- Ahmed M Ebrahim, an 8-year-old boy, presents with cough, wheezing, dyspnea and has a history of asthma and allergic rhinitis since age 3. He has recurrent swelling behind the ears diagnosed as parotid. - Examination finds depressed maxillary bones, atrophied nares, oral breathing, and vesicular breathing with rhonchi. Tests show normal CBC. - The patient likely has Selective IgA Deficiency based on his clinical history of recurrent infections, asthma, and parotid swelling. This is the most common primary immune deficiency where patients have low or absent IgA levels.

1. Fawzeia Abo Ali
Prof. of Internal Medicine & Immunology
Ain Shams Faculty of Medicine

2. • Ahmed M Ebrahim, is an 8-year-old boy presenting
to the allergy clinic complaining of cough,
wheezing and dyspnea. This was accompanied With
frequent sneezing & nasal discharge.
• His mother reports he is a known case of asthma &
allergic rhinitis since the age of 3 years.

3. • He received intermittent medications, in the form of
inhaled steroids, intranasal steroids irrigularly
together with montelukast, but attacks recure
frequently mostly with fever.
• The mother added that during the course of the
disease ,the boy has recurrent painful swelling
behind the ear ,diagnosed as parotid. The swelling
sometimes resolves spontaneously , but recurs again.

4. • Other medical history or family history :-ve
• O/E: pt is of average built and g condition was fair
• Depressed maxillary bones atrophied naries and oral
breathing
• Parotids are normal
• Chest: harsh vesicular breathing sonorous & sibilant
rhonchi allover.
• Other examination was unremarkable

5. • Cbc: normal

9. Selective IgA Deficiency
It is the most common primary immune deficiency
disease.
Patients have low or absent immunoglobulin A (IgA)
( with normal level of other immunoglobulins ,About 20-30% of
cases have IgG subclasses deficiency)
IgA protects against infections of the mucous
membranes lining the mouth, airways and digestive
tract.

12. Epidemiology
• Prevalence:
• Sex:
• greater frequency
of SIgAD in men
than in women
• Age:
• can be diagnosed
in persons of any
age.
Arabian peninsula 1-142 persons
Spain : 1-170 persons
Eastern Nigeria: 1-255 persons
Finland : 1-396 persons

13. Clinical Features of Selective IgA Deficiency:
1.susceptibility to infections.
2.autoimmune diseases: 25% to 33% (CD,IDDM)
3. allergy & asthma : 15% to 80%, also food allergy .
4. severe reactions after receiving blood products
5.Apparently healthy .

14. Coincidence of the infection diseases and allergy
with autoimmunity in IgA deficiency 37 patients

15. Selective IgA Deficiency & asthma
Allergies (including asthma, and food allergies) are
common presentation of Selective IgA Deficiency,
(15% to 83%).
epidemiological studies point at a reverse
association between IgA levels and the incidence of
allergic airway disease
Atopics may have insufficient amounts of IgA, and
so may be prone to development of allergic
diseases.

16. • Anti allergic effect of IgA
IgA is classically known for neutralizing toxins and
bacteria (viruses) & allergens at mucosal surfaces ,by
interfering with their motility, by competing for
epithelial adhesion sites, and improving the
properties of the airway secretions .
It is involved in “trapping” and removal of the
antigen (“immune exclusion”)

17. It facilitates phagocytosis in mucosal areas .
it has been reported that IgA may play an important role in
the context of the so-called “hygiene hypothesis,” Low
microbial pressure might cause the IgA system to mature
more slowly
(Serum) IgA ligation on monocytes also induces IL-10
expression and inhibits inflammatory cytokine .
And prevent hyperinflammatory responses towards
environmental allergens that cause allergic asthma.
in healthy individuals, allergen-specific IgA and IgG4 molecules
are found to be essential to suppress responses to allergens.

18. Diagnosis of Selective IgA Deficiency:
• The diagnosis of Selective IgA Deficiency is usually
suspected because of chronic or recurrent infections, ashma
autoimmune diseases, or chronic diarrhea. (Age>4 years)
1. Low levels of IgA (reported usually as < 7
mg/dL)
2. normal levels of other immunoglobulins (IgG
and IgM).
3. Occasionally, low levels of IgG2 and/or IgG4

19. Treatment of Selective IgA Deficiency
It is not currently possible to replace IgA as it does not
remain in the circulation for very long.
Treatment should be directed toward the particular
problem i.e recurrent infections, asthma ,allergy or
autoimmune disease.
If a patient has many infections, also have IgG2 and/or
IgG4 subclass a trial of immunoglobulin replacement
therapy may be considered.
Pt. is at risk of anaphylaxis when receiving blood
products.
Check for CD.

20. Vaccination and IgA deficiency:
live virus vaccines are contraindicated
Flu & pneumococcal vaccine, are specifically
recommended.
The most important aspect of therapy in IgA
deficiency is close communication between the
patient (and/or the patient’s family) and the
physician so that problems can be recognized and
treated as soon as they arise.

21. References:
• Saghafi S, Pourpak Z, Aghamohammadi A, Pourfathollah AA, Samadian A, Farghadan M, et
al. Selective immunoglobulin A deficiency in Iranian blood donors: Prevalence, laboratory
and clinical findings. Iran J Allergy Asthma Immunol. 2008; 7: 157-162.
• Aghamohammadi A, Mohammadinejad P, Abolhassani H, Mirminachi B, Movahedi M,
Gharagozlou M, et al. Primary immunodeficiency disorders in Iran: update and new
insights from the third report of the national registry. J Clin Immunol. 2014; 34: 478-490
• . Özcan C, Metin A, Erkoçoglu M, Kocabas CN. Bronchial hyperreactivity in children with
antibody deficiencies. Allergol Immunopathol (Madr). 2015; 43: 57-61.
• Özcan C, Metin A, Erkoçoglu M, Kocabas CN. Allergic diseases in children with primary
immunodeficiencies. Turk J Pediatr. 2014; 56: 41-47.
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obstructive pulmonary disease: a role for immunoglobulin A?. Proc Am Thorac Soc. 2004;
1: 125-135.
• Aghamohammadi A, Mohammadi J, Parvaneh N, Rezaei N, Moin M, Espanol T, et al.
Progression of selective IgA deficiency to common variable immunodeficiency. Int Arch
Allergy Immunol. 2008; 147: 87-92. Al-Attas RA, Rahi AH. Primary antibody deficiency in
Arabs: first report from eastern Saudi Arabia. J Clin Immunol. 1998; 18: 368-371.
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allergic asthma. Clinical and Developmental Immunology. 2013; 11.