Table 4 Immunological tests additional to baseline tests in patients with suspected PID
From: Primary immunodeficiency diseases in lung disease: warning signs, diagnosis and management
Baseline level | First levela | Second levelb | Third levelb | N (Composition of panel) | Votes in agreement (%) | Degree of agreement |
Combined | ||||||
Complete blood count IgA, IgG, IgM and IgE levels Biochemical analysise | Lymphocyte populations | Extended phenotype, lymphocyte function | Protein expression Functional and genetic studies | 34 (AI, AP) | 91.2 | Consensus |
PID-associated syndromes | ||||||
Complete blood count IgA, IgG, IgM and IgE levels Biochemical analysise | Karyotype ± CGH Array | Studies according to specific suspicion | Protein expression Functional and genetic studies | 33* (AI, AP) | 87.9 | Consensus |
Antibody production deficiencyc | ||||||
Complete blood count IgA, IgG, IgM and IgE levels Biochemical analysise | Basic antibody production study (ASLO, hemaglutinins and tetanus) | IgG subclasses Response to Salmonella typhi or pneumococcus Response to tetanus toxoidd and H. influenzae (optional) Lymphocyte populations with extended phenotype B | Protein expression Functional and genetic studies | 34 (AI, AP) | 91.2 | Consensus |
Immune deregulation | ||||||
Complete blood count IgA, IgG, IgM and IgE levels Biochemical analysise | Autoantibody panel (ANA and NOSAB, anti-neutrophils) Targeted hormone study Coombs test Vitamin B12 with neural tube defects Soluble FAS ligand Ferritin Triglycerides Fibrinogen Calcium and phosphorus | Treg Soluble CD25 FoxP3 | Protein expression Functional and genetic studies | 33* (AI, AP) | 97.0 | Consensus |
Phagocyte deficienciesd | ||||||
Complete blood count IgA, IgG, IgM and IgE levels Biochemical analysise | Oxidation test (DHR) Basic lymphocyte subpopulations | CD18/11b | – | 34 (AI, AP) | 91.2 | Consensus |
Innate immunity disorder | ||||||
Complete blood count IgA, IgG, IgM and IgE levels Biochemical analysise | Studies according to clinical suspicion | – | Protein expression Functional and genetic studies | 34 (AI, AP) | 97.1 | Consensus |
Complement deficiency | ||||||
Complete blood count IgA, IgG, IgM and IgE levels Biochemical analysise | CH50, C3, C4, autoimmunity studies | AP50 Factor quantification | Protein expression Functional and genetic studies | 34 (AI, AP) | 94.1 | Consensus |
Autoinflammatory diseasesd | ||||||
Complete blood count IgA, IgG, IgM and IgE levels Biochemical analysise | Inflammatory markers CRP ESR | SAA | Genetic studies | 33* (AI, AP) | 84.8 | Consensus |