Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

CMD Block 2 > Lecture 17: Autoimmunity > Flashcards

Lecture 17: Autoimmunity Flashcards

1
Q

What transcription factor is responsible for the tissue-specific proteins in the thymus (that contribute to self-tolerance)?

A

AIRE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What do Treg cells do?

A

Shut down the immune response once a pathogen in cleared (they also play a role in autoimmunity)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

How is type II autoimmunity similar to type II hypersensitivity?

A
  1. mediated by Ab

2. specific for cell surface and extracellular matrix

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

How is type III autoimmunity similar to type III hypersensitivity?

A
  1. mediated by Ab

2. caused by immune complexes deposited in tissues

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How is type IV autoimmunity similar to type IV hypersensitivity?

A

Mediated by T cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

T/F: IgE is the most common mediator of autoimmune diseases

A

F: IgE mediates NO autoimmune diseases

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is autoimmunity?

A

Adaptive immunity specific for self-determinants

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the 5 main tolerance mechanisms?

A
  1. negative selection (within the bone marrow and thymus)
  2. Expression of tissue specific proteins in the thymus for negative selection (driven by the transcription factor AIRE, which induces expression of proteins that are not normally expressed in the thymus)
  3. Lymphocytes do NOT have access to certain tissues
  4. T reg cells suppress autoimmune responses
  5. Anergy is induced in autoreactive B/T cells (“peripheral” tolerance; no 2nd signal)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Autoimmune Hemolytic Anemia is a type ____ autoimmune disease because:

A

II; it is IgG and IgM mediated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Autoimmune Hemolytic Anemia is caused by:

A

An antibody (IgG and IgM) specific for surface antigens of erythrocytes

This can result in activation of the classical complement pathway (thus RBC are opsonized and taken up by phagocytes with Fc receptors OR killed by MAC)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Autoimmune Hemolytic Anemia is detected by what test?

A

Direct Coomb’s hemagglutination assays

  • -Patient’s washed RBCs are combined with reagent
  • -Antihuman Ab in the reagent binds to human Ab on RBCs, which link them together
  • -Positive test = clump of RBC
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

This enzyme links platelets and blood vessels with clots:

A

von Willdebrand Factor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Autoimmune Thrombocytopenic Purpura is a type ____ autoimmune disease because:

A

II; it is IgG mediated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Autoimmune Thrombocytopenic Purpura is caused by:

A 
IgG-mediated inhibition of an enzyme responsible for cleavage of von 
Willdebrand factor (vWF)

**According to robbins, the IgG is against GpIIb/IIIa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Autoimmune Thrombocytopenic Purpura is characterized clinically by:

A

1) Low platelet count
2) Bruising
3) Neuro symptoms
4) Microangioplastic hemolytic anemia–RBC are damaged as they are pushed through microscopic clots

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What is a schistocyte?

A

Broken RBC (can be visualized in a blood smear)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Treatment for Autoimmune Thrombocytopenic Purpura (one common and 5 uncommon)

A

Plasmapheresis (exchange transfusion with plasma from healthy donors)

“Unusual Treatments”

  • -Rutiximab (CD20-specific humanized monoclonal on the surface of all B cells; this trx will deplete B cells)
  • -RhoGam (only works in RhD positive patients)
  • -Steroids
  • -Splenectomy (since this is the site where most platelets are taken up and removed)
  • -Gamma Globulin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Good Pasture syndrome is caused by:

A

An Ab specific for type-IV collagen

which lines basement membranes throughout the body

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Good Pasture syndrome is characterized by:

A

Inflammatory responses (initiated by the Ab)

  • -Kidney damage (reduced function)
  • -Pulmonary hemorrhage
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Good Pasture syndrome is a type _____ autoimmune disease.

A

II

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Good Pasture syndrome is treated by:

A
  1. Plasma exchange (plasmapheresis)

2. Anti-inflammatory drugs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Scleroderma is a type _____ autoimmune disease because:

A

II, mediated by IgG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Scleroderma is caused by:

A

inflammatory destruction of:
–vascular endothelial cells of arterioles
–smooth muscle cells
These cells are replaced with collagen and other fibrous materials

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Scleroderma is characterized by:

A
  • -localized or systemic symmetrical skin thickening
  • -hard, smooth, ivory colored areas of hardened skin
  • -neurological symptoms (because the brain is affected by inflammation/replacement)
  • -sometimes rheumatoid factor (involved in nonspecific production of Ab that have specificity for Fc of other Ab)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Scleroderma is diagnosed via the presence of:

