BY KOMAL SAHARAN

GROUP 232 E
 Immunoglobulin A
• Immunoglobulin A (IgA), as the major class of
antibody present in the mucosal secretions.
• represents a key first line of defense against
invasion by inhaled and ingested pathogens at
the vulnerable mucosal surfaces.
 IgA Subclasses

• IgA1, comprises approximately 85% of total IgA

concentration in serum. IgA1 shows a good immune
response to protein antigens and, to a lesser degree, to
polysaccharide and lipopolysaccharides.

• IgA2, representing only up to 15% of total IgA in

serum, plays an important role in the mucosa of the
airways, eyes, and the gastrointestinal tract to fight
against polysaccharide and lipopolysaccaride antigens.
It also fighting bacterial infections.
Secreting form
Selective deficiency of IgA is the most
common (PIDD). Persons with this
disorder have low or absent levels of a
blood protein called IgA.
 Classification
• Severe IgA deficiency refers to serum levels
below 0.7 mg/mL, which is the lower limit of
detection for most assays.
• Partial IgA deficiency refers to serum levels
above 0.7 mg/mL, but below the lower limit of
normal.
 causes
• Unknown in many cases
• mutation of heavy chain constant regionin
some.
• IgA deficiency is could be inherited (20% of
cases) , which means it is passed down
through families. It may be inherited as an
autosomal dominant or autosomal recessive
trait.
 PATHOPHYSIOLOGY

“B lymphocytes are unable to produce Ig A”

 Risk factor
• Caucasians People have a higher risk factor, it
is found in approximately 1 in 700 individual.

• Family history especially if the patient has

First-degree relative in this condition.
 Symptoms (1)
85–90% of IgA-deficient individuals are
asymptomatic.

 The most common symptom of Selective IgA Deficiency is

susceptibility to infections including:
• Bronchitis.
• Chronic diarrhea.
• Conjunctivitis.
• Gastrointestinal inflammation.
• Mouth infection.
 Symptoms (2)
• Otitis media.
• Pneumonia.
• Sinusitis.
• Skin infections.
• Upper respiratory tract infections.
• Bronchietasis(a disease in which the small air sacs in the lungs
become damaged and enlarge).
• Unexplained asthma.
 Diagnosis

Patient & family history.

Quantitative
immunoglobulins.

Serum
Immunoelectrophoresis.
 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.
 Complication
• 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 .What if transfusions
are necessary?
• Some patients develop common variable
immunodeficiency.
 Management
• patients who do not have any symptoms do
not need any treatment.
• prevent anaphylactic reaction secondary to
blood transfusion.
• treatment of associated diseases.
 Treatment
• No specific treatment is available. Some
people gradually develop normal levels of IgA
without treatment.

• Infections should be treated with antibiotics.

• Those with selective IgA deficiency who also

have IgG subclass deficiencies can benefit
from Intravenous immunoglobulin (IVIG).
Intravenous immunoglobulin (IVIG)
• It is a sterile solution of concentrated
antibodies extracted from healthy donors
which is administered into a vein.

• IVIG is used to treat disorders of the immune

system or to boost immune response to
serious illness.
The side effects for IVIG treatment (1)

• People with complete IgA deficiency may develop

anti-IgA antibodies if given blood products and
IVIG. This may lead to allergies or life-
threatening anaphylactic shock.

(These individuals can safely be given IgA-depleted

IVIG if this deficiency is also associated with IgG
subtype deficiency)
The side effects for IVIG treatment (2)
• Headache.
• dermatitis - usually peeling of the skin.
• infection (such as HIV or viral hepatitis).
• damage such as hepatitis caused directly by
antibodies contained in the pooled IVIG.
• acute kidney injury.
• venous thrombosis.
 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.

• But genetic counseling may be of value to prospective

parents with a family history of selective IgA deficiency.

• Vaccines, including the pneumococcal vaccine, may be

administered to prevent infections that are commonly
associated with the disorder.
 Vaccination and IgA deficiency
• Local live virus vaccine, such as intranasal
influenza vaccine, yellow fever vaccine and
live rotavirus vaccine, should not be given to
patients with IgA deficiency until their
immune status is fully evaluated.

• Other vaccines, such as those against

pneumococcus, are specifically
recommended.
 References
• Basic Immunology, Abbas 3th ed.
• http://www.nlm.nih.gov/medlineplus/ency/article/001476.htm
• http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2821513/
• http://primaryimmune.org/wp-
content/uploads/2011/04/Selective-IgA-Deficiency.pdf
• http://www.cdc.gov/vaccines/pubs/pinkbook/downloads/appendic
es/A/immuno-table.pdf
• http://bandbacktogether.com/Immunoglobulin-A-IgA-Deficiency-
resources/
• http://www.healthcentral.com/ency/408/001476.html?ic=506019
• http://www.aaaai.org/conditions-and-treatments/primary-
immunodeficiency-disease/selective-iga-deficiency.aspx