1. SELECTIVE IGA
DEFICIENCY
FATIMA AL-AWADH

2. OBJECTIVES
• Define the selective IgA deficiency.
• Recognize the structure and function of IgA.
• Mention the causes, pathophysiology, signs &
symptoms, complications, diagnosis, Treatment, Pro
gnosis and Prevention.
• State the effect of vaccination on this disorder.

3. SELECTIVE IGA DEFICIENCY
• the complete absence of the IgA class of
immunoglobulins in the blood serum and secretions.
• a relatively mild genetic immunodeficiency.
• IgA is a type of antibody that protects against
infections of the mucous membranes lining the
mouth, airways, and digestive tract.
• It is the most common of the primary antibody
deficiencies.
• IgA deficiency is believed to affect as many as 1 in
700 people, but in most of these persons it causes
no clinical problems.

4. STRUCTURE

5. FUNCTION OF IGA

6. CAUSES
• The defect causing these
deficiencies is not known in a
majority of cases; rarely, the
deficiencies may be caused
by mutations of Ig heavy chain
constant region genes.

7. PATHOPHYSIOLOGY
Alterations in trans- bacteria
inherited membrane inability to are more
maturation activator, calcium
defect in B modulator & produce Ig able to
cells cyclophilin ligand A cause
interactor gene disease

8. PATHOPHYSIOLOGY
“B LYMPHOCYTES ARE UNABLE TO PRODUCE IG A”

9. SIGNS & SYMPTOMS
85–90% of IgA-deficient individuals are
asymptomatic. Some patients with IgA deficiency
have a tendency to develop recurrent:
• sinopulmonary infections
• gastrointestinal infections and disorders
• Otitis media
• Skin infection
• Allergies

10. COMPLICATIONS
• An autoimmune disorder such as rheumatoid
arthritis.
• severe, even life-threatening, reactions to
transfusions of blood and blood products because
they develop anti-IgA antibody.

11. DIAGNOSIS
Patient & family history.
Quantitative
immunoglobulins.
Serum
Immunoelectrophoresis.

12. PATIENT & FAMILY HISTORY
• Family history of selective IgA
deficiency.
• Either chronic or recurrent
infections, allergies, auto-immune
diseases, chronic diarrhea, or some
combination of these problems.

13. QUANTITATIVE IMMUNOGLOBULINS
• rapid and accurate measurement of the
amounts of the immunoglobulins M, G, and
A.
• tests of blood serum demonstrate absence
of IgA with normal levels of the other major
classes of immunoglobulins (IgG and IgM).
NORMAL results of Ig M, G and A
IgG 560 to 1800 mg/dL
IgM 45 to 250 mg/dL
IgA 100 to 400 mg/dL

14. SERUM IMMUNOELECTROPHORESIS
• detect the presence of certain
antibody.
• It is used to detect if the patient have
anti-IgA antibodies.

15. MANAGEMENT
patients who do not have any symptoms
do not need any treatment.
prevent anaphylactic reaction
secondary to blood transfusion
treatment of associated diseases

16. PROGNOSIS
The prognosis is good in patients with IgA deficiency
if it is not associated with a significant disease.
IgA deficiency in children may resolve over time.
IgA deficiency may progress into CVID.

17. PREVENTION
• There is no means of prevention of Selective IgA
Deficiency.
• Because IgA Deficiency does not become
detectable until approximately six months of age,
prenatal and neonatal detection of this disorder is
currently not possible.

18. VACCINATION AND IGA DEFICIENCY
Contraindicated Risk-specific Effective Vaccines
Vaccines recommended
Vaccines
OPV2 Pneumococcal All vaccines likely
BCG effective. Immune
Yellow fever response might be
Other live vaccines attenuated.
appear to be safe

19. REFERANCES
• Basic Immunology, Abbas 3th ed.
• http://www.nlm.nih.gov/medlineplus/ency/article/0
01476.htm
• http://www.ncbi.nlm.nih.gov/pmc/articles/PMC282
1513/
• http://primaryimmune.org/wp-
content/uploads/2011/04/Selective-IgA-
Deficiency.pdf
• http://www.cdc.gov/vaccines/pubs/pinkbook/dow
nloads/appendices/A/immuno-table.pdf

20. THANK YOU