A

1) anti-nuclear Abs
2) anti-topoisomerase Abs
3) anti-centromere Abs (IgGs)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Scleroderma is treated by:

A

Increasing blood flow to the extremities (which will be on a patient-bypatient basis; there is no standard treatment)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Acute Rheumatic Fever is a type _____ autoimmune disease because:

A

II; mediated by IgM or IgG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Acute Rheumatic Fever is caused by:

A

antibodies that are produced during response to bacterial infection (Group A Streptococcus pyogenes)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Acute Rheumatic Fever is involved in molecular mimicry because:

A

Some streptococcal cell-wall carbohydrate components are very similar to determinants expressed in heart tissue

After an infection occurs, the bacteria specific Ab cross-react with the heart tissue determinants, causing inflammation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

Acute Rheumatic Fever is characterized by:

A 
Inflammation of heart tissue, causing:
1. Heart valve scarring
2. Myocarditis 
3. Chest pain
(symptoms occur after 1 mo)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Pemphigus vulgaris is a type _____ autoimmune disease because:

A

II; IgG4 mediated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Pemphigus vulgaris is caused by:

A

Loss of cohesion in keratinocytes
in the epidermis
Due to IgG-mediated autoimmunity against 2 proteins: desmoglein 1 and 3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Pemphigus vulgaris is characterized by:

A

(Painful) chronic skin blistering

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

Pemphigus vulgaris is diagnosed by:

A

Punch biopsy of lesion
Immunofluorescent Staining
*IgG4 Ab is considered pathologic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Pemphigus vulgaris is treated with:

A
  1. Corticosteroids
  2. Anti-Inflammatory drugs
  3. Rituximab (CD20-specific monoclonal Ab; B cell surface marker)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

Autoimmune diseases affecting the thyroid:

A
  1. Hashimoto’s thyroiditis
  2. Graves’
  3. Subacute thyroiditis
  4. Idiopathic hypothyroidism
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Autoimmune diseases affecting the pancreas (islets of langerhans)

A

Type I DM

Type 2 DM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Autoimmune disease affecting the adrenal glands

A

Addison’s

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Grave’s disease is caused by:

A

Ab that bind to TSH receptor (mimics the binding of TSH to TSH receptor results in overproduction of thyroid hormone)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Symptoms of Graves’:

A 
heat intolerance
nervousness/irritability
warm/moist skin
weight loss
enlargement of the thyroid
bulging eye syndrome 
characteristic stare (eye muscle inflammation)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

What determines whether or not an autoimmune condition will be able to affect a developing fetus?

A

If it is IgG-mediated or not (because they cross into fetal circulation via the Brambell receptor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Myasthenia gravis is caused by:

A

antibodies specific for acetylcholine receptors on muscle
cells:
–Ab binds to ACh receptors, which are then endocytosed and degraded
–example of an antagonist autoantibody

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

Myasthenia gravis is characterized by:

A

Muscles that less sensitive to neuronal stimulation (due to loss of receptors)

Weakness progresses, starting with facial muscles

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Myasthenia gravis is treated with (2):

A
  1. anti-inflammatory drugs
  2. Pyridostigmine, which inhibits acetyl cholinesterase (longer ACh half-life results in better competition for receptor binding)
45
Q

Myasthenia gravis is a type _____ autoimmune disease

A

II

46
Q

Grave’s disease is a type _____ autoimmune disease

A

II

47
Q

Subacute bacterial endocarditis is a type _____ autoimmune disease

A

III

48
Q

Subacute bacterial endocarditis is caused by:

A

IgG-mediated Ab that bind to microorganisms colonizing damaged valves

49
Q

Subacute bacterial endocarditis is associated with what bacteria?

A

Streptococcus viridans

50
Q

Typically, patients only develop subacute bacterial endocarditis if…

A

already suffered damage to their heart valves

51
Q

The actual inflammation in subacute bacterial endocarditis is caused by:

A
  1. cytokines produced by phagocytes (which recognize the opsonized bacteria)
  2. anaphylatoxins produced during the complement cascade
52
Q

Mixed Essential Cryoglobulinemia is a type _____ autoimmune disease

A

III

53
Q

Mixed Essential Cryoglobulinemia is caused by:

A

Cryoglobulins are produced and bind to the Fc regions of Ab (causing immune complex disorder)

*act similarly to rheumatoid factor

54
Q

Mixed Essential Cryoglobulinemia is often seen in patients with:

A

B cell proliferative disorder

55
Q

Mixed Essential Cryoglobulinemia often follows what infection?

