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SELECTIVE IGA

DEFICIENCY
FAT I M A A L - A W A D H

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

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.

STRUCTURE

FUNCTION OF IGA

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.

PATHOPHYSIOLOGY

inherited
maturation
defect in B
cells

Alterations in
trans-membrane
activator, calcium
modulator &
cyclophilin ligand
interactor gene

inability to
produce Ig
A

bacteria
are more
able to
cause
disease

PATHOPHYSIOLOGY
B LY M P H O C Y T E S A R E U N A B L E T O P R O D U C E I G A

SIGNS & SYMPTOMS


8590% 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

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.

DIAGNOSIS
Patient & family
history.

Quantitative
immunoglobulins.

Serum
Immunoelectrophore
sis.

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.

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

SERUM
IMMUNOELECTROPHORESIS

detect the presence of certain


antibody.
It is used to detect if the patient have
anti-IgA antibodies.

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

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.

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.

VACCINATION AND IGA DEFICIENCY

Contraindicated
Vaccines

Risk-specific
recommended
Vaccines

Effective Vaccines

OPV2
BCG
Yellow fever
Other live vaccines
appear to be safe

Pneumococcal

All vaccines likely


effective. Immune
response might be
attenuated.

REFERANCES
Basic Immunology, Abbas 3th ed.
http://
www.nlm.nih.gov/medlineplus/ency/article/00147
6.htm
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC28
21513
/
http://
primaryimmune.org/wp-content/uploads/2011/04/Se
lective-IgA-Deficiency.pdf
http://
www.cdc.gov/vaccines/pubs/pinkbook/downloads/
appendices/A/immuno-table.pdf

THANK YOU