Fawzia Abo Ali discusses the relationship between primary immunodeficiencies (PIDs) and allergies. She notes that allergies are sometimes an early warning sign of an underlying PID. PIDs like selective IgA deficiency, hyper IgE syndrome, common variable immunodeficiency, and severe combined immunodeficiency have been associated with higher rates of allergies like asthma and atopic dermatitis. A bias toward TH2 immune responses can also occur in PIDs like CVID. She stresses that PIDs should be considered in patients with severe, treatment-resistant, or infection-prone allergies. Early immunoglobulin testing is important to diagnose PIDs before organ

1. Fawzia Abo Ali
Prof. of Allergy&Immunology
Ain Shams Faculty of Medicine

2. Innate immunity adaptive immunity

4. Types of immunodeficiencyTypes of immunodeficiency
Primary
 inherited
• phagocytic cells
• complement system
• T-cells
• B-cells
• combined
.
Secondary
 Acquired
• AIDS
• Chemotherapy
• Malignancy
• Diabetes
• Steroids
• Splenectomy
• Autoimmune diseases

5. Common variable
immunodeficiency.
Selective

8. • Allergy is an important expression of misdirected
immunity, and certain PIDDs are frequently
associated with atopy.
• Allergies are sometimes a warning sign that leads
to a diagnosis of PID.
• The possibility of a PID in certain patients with
atopy should be considered if:
•
1. severe
2. does not respond to usual
treatment
3. susceptibility to infections.
4. Autoimmune phenomena

9. Epidemiology
• 1 in 140 (Middle East)
• 1 in 600 (European)
• 1 in 60,000
(Japanese).
• Sex:
• men >women
• Age:
• any age.
Arabian peninsula 1-142
persons
Spain : 1-170 persons
Eastern Nigeria: 1-255 persons
Finland : 1-396 persons
Erkoçoğlu M, Metin A, Kaya A, Özcan C, Akan A, Civelek E,
Çapanoğlu M, Giniş T, Kocabaş CN. Turk J Med Sci. 2017
Apr 18; 47(2):592-598. Epub 2017 Apr 18.

10. • SAD is a common PID that manifests as
recurrent respiratory infections .
• It protects all the mucus membrane.
• It prevent the absorption of allergens into the
bloodstream, so ,deficiency leads to mucosal
antigen exposure .
• It has been associated with asthma, allergic
rhinitis, atopic dermatitis (AD), and food allergy
(FA) in 50% of the patients.

11. Allergic diseases in patients with IgA
deficienc

12. • It was noted that severity of allergic diseases is
correlated to low IgA concentration in serum than a
concentration of IgE
• IgE concentrations may increased in selective IgA
deficiency, which may be due to a compensatory
mechanism for low secretory IgA level .
• Severe anaphylactoid transfusion reactions are seen in
patients with SIgAD

14. • In (sIgMD) ,recurrent
respiratory tract infections
are the predominant clinical
feature. Allergic diseases
and asthma coexist with
this immune deficiency in
7-8% of affected children
and as much as 33% of
adult patients

15. Common variable immunodeficiency (CVID)Common variable immunodeficiency (CVID)
• (CVID) is a heterogeneous disorder leading to
defective immunoglobulin production>2.
• Recurrent sinopulmonary infections-
autoimmunity-allergy.
• Age of diagnosis 2-40 years of age.
• Unfortunately, late diagnosis is characteristic of
this immunodeficiency. It was reported that a
case of CVID was treated as allergic respiratory
disease for 40 years before the diagnosis of
immunodeficiency was made .