A

Hep C

56
Q

The three symptoms of Mixed Essential Cryoglobulinemia:

A

Meltzer’s triad:
purpura
arthralgia
myalgia

57
Q

Systemic Lupus Erythmatosis is caused by:

A

autoantibodies specific for many self macromolecules (DNA, histones, ribosomes, ect.)

When cells die and release soluble macromolecules (normal process):
–immune complexes form
–deposited in vessels/kidneys/joints
leading to more inflammation

58
Q

Systemic Lupus Erythmatosis is a type _____ autoimmune disease

A

III

59
Q

Systemic Lupus Erythmatosis is characterized by:

A

“butterfly” facial rash
loss of kidney function:
–thickening of basement membrane
–infiltration of neutrophils

60
Q

Why is Lupus a progressive disease?

A

Once inflammation occurs, it leads to further cell destruction and priming of immune responses

This leads to MORE inflammation, more cell destruction, etc etc etc

61
Q

How are responses primed in lupus?

A

A CD4 T cell specific for self (e.g. histone protein) can provide stimulation for B cells that are also specific for other determinants in the complex

This B cell can then present to T cells specific for any proteins in the complex

(he was unclear on this process**)

62
Q

What disease is caused by auto-Ab that serve as receptor antagonists?

A

Myasthenia gravis

63
Q

Insulin dependent DM is a type _____ autoimmune disease

A

IV

64
Q

Insulin dependent DM is caused by:

A

destruction of insulin producing β cells in Islets of Langerhan’s by CD8 CTLs

CD8’s recognize beta-cell determinant

65
Q

What is histologically different about the pancreas of IDDM patients?

A

Lymphocyte infiltration (primarily CD8 T cells)

66
Q

Insulin dependent DM is treated by:

A

daily injections of pig/bovine insulin

67
Q

What if IDDM patients make an immune response to non-human insulin?

A

an immune-complex hypersensitivity results

and they get human recombinant insulin

68
Q

RA is a type _____ autoimmune disease mediated by:

A

IV; TH1 cells

69
Q

RA is caused by:

A

Autoreactive T cells that initiate inflammatory responses in the joints (damages cartilage and ligament tissue)

And B cells–evidenced by rituximad success

70
Q

What is a common (but not required) immuno feature of RA?

A

Production of rheumatoid factor (IgG, IgM, and IgA) specific for Fc of other Ab

71
Q

RA is treated with: (2)

A

1) TNF-alpha specific Ab (infliximab)

2) Use of ADCC to deplete B cells with anti-CD20 Ab (rituximab)

72
Q

What does rituximab do?

A

Monoclonal Ab that targets B cells for destruction, mostly by NK cells

73
Q

Sjögren’s Syndrome is a type _____ autoimmune disease mediated by:

A

IV; autoreactive T cells

74
Q

Sjögren’s Syndrome is caused by:

A

autoreactive T cells initiate inflammatory destruction of exocrine glands (i.e. tears
and saliva production)

75
Q

Sjögren’s Syndrome is characterized by:

A

dry eyes
dry mouth
(sometimes skin, nose and vaginal dryness)

76
Q

Sjögren’s Syndrome is diagnosed via: (2)

A

1) Schirmer test to measure tear output (most important)
2) anti-nuclear Ab and rheumatoid factor tests
* rheumatoid factor will not necessarily be present

77
Q

Sjögren’s Syndrome is treated with:

A

Treatment of symptoms (artificial tears, punctal plugs, goggles)

78
Q

Multiple sclerosis is a type _____ autoimmune disease mediated by:

A

IV, TH1 CD4

79
Q

Multiple sclerosis is caused by:

A

TH1 cells specific for myelin basic protein initiate immune response directed at myelin sheath of nerve cells (demyelination in CNS)

80
Q

Multiple sclerosis is characterized by:

A 
motor weakness
impaired vision
lack of coordination 
spasticity
progressive, but very variable
81
Q

Multiple sclerosis is treated by:

A

(Reduction of symptomatic attacks)

1) immunosuppressive drugs
2) IFN-β1

82
Q

Multiple sclerosis is diagnosed by the presence of:

A

Very few specificities of (oligoclonal) IgG in CSF

83
Q

T/F: There is no association between MHC and autoimmune disorders.

A

F: Certain MHC I or II are associated with certain autoimmune diseases

84
Q

Bacteria that is responsible for several autoimmune conditions through mimicry:

A

Campylobacter jejuni

85
Q

How do reactions occur to immuno privileged sites?

