Table III.
Warning signs of PIDD for infectious disease specialists
| Clinical occurrences | PIDD | Lab screening tests |
|---|---|---|
| Infections from extracellular bacteria | Antibody deficiencies | AMI |
| Complement deficiencies | C, ANA | |
| Neutropenias | P | |
| IRAK-4, MyD88 | II, RCP | |
| Infections due to Neisseria meningitidis | Complement deficiency of terminal components (Membrane attack complex) | C + AP50 |
| Infection from S aureus, and gram-negative bacterias: Serratia marcescens, Burkholderia cepacia and gladioli,Nocardia spp, Chromobacterium violaceum, Granulobacter bethesdensis. | Chronic granulomatous disease (CGD) | P |
|
Hyper IgE syndrome (HIES) Features: pneumonia from S aureus, eczema, fungal infection, joint hypermobility, coarse facial features |
Serum IgE, eosinophilia Specific Scorea |
|
| Infection from fungi: Pneumocystis jiroveci; Aspergillus and Candida albicans. | T cell defects | CMI |
| CD40 ligand (L) deficiency | AMI | |
| HIES |
Serum IgE, eosinophilia Specific Scorea |
|
| CGD | P | |
| Infection from Candida albicans | Chronic mucocutaneous candidiasis | CMI + T lymphocyte proliferation induced by Candida |
| Infection by atypical Mycobacteria/Salmonella and/or Bacillus Calmette-Guérin side effects; Paracoccidioides sp, Leishmania, Cryptococcus | T cell deficiencies | CMI |
| Severe combined immunodeficiency (SCID) | AMI + CMI | |
| Mendelian susceptibility to mycobacterial diseases | P and/or II | |
| Infections from Herpes | T and NK cell deficiencies | CMI |
| Fulminant or chronic infection by Epstein-Barr virus |
Familial hemophagocytic lymphohistiocytosis (FHL) syndrome X-linked lymphoproliferative (XLP) syndromes, types 1 or 2 |
CBC, triglycerides, ferritin, serology EBNA |
| Recurrent or persistent Cryptosporidium, Isospora | CD40L deficiency | AMI |
| Common variable immunodeficiency (CVID) | AMI | |
| Giardiasis | Antibody deficiencies | AMI |
| Complications due to BCG, rotavirus or Varicella vaccines | SCID, CGD | CMI and/or II and/or P |
| Complications due to oral polio vaccine | Antibody deficiencies | AMI |
| Persistent fever of unknown origin | Autoinflammatory diseases | ANA, RCP, blood smear |
Abbreviations: AMI antibody mediated immunity, ANA antinuclear antibodies, CBC complete blood count, CMI cellular mediated immunity, EBNA Epstein-Barr nuclear antigen, II innate immunity, P phagocytosis, RCP reactive C protein
aScore for classical hyper IgE diagnosis [26]