Autoimmunity

Question 1

Avoids excessive lymphocyte activation and tissue damage during normal immune responses to pathogens and infections

Answer 1

Immune Regulation

Question 2

Immune regulation prevents inappropriate immune responses and reactions specific for

Answer 2

Self antigens

Question 3

Failure of tolerance to control immune mechanisms leads to

Answer 3

Autoimmunity

Question 4

A specific unresponsiveness to a self antigen

Answer 4

Immunological self-tolerance

Question 5

A breakdown or failure in the mechanisms that mediate self-tolerance leads to

Answer 5

Autoimmunity

Question 6

What are the two types of tolerance?

Answer 6

Central tolerance and peripheral tolerance

Question 7

The selection of B cells (bone marrow) and T cells (thymus)

Answer 7

Central tolerance

Question 8

Central tolerance is not perfect. Some self-reactive T cells escape thymic

Answer 8

Negative selection

Question 9

These T cells that escape are controlled by

Answer 9

Peripheral tolerance

Question 10

The principal fate of lymphocytes that recognize self is

Answer 10

Death

Question 11

Some T cells in the thymus and periphery differentiate into

Answer 11

Regulatory T lymphocytes

Question 12

What is the phenotype of regulatory T cells?

Answer 12

CD4+

• high IL-2R
• Low IL-7R
• Foxp3+

Question 13

A human autoimmune disease called Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) is caused by

Answer 13

Foxp3 mutations

Question 14

Most commonly manifests with early onset, insulin-dependent diabetes mellitus (T1 diabetes)

Answer 14

IPEX

Question 15

Most children affected with IPEX die within the

Answer 15

First 2 years of life

Question 16

One major cell group with promising therapeutic regulation is

Answer 16

Tregs (T-regulatory cells)

Question 17

In the thymus, T cell selection occurs on

Answer 17

MHC-peptide

Question 18

But not all self proteins are expressed in the thymus. So how do proteins from tissue-specific genes get expressed in the Thymus?

Answer 18

AIREs

Question 19

Rare autosomal recessive disorder with the hallmarks of Chronic mucocutaneous candidiasis (yeast infections), Chronic hypoparathyroidism, and Addison’s disease

Answer 19

Autoimmune Polyglandular Syndrome (APS) or APECED

Question 20

APs and APECED will eventually result in other autoimmune diseases such as

Answer 20

Type 1 diabetes

Question 21

Patients with APS and APECED have a mutation in the transcription factor

Answer 21

AIRE

Question 22

Regulates autoimmunity by promoting the ectopic expression of peripheral tissue restricted antigens in the thymus

Answer 22

AIRE

Question 23

Central tolerance is not perfect. Some self-reactive B cells escape negative selection in the

Answer 23

Bone Marrow

Question 24

These B cells are controlled by

Answer 24

Bone Marrow

Question 25

Relating to, or caused by autoantibodies or T cells that attack molecules, cells, or tissues of the organism producing them

Answer 25

Autoimmunity

Question 26

We are supposed to be tolerant to self-antigens so how is autoimmunity initiated?

Answer 26

Reduction/loss in Treg activity

Question 27

Autoimmune diseases usually arise

Answer 27

Spontaneously

Question 28

The disease is restricted to specific organs of the body. Autoantigens from one or a few a organs are recognized and thus, the disease is limited to these organs

Answer 28

Organ-specific autoimmunity

Question 29

Autoimmune disease that effects the whole body

Answer 29

Systemic autoimmune diseases

Question 30

RA, SLE and scleroderma are examples of

Answer 30

Systemic autoimmune diseases

Question 31

The genetic basis of autoimmunity is that it tends to run in

Answer 31

Families

Question 32

Are mostly multigenic

Answer 32

Human AI diseases

Question 33

Have a major genetic association with autoimmune diseases

Answer 33

MHC genes

Question 34

In many cases MHC link has been tracked back to peptide presentation (and subsequent activation of

Answer 34

CD4 or CD8 T cells

Question 35

What is the HLA association of RA?

Answer 35

DR4 + DR1

Question 36

What is the HLA association of Type 1 DM?

Answer 36

DR3 + DR4

Question 37

Some variants predispose to multiple autoimmune diseases providing confirmation that many diseases share

Answer 37

Common pathways

Question 38

Multiple genes are involved in predisposition to each

Answer 38

AI disease

Question 39

Autoimmunity development is also influenced by

Answer 39

Environmental Factors

Question 40

Inbred mice that are genetically identical and are destined to get diabetes

Answer 40

NOD mice

Question 41

Can lead to autoimmunity by causing T cells and antibodies to become activated, which results in the potential for them to cross-react with self antigens

Answer 41

Infection

Question 42

A non-self protein that looks like self (mimicry) when delivered via a pathogen provides all the signals necessary to activate the immune system and cause

Answer 42

Autoimmunity

Question 43

Infection provides an environment that promotes

Answer 43

Lymphocyte activation

Question 44

To serve as an example of this, NOD mice have an increase in the severity of diabetes when infected with

Answer 44

Coxsackie virus

Question 45

Viral infection leads to inflammation, tissue damage and release of sequestered islet antigens and subsequent generation of

Answer 45

Self-reactive T cells

Question 46

Coxsackie B4 nuclear protein is a mimic of human

Answer 46

GAD

Question 47

Excessive prevention of early childhood exposure to dirt and pathogens can stunt the development of the immune system. This is called the

Answer 47

Hygiene hypothesis

Question 48

Autoimmune disease can be triggered by

Answer 48

Drugs

Question 49

What are the two types of autoimmunity that can be caused by drugs?

Answer 49

1. ) Immunological response (reversible) | 2. ) True drug autoimmunity

Question 50

The immunological response is also called

Answer 50

Drug hypersensitivity

Question 51

An example of drug hypersensitivity is

Answer 51

Acacavir Hypersensitivity Syndrome (AHS)

Question 52

An anti-retroviral (reverse transcriptase inhibitor) used to treat HIV

Answer 52

Acacavir

Question 53

Progresses initially independently of drug withdrawal requires some form of immunosuppressive treatment

Answer 53

A true autoimmune process induced by a drug

Question 54

Autoimmune diseases tend to be chronic because the initiating trigger often cannot be

Answer 54

Eliminated

Question 55

An immune response starts with one antigen but quickly spreads to many other antigens. This is called

Answer 55

Epitope Spreading

Question 56

In an organ-specific T cell-mediated autoimmune disease, various genetic loci may confer susceptibility to autoimmunity, in part by influencing the maintenance of

Answer 56

Self-tolerance

Question 57

Environmental triggers, such as infections and other inflammatory stimuli, promote the influx of lymphocytes into tissues and the activation of

Answer 57

Self-reactive T cells

Question 58

What is the prototypic immune complex mediated disease?

Answer 58

Systemic Lupus Erythemtosus (SLE)

Question 59

An anti-IFN-a antibody (type I interferon) used to treat SLE

Answer 59

Sifalimumab

Question 60

What are three other new strategies for treating SLE?

Answer 60

TLR inhibition, B cell depletion, and Anti-BAFF antibodies

Question 61

An IL-1 antagonist used to treat RA

Answer 61

Anakinra

Question 62

Tocilizumab is used to treat RA. It functions as an

Answer 62

Anti-IL-6 receptor antibody