Abstract
Primary antibody deficiencies (PADs) are the most common types of primary immunodeficiency diseases (PIDs), accounting for approximately half of the diseases. The spectrum of PADs is broad, ranging from patients with a severe reduction of all serum immunoglobulin classes (Ig) and totally absent B cells to patients who have a selective antibody deficiency with normal serum immunoglobulin. Many of these disorders share a clinical phenotype with common features such as chronic and recurrent infections, chronic inflammation, and autoimmunity. Hypogammaglobulinemia is the major hallmark of patients with PADs, and the main manifestation is recurrent bacterial infections, predominantly occurring in the respiratory and gastrointestinal traces. (See Table 1.2 and Fig. 1.9 for updated classification of predominantly antibody deficiencies)
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