FAT I M A A L -AWA D H

SELECTIVE IGA DEFICIENCY

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

“ B LY M P H O CY T E S A R E U N A B L E T O P R O D U C E I G A”

PATHOPHYSIOLOGY

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

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/001476.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/downloads/appendices/A/immuno-table.pdf

THANK YOU