Alzheimer’s Disease of the Immune System: A New Variant of Immune Deficiency

“…This complement is also involved in the adaptive immune response and is considered to be a functional bridge between the innate and adaptive responses, making it an essential and efficient player in immune homeostasis and acquired immunity. Complement proteins form a trio of intersecting enzyme cascades that result in a number of protective mechanisms that bind to foreign surfaces and, thus, fight viruses, bacteria, and other foreign substances [5]. Once the complement cascade has been initiated, there are mechanisms that inhibit or slow down the process reaction at various steps.…”