Table 2.
Clinical complications of primary antibody deficiency
| Clinical complications | Predominantly associated with | Occurrencea |
|---|---|---|
| Recurrent URTI | All PAD | Very common |
| Recurrent ENT infections | All PAD | Very common |
| Recurrent severe pneumonia | Cong. agamma., CSR def., CVID | Very common |
| Bronchiectasis | Cong. agamma., CSR def., CVID | Common |
| Interstitial pulmonary abnormalities | CVID | Rare |
| Sepsis | Cong. agamma., CSR def., CVID | Rare |
| Bacterial osteomyelitis/arthritis | Cong. agamma., CSR def., CVID | Rare |
| Bacterial meningitis | Cong. agamma., CSR def., CVID | Rare |
| Chronic enteroviral meningoencephalitis | Cong.agamma., CD40L def | Rare |
| Recurrent giardiasis | All PAD | Common |
| Campylobacter gastroenteritis | All PAD | Rare |
| Nodular lymphoid hyperplasia | CVID | Rare |
| Inflammatory bowel disease | AID, CVID | Rare |
| Enteropathy (chronic diarrhoea of origin) | CVID, cong. agamma, sIgAD, AID | Common unknown |
| Autoimmune disease | CVID, sIgAD, AID | Common |
| Allergic disorders | CVID, sIgAD | Common |
| Granulomas (lung, bowel, other) | CVID | Rare |
| (Hepato)splenomegaly | CVID | Common |
| Lymphoid hyperplasia | CVID, AID | Common |
| Malignancies, Mainly lymphoma | CVID | Rare |
| Neutropenia | XLA, CD40L | Common |
URTI upper respiratory tract infection, BTK Bruton’s tyrosine kinase, AID activation-induced cytidine deaminase, PAD primary antibody deficiencies, ENT cong. agamma congenital agammaglobulinemias ears, nose, throat, XLA X-linked agammaglobulinemia, CVID common variable immunodeficiency, sIgAD selective IgA deficiency
aEstimated occurrency rate; rare (<10%), common (10–50%), very common (>50%)