2
4
5
A
B
C
D
E
F
G
H
I
J
K
L
M
N
O
P
Q
R
S
T
U
V
W
X
Y
Z
Antibodies
Antibodies

Selective deficiency of IgA
Definition:
Low levels of or complete absence of immune globulin A, which causes decreased immune function in the mucosal surfaces (e.g., mouth, gastrointestinal tract, and lungs) and results in increased risk for respiratory and gastrointestinal infections. It is associated with autoimmune diseases.

Alternative Names:
IgA deficiency

Causes, incidence, and risk factors:

IgA deficiency may be inherited as either an autosomal dominant (requiring only one parent to pass down the defective gene) or recessive (requiring defective genes from both) trait and is found in approximately 1 in 700 individuals of European origin. It is less common in people of other ethnicities.

Affected people often have chronicdiarrhea caused by intestinal infections, frequent respiratory infections. Many develop autoimmune diseases like rheumatoid arthritis and lupus erythematosus.
Symptoms:
Signs and tests:
  • Serum immunoelectrophoresis
  • Quantitative immunoglobulins (serum IgA extremely low or absent)
Treatment:
No specific treatment is available for IgA deficiency. However, transfusion of IV Ig (intravenous immune globulin) can be harmful. Some people gradually develop normal levels of IgA without treatment. Individual infections should be treated with appropriate antibiotics. Autoimmune disease treatment is based on the specific problem.
Expectations (prognosis):

Selective IgA deficiency is more benign than many other immunodeficiency diseases.

Some people with IgA deficiency will recover spontaneously and begin to produce IgA in larger quantities over a period of years. People with a complete absence of IgA may develop allergies or anaphylactic shock if given gamma globulin.
Complications:
An autoimmune disorder may also develop. Patients with IgA deficiency may develop antibodies to IgA, and can have severe reactions (including anaphylaxis, a potentially life-threatening allergic reaction) to transfusions of blood and blood products. If transfusions are necessary, they should ideally come from another IgA-deficient individual.
Calling your health care provider:
Consider genetic counseling if there is a family history of selective IgA deficiency and children are planned.

If you have an IgA deficiency, be sure to mention it to the provider if gamma globulin is suggested as a treatment for any condition.
Prevention:
Genetic counseling may be of value to prospective parents with a family history of selective IgA deficiency.
Review Date: 12/1/2001
Reviewed By: Frederic F. Little, M.D., Department of Allergy and Pulmonary/Critical Care Medicine, Boston University School of Medicine, Boston, MA. Review provided by VeriMed Healthcare Network.
A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is the first of its kind, requiring compliance with 53 standards of quality and accountability, verified by independent audit. A.D.A.M. is among the first to achieve this important distinction for online health information and services. A.D.A.M. is also a founding member of Hi-Ethics (www.hiethics.com) and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2003 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
adam.com