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