Autoimmunity

Question 1

tolerogens

Answer 1

antigens that induce self tolerance

Question 2

immunogens

Answer 2

antigens that induce immune response

Question 3

central tolerance of T cells location and mechanisms

Answer 3

thymus

deletion or become regulatory T cells

Question 4

central tolerance of B cells location and mechanisms

Answer 4

bone marrow

receptor editing
deletion and anergy

Question 5

peripheral tolerance of T cells location and mechanisms

Answer 5

spleen, nodes

anergy, suppression and deletion

Question 6

peripheral tolerance of B cells location and mechanisms

Answer 6

spleen/lymph nodes

anergy, suppression and deletion

Question 7

development of regulatory T cells in thymus

Answer 7

during negative selection if the T cells recognize self they can be deleted or become regulatory T cells

Question 8

autoimmune regulator protein

Answer 8

is a transcription factor that is in thymus and helps with presentation of the peripheral tissue self antigens…critical role in self tolerance and negative selection

Question 9

APECED (autoimmune polyendocrinopathy candidiasis ectodermal dystrophy)

Answer 9

recessive inheritance of mutation for AIRE gene leading to autoantibodies

presents with recurrent candidiasis, adrenal insufficiency and hypoparathyroidism

Question 10

common autoantibodies in APECED for candidiasis

Answer 10

IL-17 and IL-22

Question 11

master sign of the regulatory T cells

additional signs

Answer 11

FoxP3+

CD3, CD4, CD25

Question 12

two routes leading to T cell anergy with costimulation

Answer 12

1. dendritic cells need microbe to upregulate B7 expression so if T cell binds to dendritic that does not have the microbe antigen the T cell is anergized through inhibitory signals
2. if T cell binds to dendritic with a microbial antigen but does nto work costimulation then will be anergized

Question 13

CTLA-4 receptor on T cells

Answer 13

Inhibitory molecule

this is the receptor involved with negative regulation of immune response and self tolerance

Question 14

Ipilimumab

Answer 14

monoclonal antibody that bind CTLA4 enhancing T cell activation

Question 15

Abatecept

Answer 15

CTLA4 molecule and Fc region IgG1 in drug form to bind CD80 on T cells and interrupt co stimulation…anergy

cancer drug

Question 16

cytokine tht induces FoxP3 in regulatory T cells

Answer 16

TGF-beta

Question 17

regulatory T cells mechanisms of suppression

Answer 17

expresses cytokines

expressed CTLA4

express IL2 receptor and will capture IL-2

Question 18

IPEX (immune dysregulation polyendocrinopathy enteropathy X linked syndrome)

Answer 18

X linked recessive

mutations in FoxP3 on T reg cells

triad is dermatitis, endocrine issue, and chronic diarrhea

Question 19

FasL and Fas

Answer 19

FasL is the death receptor on the T cell and Fas is the ligand that binds to signal apoptosis in the periphery

Question 20

ALPS (autoimmune lymphoproliferation syndrome)

Answer 20

dominant disorder of apoptosis with Fas and FasL problems leading to accumilation of the lymphoid cells

splenomegaly, lymphadenopathy, hepatomegaly

Question 21

ALPS risks

Answer 21

autoimmune disorders of the blood and lymphomas

Question 22

molecular mimicry in autoimmunity

Answer 22

microbe antigen very similar to self antigen that a self lymphocyte can recognize and will lead to activation of the self reactive T cell

Question 23

Systemic Lupus Erythematosus B and T cell problem

Answer 23

defective B and T cell tolerance

Question 24

Lupus genetic linkages

Answer 24

complement 1,2,4 issues

TREX1

HLA DR2,3

Question 25

lupus environmental factors

Answer 25

UV radiation, viral infection, drugs and chemicals

Question 26

lupus hormone problems

Answer 26

mainly women in childbearing years

estrogen induced flares

Question 27

SLE mechanism

Answer 27

mainly antinuclear antibodies leading to B and T cell defective tolerance

Question 28

SLE symptoms

Answer 28

malar rash
discoid lupus rash
photosensitivity
oral/nasal ulcerations 
arthritis
nephritis
encephalopathy (seizures)
pericarditis
cytopenias

Question 29

Testing for SLE

Answer 29

positive antinuclear antibodies

Question 30

Antibodies to smith nuclear antigen and dsDNA

Answer 30

two good signs for SLE