Selective antibody deficiency with normal immunoglobulins (Ig) is characterized by deficient specific antibody response to polysaccharide antigens but not to protein antigens, despite normal or near normal serum levels of immunoglobulins, including IgG subclasses.
(See also Overview of Immunodeficiency Disorders and Approach to the Patient With an Immunodeficiency Disorder).
Selective antibody deficiency with normal immunoglobulins (SADNI) is a primary immunodeficiency disorder. It is one of the most common immunodeficiencies that manifests with recurrent sinopulmonary infections. Selective antibody deficiencies can occur in other disorders, but SADNI is a primary disorder in which deficient response to polysaccharide antigens is the only abnormality (see table Humoral Immunity Deficiencies). The inheritance and pathophysiology have not been elucidated, but some evidence suggests that the cause may be inherited molecular abnormalities.
A subset of patients with SADNI initially have an appropriate response to polysaccharide antigen but lose antibody titers within 6 to 8 months (called SADNI memory phenotype).
Patients have recurrent sinopulmonary infections and sometimes manifestations that suggest atopy (eg, chronic rhinitis, atopic dermatitis, asthma). Severity of the disorder varies.
Young children may have a form of SADNI that resolves spontaneously over time.
Diagnosis of SADNI
Usually normal immunoglobulin levels (IgG, IgA, IgM, and IgG subclasses)
Deficient or absent response to polysaccharide vaccines
Haemophilus influenzae type b).
A normal response to PPV for children age 2 to 5 years is defined as protective antibodies to ≥ 50% of the serotypes tested, with at least a 2-fold increase in the titers; patients 6 to 65 years should have protective antibodies to ≥ 70% of serotypes. Patients with abnormal results can be classified phenotypically as follows (1):
Memory phenotype: Normal initial response to PPV23, which is lost within 6 months.
Mild phenotype: Multiple serotypes (> 50% for children 2 to 5 years or > 30% for patients 6 to 65 years) without protective titers or without a 2-fold increase in titers
Moderate phenotype: Protective titers to ≥ 3 serotypes but fewer than the expected number for their age
Severe phenotype: Protective titers to ≤ 2 serotypes, and the titer, if present, tends to be low (< 1.3–2.0 microg/mL [< 1.3–2.0 mg/L]).
Family members of known patients do not need screened unless they manifest a similar clinical picture.
Diagnosis reference
1. Orange JS, Ballow M, Stiehm ER, et al: Use and interpretation of diagnostic vaccination in primary immunodeficiency: a working group report of the Basic and Clinical Immunology Interest Section of the American Academy of Allergy, Asthma & Immunology. J Allergy Clin Immunol 130 (3 Suppl):S1–S24, 2012. doi:10.1016/j.jaci.2012.07.002
Treatment of SADNI
Pneumococcal conjugate vaccine
Sometimes prophylactic antibiotics and sometimes immune globulin replacement therapy
Patients should be vaccinated with the pneumococcal conjugate vaccine.
Rarely, when infections recur frequently despite prophylactic antibiotics, immune globulin replacement therapy can be given.
