Autoimmunity Flashcards

1
Q

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

A

Immune Regulation

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2
Q

Immune regulation prevents inappropriate immune responses and reactions specific for

A

Self antigens

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3
Q

Failure of tolerance to control immune mechanisms leads to

A

Autoimmunity

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4
Q

A specific unresponsiveness to a self antigen

A

Immunological self-tolerance

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5
Q

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

A

Autoimmunity

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6
Q

What are the two types of tolerance?

A

Central tolerance and peripheral tolerance

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7
Q

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

A

Central tolerance

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8
Q

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

A

Negative selection

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9
Q

These T cells that escape are controlled by

A

Peripheral tolerance

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10
Q

The principal fate of lymphocytes that recognize self is

A

Death

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11
Q

Some T cells in the thymus and periphery differentiate into

A

Regulatory T lymphocytes

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12
Q

What is the phenotype of regulatory T cells?

A

CD4+

  • high IL-2R
  • Low IL-7R
  • Foxp3+
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13
Q

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

A

Foxp3 mutations

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14
Q

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

A

IPEX

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15
Q

Most children affected with IPEX die within the

A

First 2 years of life

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16
Q

One major cell group with promising therapeutic regulation is

A

Tregs (T-regulatory cells)

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17
Q

In the thymus, T cell selection occurs on

A

MHC-peptide

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18
Q

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

A

AIREs

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19
Q

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

A

Autoimmune Polyglandular Syndrome (APS) or APECED

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20
Q

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

A

Type 1 diabetes

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21
Q

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

A

AIRE

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22
Q

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

A

AIRE

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23
Q

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

A

Bone Marrow

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24
Q

These B cells are controlled by

A

Bone Marrow

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25
Q

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

A

Autoimmunity

26
Q

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

A

Reduction/loss in Treg activity

27
Q

Autoimmune diseases usually arise

A

Spontaneously

28
Q

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

A

Organ-specific autoimmunity

29
Q

Autoimmune disease that effects the whole body

A

Systemic autoimmune diseases

30
Q

RA, SLE and scleroderma are examples of

A

Systemic autoimmune diseases

31
Q

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

A

Families

32
Q

Are mostly multigenic

A

Human AI diseases

33
Q

Have a major genetic association with autoimmune diseases

A

MHC genes

34
Q

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

A

CD4 or CD8 T cells

35
Q

What is the HLA association of RA?

A

DR4 + DR1

36
Q

What is the HLA association of Type 1 DM?

A

DR3 + DR4

37
Q

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

A

Common pathways

38
Q

Multiple genes are involved in predisposition to each

A

AI disease

39
Q

Autoimmunity development is also influenced by

A

Environmental Factors

40
Q

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

A

NOD mice

41
Q

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

A

Infection

42
Q

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

A

Autoimmunity

43
Q

Infection provides an environment that promotes

A

Lymphocyte activation

44
Q

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

A

Coxsackie virus

45
Q

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

A

Self-reactive T cells

46
Q

Coxsackie B4 nuclear protein is a mimic of human

A

GAD

47
Q

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

A

Hygiene hypothesis

48
Q

Autoimmune disease can be triggered by

A

Drugs

49
Q

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

A
  1. ) Immunological response (reversible)

2. ) True drug autoimmunity

50
Q

The immunological response is also called

A

Drug hypersensitivity

51
Q

An example of drug hypersensitivity is

A

Acacavir Hypersensitivity Syndrome (AHS)

52
Q

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

A

Acacavir

53
Q

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

A

A true autoimmune process induced by a drug

54
Q

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

A

Eliminated

55
Q

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

A

Epitope Spreading

56
Q

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

A

Self-tolerance

57
Q

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

A

Self-reactive T cells

58
Q

What is the prototypic immune complex mediated disease?

A

Systemic Lupus Erythemtosus (SLE)

59
Q

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

A

Sifalimumab

60
Q

What are three other new strategies for treating SLE?

A

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

61
Q

An IL-1 antagonist used to treat RA

A

Anakinra

62
Q

Tocilizumab is used to treat RA. It functions as an

A

Anti-IL-6 receptor antibody