1. PRIMARY & SECONDARY
ANTIBODY DEFICIENCY

2. ANTIBODIES & IMMUNOGLOBULINS

3. PRIMARY ANTIBODY DEFICIENCY
The European internet-based patient and research database for primary immunodeficiencies: results
Gathman et al., Clin Exp Immunol (2009); 157 Suppl 1: 3-11.

5. Brit Med J (1989); 298: 516-7

6. THERAPEUTIC IMMUNOGLOBULIN
1970s - IMIg
1980s - IVIg
1990s - IVIg, SCIg
2000s - product safety
- infusion rates / concentration
- immunoglobulin retrieval

7. REPLACEMENT THERAPY

8. TREATMENT OUTCOMES
Wood et al. Clin Exp Immunol (2007); 149:

9. EFFICACY & ADVERSITY Immunoglobulin Excipients Soluble CD4/ CD8/ HLA
Cytokines
Clin Exp Immunol (2004); 136: 111-3

10. IVIg & SCIg
ESID Register 2009

11. HOME THERAPY

12. 2008 and 2011

13. SAME OLD SAME OLD Core of PID management No alternatives
Lifelong requirement (usually)
Effective
(bacterial infection, antibiotic usage, QoL, hospitalisation, life expectancy)
Dose requirement  in:
- frequent breakthrough infections
- chronic inflammation / tissue damage
- poor prognosis disease variants

14. WHAT’S NEW? The three Rs: Reorganisation Reclassification Aarrrgh
- ongoing uncertainties over dosing / target levels

15. DOSE?
Impact of trough IgG on pneumonia incidence in primary immunodeficiency: A meta-analysis of clinical studies.
Orange JS et al. Clinical Immunology (2010); 137: 21-30

16. DOSE: INDIVIDUALISATION
‘The goal of replacement therapy should be to improve clinical
outcome and not to reach a particular IgG trough level.’
J Allergy Clin Immunol (2010);125:

17. DOSE: INDIVIDUALISATION
‘….individualizing the dosage….is preferable to using mean pharmacokinetic
parameters.’
Clin Immunol (2011);139:133-41

18. RECLASSIFICATION Specific Antibody Deficiency  Kawasaki Disease
 ‘Other’ Section

19. REORGANISATION

20. PRIMARY ANTIBODY DEFICIENCY DISORDERS

21. SPECIFIC DISORDERS Thymoma with immunodeficiency (Good’s Syndrome)
Combined immunodeficiencies requiring
haemopoietic stem cell transplantation
(HSCT)
Specific antibody deficiency
(SAD)
Transient hypogammaglobulinaemia of infancy
(THI)

22. RECOMMENDATION / REQUIREMENT
SPECIFIC DISORDERS
DISORDER
RECOMMENDATION / REQUIREMENT
GOOD’S
Profound B cell depletion / significant antibody deficiency
HSCT
Duration based on B cell reconstitution post-transplantation
SAD
Robust application of selection criteria
THI
Define planned duration of therapy prior to initiation
(GRADE C, LEVEL III)

23. SUMMARY: PID

24. SECONDARY ANTIBODY DEFICIENCY
PRIMARY
SECONDARY
Malignant disease
Drugs
Protein-losing states
Infection (cause & effect)
Systemic disease
Iatrogenic causes
Chromosomal abnormalities

25. WHAT’S NEW? Secondary Antibody Deficiency 
Revision / collation into a single indication
+ review outcomes (infection / hospitalisation)
+ dosing (minimum IgG trough 6 g/L)

26. RECOMMENDATIONS Irreversible hypo-
Hypo- associated with CLL/NHL/MM etc.
and

27. GUIDELINES Evidence-based use Consistency of care
‘Systematically developed statements to assist practitioner and patient decisions about appropriate health care for specific clinical circumstances’
Evidence-based use
Consistency of care
Access to safe, high quality products
Security of supply
Utilising scarce resource

29. OUTCOMES COMPLICATIONS PROGRESSION OF COMPLICATIONS QUALITY OF LIFE
WORKING CAPACITY
LIFE EXPECTANCY
OPTIMISED GROWTH / DEVELOPMENT