Autoimmune Disorders Flashcards

1
Q

Define autoimmunity

A

an adaptive immune response against self antigen that is directed toward healthy tissue and leads to impaired tissue

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2
Q

What are the 4 factors that contribute to a persons risk for developing autoimmune diseases

A
  • Genetic
  • Race
  • Sex/Gender
  • Environment
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3
Q

What are the main genetic determinants for autoimmune diseases?

A

MHC I and MHC II loci

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4
Q

Why do females tend to have a higher risk for autoimmune diseases?

A

presence of estrogen, progesterone and a different microbiome than men

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5
Q

T reg cells are also known as

A

CD25

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6
Q

T reg cells suppress

A

auto reactive CD4 T cells

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7
Q

What 2 possible genetic mutations can lead to autoimmune disease

A
  1. The absence of Autoimmune Regulator Protein (AIRE)

2. FoxP3 Mutations

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8
Q

What does AIRE play a role in?

A

Negative selection

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9
Q

Autoimmune polyglandular disease is a result of the absence of

A

Autoimmune regulator protein (AIRE)

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10
Q

List 4 clinical presentations of autoimmune polyglandular disease (APD)

A
  • begins in infancy
  • hypoparathyroidism
  • nail dystrophy
  • adrenal failure
  • ovarian failure
  • type 1 diabetes
  • candidiasis
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11
Q

APD clinical association involves what areas (6)

A

endocrine glands, skin, hair, nails, ovaries, teeth

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12
Q

What does FoxP3 play a role in?

A

Treg cell differentiation

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13
Q

IPEX is a result of _____ mutation

A

FoxP3 mutation

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14
Q

What cells are elevated in IPEX

A

Th17 cells

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15
Q

When does IPEX begin to present

A

early in life

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16
Q

List the presentations of IPEX (3)

A
  • eczema
  • diarrhea
  • Type 1 diabetes
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17
Q

What are the 5 ways that infection can trigger autoimmune disease

A
  1. Disruption of cell or tissue barrier
  2. Binding of pathogen carrier to be recognized
  3. Molecular mimicry
  4. Superantigen
  5. Bystander effect
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18
Q

What trigger event can avoid co-stimulaiton

A

Superantigen

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19
Q

What is the effect of the disruption of cell or tissue barrier?

A

The release of self antigen

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20
Q

How does binding of a pathogen to self protein trigger immune disease?

A

pathogen can act as a carrier to allow self antigen response

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21
Q

______ gives microbes the ability to nonspecifically activate T cells

A

Superantigen

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22
Q

Describe the bystander effect

A

A T cell can bind self antigen on MHC but will not receive second signal from that cell but if there is an infection nearby that cell can give the T cell the co-stimulatory signal

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23
Q

What is the target of Graves Disease?

A

Thyroid; TSH receptor

24
Q

What is the Target in Myasthenia Gravis

A

Acetylcholine receptor

25
Q

What is the target in Autoimmune Hemolytic Anemia?

A

RBC antigens

26
Q

What is the target in Goodpasture’s Syndrome

A

Type IV collagen ECM

27
Q

What is the target in Rheumatic Fever

A

Antibody against S. pyogens that cross reacts with myosin

28
Q

What is the target in Rheumatoid Arthritis?

A

Antibodies against self IgG

29
Q

What is the target in Systemic Lupus Erythematous (SLE)

A

antibodies against parts of cytoplasm and nucleus

30
Q

What is the target in Hashimoto’s Thyroiditis

A

Thyroid antigens

31
Q

What is the target in Type 1 Diabetes?