16. • A bias towards Th2-mediated immune response
was also documented in patients with (CVID)
• A high incidence of asthma, usually diagnosed
after the initial presentation, was seen in 83%
pediatric patients with CVID.10% of them have
had asthma and allergic rhinitis without antigen-
specific IgE.
• But they presented a positive response to
bronchial provocation tests with allergens
• Celestin J, Welker D, Edwards TB, Bonilla F. Undiagnosed
panhypogammaglobulinemia masquerading as atopy. J Allergy Clin Immunol
1999;103 (1 pt 2):S146

18. Hyper IgE syndrome

19. Pathogenesis of allergy in PID patients:Pathogenesis of allergy in PID patients:
1.Dysregulation of the immune response mainly due to:
Regulatory T cells:
o Defeciency
o Anergy
o Decreased activity
resulting in predominate Th2-dependent immune response.
2.Activation of CD4+ T cells plays a key role and allergic
inflammation .
3.defective antigen elimination

20. HIV

21. Allergy in HIV patients:
• People with HIV infection are known to have high
levels of allergic antibody (IgE), especially as the
CD4+ T-cell levels drop.
• These high IgE levels are not likely to be indicative of
worsening allergies, however, but are likely a sign of
worsening immunodeficiency due to B cell dysfunction.
• The IgE antibodies are directed against
various pathogens (including HIV), rather than against
allergens.
• Treating asthma in HIV-infected people is similar to
that in people with HIV infection, although oral
corticosteroids should be avoided whenever possible,
due to their suppressive effect on the immune system.

22. Drug Allergy in People with HIV
• People with HIV infection have higher
rates of drug allergy reactions.
• This is particularly true for trimethoprim-
sulfamethoxazole, desensitization for TMP-SMX
allergy is frequently successful, which is often
required to treat opportunities infections in people
with HIV.
• Abacavir antiviral drug also causes a life-
threatening hypersensitivity reaction in 5-8% of
HIV-infected people.

23. Saeed Al Qemzi with his son
Khalifa Al Qemzi , who was
affected with SCID. The child is
living a normal life now, after
going through gene therapy.
David Vitter Khalifa Alqemzy

24. Home message:
 Primary Immunodeficiency
Masquerading as Allergic Disease
 Allergies are sometimes a warning
sign that leads to a diagnosis of PID.
 If allergy is associated with recurrent
infections or autoimmunity ,immune
deficiency is suspected .
 The immune deficiencies and allergy
have mutual impact ,should be
considered as a specific overlapping
syndrome.

25.  In patients with allergy, recognition of immunodeficiency
is important because Immune suppression with systemic
corticosteroids in these immune compromised
individuals can lead to life threatening infections
 Both the medical and general communities need to be
aware of Primary Immune deficiencies.
 Early check for serum immunoglobulins in suspected
patients is crucial to diagnose PIDs early before the
occurrence of significant organ damage .

26. References:
• Celestin J, Welker D, Edwards TB,Bonilla F. Undiagnosed
panhypogammaglobulinemia masquerading as atopy. J Allergy Clin
Immunol 1999;103 (1 pt 2):S146.
• N. Kutukculer, N. E. Karaca, O. Demircioglu, and G. Aksu, “Increases in serum
immunoglobulins to age-related normal levels in children with IgA and/or IgG
subclass deficiency,” Pediatric Allergy and Immunology, vol. 18, no. 2, pp. 167–173,
2007.
• A. Aghamohammadi, T. Cheraghi, M. Gharagozlou et al., “IgA deficiency: correlation
between clinical and immunological phenotypes,” Journal of Clinical Immunology, vol.
29, no. 1, pp. 130–136, 2009.
• Aleksandra Szczawinska-Poplonyk, “An Overlapping Syndrome of Allergy and
Immune Deficiency in Children,” Journal of Allergy, vol. 2012, Article ID 658279, 9
pages, 2012.
• Tuano KS, Orange JS, Sullivan K, Cunningham-Rundles C, Bonilla FA, Davis CM.
Food allergy in patients with primary immunodeficiency diseases: prevalence within
the US Immunodeficiency Network (USIDNET). J Allergy Clin Immunol.
2014;135(1):273-5.
• Bjelac, Jaclyn A. et al.Allergic disease in patients with common variable
immunodeficiency at a tertiary care referral center
• Annals of Allergy, Asthma & Immunology , Volume 120 , Issue 1 , 90 - 92

27. Thank
you