A
  1. Trauma to site (e.g. one eye) results in release of antigens that are otherwise sectioned off
  2. Self antigens are carried to secondary lymph, where they activate T cells
  3. Effector T cells return to attack site or both sites (e.g. placenta or testes)
86
Q

Molecular mimicry of group A strep causes:

A

Rheumatic fever

87
Q

How does MHC presentation cause molecular mimicry?

A

the same MHC presents a pathogen peptide AND a self peptide that mimics it

Once the T cell is activated by the pathogen (presented on MHC), it will respond to the self peptide on MHC as well

(This leads to macrophage activation, inflammation)

88
Q

Guillain Barré Syndrome is a type _____ autoimmune disease mediated by:

A

II; IgG specific for gangliosides

89
Q

Guillain Barré Syndrome is caused by:

A

Immune response made against pathogen (C. jejuni) cross-reacts with IgG specific for gangliosides–this results in demyelination

(gangliosides = common components of human nerve tissues)

90
Q

Guillain Barré Syndrome is treated by: (2)

A

1) plasma exchange

2) immunosuppressive drug

91
Q

Guillain Barré Syndrome is characterized by:

A
  • -symmetrical weakness of lower limbs, which rapidly ascends to upper limbs
  • -symmetrical weakness of face (difficulty swallowing and breathing; drooling)
  • -partial paralysis often occurs
92
Q

T/F: Typically, Guillain Barré patients fail to regain function of limbs.

A

F: Usually patients regain most motor function with appropriate and effective treatment

93
Q

What often precedes Guillain Barré Syndrome?

A

Infection with Campylobacter jejuni

94
Q

Wegener’s Granulomatosis is a type _____ autoimmune disease mediated by:

A

II; IgG produced following a bacterial or viral infection

95
Q

Wegener’s Granulomatosis is treated by: (2)

A

1) plasma exchange

2) anti-inflammatory drug treatment

96
Q

Wegener’s Granulomatosis is characterized by:

A

1) rhinitis (pain, stuffiness, nose bleeds) and conjuctivitis–usually first
2) lung infiltrates
3) glomerulonephritis (rapidly progressive)
4) granulomas (usually found in all affected tissues, but not always)

97
Q

Wegener’s Granulomatosis is caused by:

A

anti-neutrophil cytoplasmic Ab bind to neutrophils, causing them to become activated

Neutrophils upregulate adhesin expression and bind to vascular endothelial cells

Neutrophils then degranulate causing damage to the vasculature (vasculitis)

98
Q

Most common determinant involved in Wegener’s Granulomatosis?

A

proteinase-3

99
Q

Three diseases associated with molecular mimicry:

A

Wegener’s Granulomatosis
Guillain Barré Syndrome
Acute rheumatic fever

100
Q

In this disease, Ab cross react with cardiac tissue:

A

Acute rheumatic fever

101
Q

In this disease, vWF cleavage is inhibited:

A

Thrombocytopenic Purpura

102
Q

In this disease, Ig that become insoluble at lowered temperatures are produced:

A

Mixed Essential Cryoglobulinemia

103
Q

These Ig behave similar to rheumatoid factor (i.e. they bind Fc of Ab regions)

A

Cryoglobulins (binding to Fc causes an immune complex disorder)

104
Q

This disease is associated with inflammation that damages cartilage and ligament tissues:

A

Rheumatoid Arthritis

105
Q

This disease is associated with loss of keratinocyte adhesion:

A

Pemphigus vulgaris

106
Q

T/F: All autoimmune diseases involve a breach of T cell tolerance

A

T (I got this from the textbook)

107
Q

Why are more genetic disorder linked to MHC II?

A

Because more autoimmune disorders are linked to CD4 (I got this from the textbook)

108
Q

Little SusieJo Epstein noticed that walking on her hands (something she can usually do for 0.25 mile) was becoming increasingly difficult. When she complained of difficulty chewing gum and swallowing it, her auntie took her to the ER (where eye drooping was noted). Tests detected impaired nerve-muscle transmission, and she was treated with pyridostigmine. What Ab does her blood test show?

A

Anti-ACh receptor antibodies

109
Q

T/F: Smokers with Good Pasture syndrome often have pulmonary hemorrhage.

A

T: Smoking damages alveoli basement membrane; due to this damage, anti-type-IV-collagen can get into the basement membranes and deposit/activate/rupture

(I got this from the textbook)

CMD Block 2 (38 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions