Presenting features of antibody deficiency

Nearly all patients with severe PAD present with infection, although in a minority the hypogammaglobulinaemia is discovered during investigation of autoimmune or inflammatory bowel disease (Figure 22.3). There is usually a gradual escalation of bacterial infection in the respiratory tract, with recurrent bronchitis, persistent nasal catarrh and sinusitis, and otitis media in children. The organisms most commonly isolated are non-typeable Haemophilus influenzae, pneumococci and Moraxella catarrhalis.

A few patients present with an acute, life-threatening septicaemia, sometimes with meningitis caused by pneumococci, capsulated H. influenzae and occasionally meningococci. A minority are susceptible to chronic enteroviral infection, particularly with echoviruses, that cause an insidious meningo-encephalitis and sometimes a dermatomyositic-like condition and/or myocarditis. Immunization with live attenuated vaccines is contraindicated, particularly with oral polio vaccine (OPV), because some patients have subsequently developed paralytic poliomyelitis. About 10% of patients are prone to chronic mycoplasma infection, usually manifesting as arthritis or ureth-ritis, but sometimes as deep abscesses in the lungs or elsewhere. As only a minority of patients are prone to enteroviral or mycoplasmal infection, it is likely that they lack additional protective factors for these specific organisms. In the bowel, campylobacter or giardia infection is common and often difficult to eradicate with standard therapy.

Individuals with IgAD are usually healthy and need take no special precautions with vaccines. However, they often make anti-IgA antibodies, which in a small minority are of sufficiently high titre and affinity to cause anaphylactic reactions during blood or blood product infusion. There is no consensus among immunologists on whether IgAD individuals should be routinely monitored for anti-IgA antibodies, mainly because there is very poor correlation between the level of these antibodies and severe reactions.

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