Background
Primary antibody deficiencies (PAD) constitute the majority of all
primary immunodeficiency diseases (PID) also termed inborn errors of
immunity (IEI). This category (PAD) represents around 52% of all IEI
and the proportion overall is still greater given that antibody
deficiency is a component of other groups including combined
immunodeficiencies (CID and SCID), autoinflammatory disorders, diseases
of immune dysregulation and other well defined PIDs (1,2). Secondary
antibody deficiencies (SAD) represent a larger and expanding number of
individuals resulting from the use of a wide range of immunosuppressive
therapies, in particular those targeting B cells, and may also result
from renal or gastrointestinal immunoglobulin losses, infections (HIV,
malaria) and globally malnutrition (3).
The manifestations of PADs are protean and encompass a range of
infectious and non-infectious complications, including autoimmune,
lymphoproliferative, granulomatous and allergy manifestations due to an
immunedysregulation, and increased risk of malignancy in some forms of
PADs (4,5).