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30 Cards in this Set

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  • Back
What is autoimmunity?
An immune reaction against self-antigens
What is the normal human's lack of response to self antigens called?
Immunologic tolerance
Are autoantibodies always pathologic?
No, they can be found circulating inocuously in older people
What are the 2 general types of self-tolerance?
1. Central tolerance
2. Peripheral tolerance
What is Central tolerance?
The deletion of self-reactive Tcells and Bcells during maturation in the thymus and bone marrow.
What is peripheral tolerance?
The deletion or anergy of lymphocytes that recognize self-Ag in peripheral tissues.
How does deletion of self-recognizing lymphocytes occur?
Via apoptosis
How does anergy of self-recognizing lymphocytes occur?
Via lack of B7 on the self APC, which renders the Tcell nonfunctional and unresponsive.
What is antigen sequestration?
Hiding antigens from the immune system because the tissues are not in communication with blood and lymph
What are 3 immune-privileged sites?
-Brain
-Eye
-Testes
What happens if certain trauma or infection causes immune-privileged tissues to release their proteins?
Autoimmune response that causes inflammation and damage.
What are genetic factors that influence autoimmunity?
Susceptibility genes
What is one of the best defined susceptibility genes for autoimmunity?
HLA
What is the B27 HLA allele associated with?
Ankylosing spondylitis
What is the DR4 HLA allele associated with?
Rheumatoid arthritis
What is the DR3 HLA allele associated with?
sjogren syndrome
What is the DR3/DR4 HLA allele associated with?
Type I Diabetes
What populations have increased prevalence of autoimmunity due to hormonal factors?
-Women
-Those with klinefelters syndrome (extra X ch')
What hormone confers protection against autoimmunity?
Testosterone
2 mechanisms by which infections induce autoimmunity:
1. Upregulated B7/CD28 on host cells
2. Cross-reacting of a microbial antigen with self-reactive Tcells; after clonal expansion then they attack the self proteins.
What are the 4 categories of antinuclear antibodies?
-Anti-DNA
-Anti-histone
-Anti-nonhistone proteins bound to DNA
-Antinucleolar
What type of test is an ANA assay?
An immunofluorescence assay
For what disease is the ANA most sensitive?
SLE
What ANA titer will most individuals have?
1:40 (most)
1:80 (a few)
1:320 (very few)
So what would you definetely call an abnormal ANA assay?
>1:40 (number smaller than 40)
Once an ANA test returns positive, what is the next step in diagnosing autoimmune diseases?
Assaying for specific antibodies
What are 2 specific Ab's diagnostic for SLE?
Anti-ds-DNA
Anti-Sm
What is the specific Ab diagnostic for disseminated lupus?
Anti-histone
What are 2 specific Ab's diagnostic for Sjogren's syndrome?
Anti-RNP-SS-A (Anti-Ro)
Anti-RNP-SS-B (Anti-La)
What is the specific Ab diagnostic for limited scleroderma?
Anticentromere