Immunoglobulin A deficiency

Immunoglobulin A deficiency (IgA deficiency): Description, Causes and Risk Factors: ImmunoglobulinImmunoglobulin A (IgA) antibodies are primarily found in the nose, airway passages, digestive tract, ears, eyes, saliva, tears, and vagina. These antibodies protect body surfaces that are frequently exposed to foreign organisms and substances from outside of the body. The IgA antibodies make up about 10-15% of the antibodies found in the body. The IgA class of immunoglobulins has the specific function of protecting the body's mucosal surfaces (eyes, mouth, throat, lungs, gastrointestinal, and genitourinary tract) from infection. While IgA is aided in this role to some degree by other classes of immunoglobulins, the lack or severe deficiency of IgA at these body sites makes one or more prone to recurrent infection, allergies, chronic diarrhea, or autoimmune diseases. In autoimmune diseases, the immune system mistakenly attacks the body's own tissues. Selective IgA deficiency is the most common of the primary immunodeficiencies. It is defined as the total absence or severe deficiency of IgA. Blood serum levels for IgA deficient persons are usually found to be 7 mg/dL or less, while serum IgA in normal adults ranges from 90 to 450 mg/dL. The prevalence in Caucasians is around one in 500, whereas in some Asian populations it is very uncommon. Most individuals with IgA deficiency are clinically asymptomatic. Those with symptoms of immunodeficiency have predominantly sinopulmonary or gastrointestinal infections, which are more severe when associated with IgG2, IgG4 or specific antibody deficiency. IgA deficiency is believed to be one end of a spectrum of immunodeficiency with common variable immunodeficiency at the most severe end. Although primary IgA deficiency is the most commonly encountered form, secondary deficiencies due to drugs or viral infections are recognized. IgA deficiencies can be partial or transient. Primary IgA deficiency is caused by a defect of terminal lymphocyte differentiation, which leads to underproduction of serum and mucosal IgA; affected individuals have normal IgA genes. A number of non-immunoglobulin genes have been implicated in IgA deficiency. Medical research has not yet determined the exact cause or causes of Selective IgA deficiency. In some families, there is evidence of familial occurrence, suggesting both autosomal dominant (only one abnormal gene is required which dominates the other) and recessive modes (two abnormal genes, one from each parent, are required) of transmission. Selective IgA deficiency also occurs frequently in immediate relatives of persons with common variable immunodeficiency, suggesting similarity in causes of the two disorders. In rare cases, partial IgA deficiency has been linked to deletions of the Immunoglobulin IgA1 or IgA2 genes on chromosome 14, as well as to genes in the major histocompatibility complex on chromosome 6. This region determines powerful antigens that produce strong T-cell responses. Other reports link the disorder to partial deletion of the long or short arm of chromosome 18, which results in what is called the 18-q syndrome: carp shaped mouth, reduced prominence of the mid facial area, nystagmus (involuntary, rapid, rhythmic eye movement, hypotonia (reduced muscle strength), atretic (congenital absence or closure), or stenotic (abnormal narrowing or constriction) ear canals, hearing loss, and mental retardation. However, the vast majority of persons with Selective IgA Deficiency have no evidence of chromosomal abnormalities. The association of IgA deficiency has been found in some patients with ataxia telangiectasia (irregularities or failure of muscle coordination affecting eyes and skin), frequent respiratory infections, and immunodeficiency (re: Louis Bar Syndrome), a hereditary, progressive disease. Additionally, Selective IgA deficiency can occur as a consequence of congenital intrauterine infection with rubella (German or three day measles), toxoplasmosis (disease caused by protozoan infection), or cytomegalovirus (a common type of herpes virus). A temporary form of acquired Selective IgA deficiency has been reported following treatment with penicillamine for Wilson's Disease, which is an inherited disorder caused by accumulation of copper in the body causing multiple symptoms, or phenytoin/hydantoin (brand name: Dilantin) for seizure disorders. However, when these drugs are withdrawn, the Immunoglobulin IgA deficiency is reversed. There have been many diseases reported in association with IgA deficiency, particularly autoimmune diseases. The most common association is with celiac disease (CD), which has special significance since CD is usually diagnosed by detection of specific IgA antibodies that are obviously lacking in IgA deficiency. Risk Factors: Allergic disorders.
  • Recurrent infections.
  • Asthma.
  • Food allergies.
  • Crohn's disease.
  • Relation to patient with hypogammaglobulinemia.
  • Celiac disease.
  • Chromosomal abnormality.
  • Possible malignancy.
  • Endocrinopathy (any disease caused by disorder of the endocrine glands)
  • Hypersplenism (an increased spleen activity causing anemia, increased red blood cell destruction, and spleen enlargement) and thrombocytopenia (abnormally low platelet count).
  • Intestinal nodular hyperplasia (excessive formation of intestinal nodes).
  • Recurrent giardiasis (protozoan infection).
  • Pulmonary hemosiderosis (deposits of iron-containing pigment from hemoglobin in lungs due to red blood cell destruction).
  • Rheumatoid arthritis (chronic inflammatory disease causing joint changes and deformities).
  • Juvenile rheumatoid arthritis (rheumatoid arthritis affecting juveniles with onset before 16; remission occurs in 75% of patients).
  • Systemic lupus erythematosus (chronic inflammation of connective tissues affecting skin, joints, kidneys, and nervous system).
  • Thyroiditis (inflammation of the thyroid gland).
  • Transfusion reaction.
  • Pernicious anemia (anemia due to low intestinal absorption of vitamin B12).
  • Dermatomyositis (connective tissue disease with edema, dermatitis, and muscle inflammation).
  • Coomb's positive hemolytic anemia (positive for antibodies to part of red blood cell causing anemia due to red blood cell destruction).
  • Idiopathic Addison's disease (deficiency of secretions of adrenocortical hormones from the adrenal gland which affects almost all body systems)
  • Sj

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