A

Insulin and glutamate decarboxylase

32
Q

What is the target in Celiac Disease

A

gluten metabolites

33
Q

What is the mechanism for Graves Disease

A

IgG antibodies directed against the Thyroid stimulating hormone receptor act as an agonist so that the receptor acts as if it was receiving TSH this leads to an increased production of Thyroid Hormone

34
Q

What is the mechanism for Myasthenia Gravis

A

IgG antibodies are made against the acetylcholine receptor acting as an antagonist, triggering the ACh receptor to be endocytose and degraded

35
Q

What is the mechanism for Autoimmune Hemolytic Anemia (warm and cold)

A

Warm: IgG antibodies are made against self Rh factor trigger destruction by opsonization or complement
Cold: IgM antibodies are made against glycophorin (I antigen)

36
Q

What is the mechanism for Goodpasture’s Syndrome

A

IgG autoantibodies directed against type IV collagen of glomerulus basement membrane resulting in the activation complement causing inflammatory damage

37
Q

What is the mechanism for Rheumatic Fever

A

The antibodies IgM against S. pyrogens cross react with myosin found in the heart, joints, and kidneys causing inflammation in these organs

38
Q

What is the mechanism for Rheumatoid Arthritis

A

Production of IgG antibodies (rheumatoid factor) against self IgG which forms an immune complex that deposits in joints leading to complement activation and inflammation.
Antibodies can also be made against epitopes of self proteins (peptidlyl arginine deaminase)

39
Q

What is the mechanism for Systemic Lupus Erythematous

A

Autoantibodes are formed against antinuclear antigens such as ribonucleic proteins, DS DNA and lipoporteins to form immune complexes that deposit in tissues

40
Q

What is the mechanism for Hashimoto’s Thyroiditis

A

Th1 cells induce an inflammatory response that cause a change in architecture of the thyroid, forming ectopic lymphoid tissue causing an underactive thyroid

41
Q

What is the mechanism for Type 1 Diabetes

A

T cells and antibodies are formed against insulin and glutamate decarboxylase and proteins of beta islet cels causing destruction preventing the production of insulin

42
Q

What is the mechanism for Celiac Disease

A

Tissue transglutaminase converts glutamine to glutamic acid–>glutamic acid has higher affinity to bind to MHC–> presentation of glutamic acid to CD4 T cells–>inflammatory response

43
Q

What are the clinical features of Graves Disease (7)

A
  • Hyperthyroidism
  • Weight loss
  • Irritability
  • Enlarged Thyroid
  • Bulging Eyes
  • Nervousness
  • Heat Tolerance
44
Q

What are the clinical features of Myasthenia Gravis

A
  • muscle weakness
  • ptosis
  • diplopia (double vision)
  • Weakening of chest muscles
45
Q

What are the clinical features of Autoimmune Hemolytic Anemia *not discussed

A
46
Q

What are the clinical features of Goodpasteur’s Syndrome (3)

*2 occur in smokers

A
  • glomerlonephritis
  • pneumonitis
  • pulmonary hemmorage
47
Q

What are the clinical features of Rheumatic Fever

A

-inflammation and heart damage

48
Q

What are the clinical features of Rheumatoid Arthritis (2)

A
  • Arthiritis

- damage to cartilage, ligaments, tendons, bones

49
Q

What are the clinical features of SLE (4)

A
  • glomerulonephritis
  • arthiritis
  • Vasculitis
  • Rash
50
Q

What are the clinical features of Hashimoto’s Thyroiditis (5)

A
  • Hypothyroidism
  • fatigue
  • weight gain
  • sensitivity to cold
  • weakness
51
Q

What are the clinical features of Type 1 Diabetes

A

-inability to control blood sugar levels

52
Q

What are the clinical features of Celiac Disease

A

-reduced absorption capacity in jejunum

53
Q

What are the treatment options for Hashimoto’s Thyroiditis

A

replacement therapy with thyroid hormones

54
Q

The rheumatoid factor is directed against the _____ receptor

A

Fc

55
Q

What is the infection association in Type 1 Diabetes

A

T cells are produced against cosakie virus and they cross react with insulin.

56
Q

What enzyme in those with celiac disease converts glutamine to glutamic acid?

A

Tissue transglutaminase

57
Q

What is the result of the conversion of glutamine to glutamic acid in the case of Celiac Disease

A

Glutamate has a more negative charge making it have a higher affinity to bind with MHC resulting in the activation of CD4 T cells that result in cytokine release and reactive oxygen species destruction