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.
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.
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 Alterations in trans- bacteria inherited membrane inability to are morematuration activator, calciumdefect in B modulator & produce Ig able to cells cyclophilin ligand A cause interactor gene disease
PATHOPHYSIOLOGY“B LYMPHOCYTES ARE UNABLE TO PRODUCE IG A”
SIGNS & SYMPTOMS85–90% of IgA-deficient individuals areasymptomatic. Some patients with IgA deficiencyhave 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. Quantitativeimmunoglobulins. 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.
MANAGEMENTpatients who do not have any symptomsdo not need any treatment. prevent anaphylactic reaction secondary to blood transfusiontreatment of associated diseases
PROGNOSISThe prognosis is good in patients with IgA deficiencyif 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 DEFICIENCYContraindicated Risk-specific Effective VaccinesVaccines recommended VaccinesOPV2 Pneumococcal All vaccines likelyBCG effective. ImmuneYellow fever response might beOther live vaccines attenuated.appear to be safe