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Mechanisms to get rid of self-reactive T-cells: (4)
Ignorance, anergy, phenotypic skewing, and apoptosis
What factor exists to regulate autoimmune T cells?
Where is it expressed?
AIRE- expressed inside the thymic medullary cells
How does AIRE work?
Causes medullary thymus to express self-antigens (including antigens which are specific/located solely in the eye.)
What is APECED?
Autosomal recessive disorder for AIRE mutant.
AP? in APECED.
Autoimmune polyendocrinopathy- autoimmunity against the endocrine glands
C? in APECED
Candidiasis- fungal infection can be seen on the tongue.
ED? in APECED
Ectodermal dysplasia- funny looking faces.
Ignorant T-cells can be activated by:
Viral infection of the cell causes release of self antigen to where it can be recognized.
Wrong stimulus or high lelvels of co-stimulatory molecules by dendritic cells.
What happens when antigen sequestration fails?
Autoimmunity through the activation of ignorant T-cells.
Phenotypic skewing
Refers to the ability of a T-reg cell or a tolerogenic dendritic cells ability to regulate the cytokine released by T h cells.
What is activation induced cell death?
After a T cell is activated, there is a certainty that the T cell will undergo apoptosis.
What is ALPs?
Disorder where there is a defect in Fas and FasL. T cells are unable to undergo apoptosis.

Autoimmune Lymphoproliferative syndrome
What is Idiopathic thrombocytopenic purpura?
Platelets of the individuals are attacked
Neutropenia:
Neutrophils are attacked.
What is a clinical symptom of ALPs?
Hemolytic anemia, Purpura, Neutropenia.

Enlarged spleens, livers, and lymph nodes
T regulatory cells produce: (2)
IL-10
TGF-beta
These cytokines decrease the APC function.
T regulatory cells affect dendritic cells in what way?
Through removal of co-stimulatory molecules which might be expressed on their surface.
IPEX-
Immunodysregulation polyendocrinopathy enteropathy X-linked syndrome.
X-linked mutation of the FoxP3 gene.
What is the importance of IL-22 and which cells produce this interleukin?
Mediates Candidiasis and Staphylococcus defence. Produced by Tregs, Th17.
Single organ based autoimmune diseases:
Diabetes
MS
IBD
Multiple organ based autoimmune diseases:
SLE- systemic lupus erythromatus. Ribonucleoproteins serve as the antigen.
Monozygotic twins
have higher concordance of presence of autoimmune disease. Do not necessarily have the same disease.
Which is more dominant? autoantibody or auto t cell?
antibody is more dominant.
SLE is associated with what immune protein?
It is associated with C4 complement protein.
Goodpasture's syndrome:
Smokers disease, kidney disease in all patients.
Lack of tissue integrity allows autoantibody to have access to the basement membrane of the lungs.
What are the immunologically privileged sites? (5)
Brain, eye, testis, uterus, and hamster cheek pouch
Medication that can act as autoantibodies:
Procainamid
Theories of development of Autoimmunity: (4)
Failure in the mechanisms of tolerance
Molecular mimicry
Release of inflammatory mediators
Modifications of self Ag
In addition, infections can increase autoimmunity by one other way:
Increasing the level of expression of co-stimulatory molecules on APCs.
Change in amino acide in MHC
causes susceptibility to IDDM. (insulin-dependent diabetes mellitus)