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Pathology > Immunopathology II > Flashcards

Immunopathology II Flashcards

1
Q

What is the cause of GVHD?

A

Recipient’s APC activate CD4 T4 cells, from the graft, causing an immune response

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2
Q

What causes acute GVHD?

A

Donor cytotoxic T cells or cytokines from helper T cells destroy epithelial cells

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3
Q

What are the skin changes in acute GVHD?

A

Rash, exfoliation

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4
Q

What are the GIT changes in GHD?

A

Ulcerative gastroenteritis

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5
Q

What are the hepatic changes in acute GVHD?

A

Bile duct necrosis

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6
Q

What happens to the immune system in acute GVHD?

A

Immunosuppression

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7
Q

What are the histological changes in the bile ducts in acute GVHD?

A

Loss of cuboidal epithelium

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8
Q

Does acute always precede chronic GVHD?

A

No

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9
Q

What is the MOA of chronic GVHD?

A

Development of autoreactive T cells from donor stem cells that cannot be clonally deleted

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10
Q

What are the three places that are most often damaged in chronic GVHD?

A
  1. Dermis and skin appendages
  2. GI mucosa
  3. Jaundice
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11
Q

What are the two, broad types of autoimmune disease?

A

Systemic

Single cell or organ

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12
Q

Diffuse scleroderma is caused by what?

A

Topoisomerase I attacked

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13
Q

Limited scleroderma is caused by what?

A

Centromere proteins acctacked

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14
Q

What are the three criteria for autoimmune disease?

A
  1. Immunological rxn to a self antigen
  2. Reaction is primary o pathogenesis
  3. No other well defined cause or identifiable etiology
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15
Q

How do you diagnose autoimmune diseases?

A

Lab tests
Biopsies
clinical characteristics

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16
Q

What happens to antigen naive T cells in the thymus?

A

Maturation and selection

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17
Q

What are the two things that are necessary to activate a T cell? What happens if these are not present?

A

B7 from APC, and signals from Th cells

If not present, then anergy

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18
Q

CD28 ligand on T cells bind to what on APCs?

A

B7

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19
Q

What is peripheral tolerance?

A

When T cells bind to MHC, but not bind to B7, then they become anergic (or apoptose)

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20
Q

Expression of what genes confers higher susceptibility to loss of self tolerance?

A

D locus on MHC class I

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21
Q

Polymorphism in what gene that encodes a protein Y-kinase is implicated in autoimmunity?

A

PTPN-22

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22
Q

How do microbes stimulate autoimmunity?

A

cross reactivity or molecular mimicry

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23
Q

Which gender is more susceptible to autoimmune disease?

A

Females

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24
Q

SLE occurs when, and in whom?

A

Females around 2-3rd decade

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25
Q

What is characteristic of both the butterfly rash, and the discoid rash seen in SLE?

A

Photosensitive

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26
Q

What is the malar rash in SLE?

A

fixed erythema, flat or raised over the malar eminences tending to spare the nasolabial folds

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27
Q

What is the discoid rash in SLE?

A

Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur

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28
Q

What are the neurological disorderes with SLE?

A

Szs or psychosis

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29
Q

What are the hematologic disorder that occurs in 100% of pts with SLE?

A

hemolytic anemia
leukopenia
Lymphopenia

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30
Q

What is the serositis in SLE?

A

Pleuritis or pericarditis document by ST elevation in all leads

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31
Q

What is the renal disorder seen in SLE?

A

Proteinuira

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32
Q

What are the immunologic disorders with SLE?

A

Anti-dsDNA, anti SM + anti phospholipid abs

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33
Q

What is the genetic locus associated with SLE? What does this code for?

A

HLA-DQ

MHC class II

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34
Q

What are the environmental facotors associated with SLE?

A

UV light
Viruses
Drugs
hormones

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35
Q

What is the immune response to SLE?

A

Self reactive helper T cells escapse tolerlance drie antibody production by B cells

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36
Q

What type of autoimmunity if SLE?

A

II, III (antibodies and immune complexes)

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37
Q

What is the immune response to SLE?

A

Self reactive helper T cells escape tolerance drive autoantibody production by B cells

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38
Q

What are the common symptoms with SLE? (5)

A 
renal disease
Arthritis
Fever
Fatigue
weight loss
39
Q

What causes the autoimmunity of SLE?

A

Increased burden of apoptotic bodies, thus increased nuclear antigen

40
Q

What is the hallmark of SLE, and is used in diagnosis?

A

Antinuclear antibodies (ANA), reflecting a loss of tolerance

41
Q

What are the 4 categories of Ag that ANAs are directed against in SLE?

A

DNA
HIstones
Proteins bound to RNA
Nuclear Ag

42
Q

What is the diagnostic technique used in SLE?

A

Immunofluorescent staining

43
Q

What is ANA testing?

A

Uses human tissue cell culture nuclei as a substrate for diagnostic screening and toerh types of nuclei

44
Q

What are the two self antigens that are attacked in SLE?

A

Double stranded DNA

Histones

45
Q

What is the self antigen attacked in Diffuse scleroderma? Limited?

A 
Diffuse = Topoisomerase I
Limited = Centromere proteins
46
Q

Why can you be totally healthy, but still have reactive self antibodies in your serum?

A

Damaged tissue is targeted

47
Q

What is central tolerance?

A

Destruction of T cells from binding too well to MHC cells in the thymus

48
Q

What are the three MOA of peripheral tolerance?

A
  1. No B7
  2. Th suppression of self reactive T cells
  3. Apoptosis
49
Q

What three bits of information are obtained from ANA testing?

A

Positive/negative
Titer
Pattern of staining

50
Q

What does the peripheral rim staining pattern of ANA indicate?

A

Indicates Ab to ds-DNA

Seen in SLE

51
Q

Homogenous staining of ANA is found for what three antibodies? Which two disorders is this particularly found in?

A

Ab to DNP, ds-DNA, histones

Seen in RA and SLE

52
Q

Speckled ANA staining pattern occurs for what four antibodies?

A

Anti-sm
Anti-ro and La
anti-Scl 70

53
Q

Nucleolar ANA staining pattern is found for what antibody?

A

Anti-centromere

54
Q

Speckled pattern with anti- Sm and RNP indicates what?

A

Sjogren’s syndrome

55
Q

Speckled anti Scl-70 indicates what?

A

PSS (systemic)

56
Q

Speckled anti-centromere indicates what?

A

PSS (CREST)

57
Q

Speckled anti-nucleolar indicates what?

A

SLE and PSS

58
Q

Of the following diseases:

  • SLE
  • Drug-induced LE
  • Systemic sclerosis (diffuse)
  • Limited scleroderma (CREST)
  • Sjogren’s,

which has “many nuclear antigens”?

A

They all do, but SLE and drug induce SLE >95%

59
Q

Of the following diseases:

  • SLE
  • Drug-induced LE
  • Systemic sclerosis (diffuse)
  • Limited scleroderma (CREST)
  • Sjogren’s,

which has anti ds-DNA abs?

A

SLE

60
Q

Of the following diseases:

  • SLE
  • Drug-induced LE
  • Systemic sclerosis (diffuse)
  • Limited scleroderma (CREST)
  • Sjogren’s,

which has anti-histones?

A

> 95% drug induces SLE

61
Q

Of the following diseases:

  • SLE
  • Drug-induced LE
  • Systemic sclerosis (diffuse)
  • Limited scleroderma (CREST)
  • Sjogren’s,

which has anti-nuclear RNP?

A

SLE (30-40%)

62
Q

Of the following diseases:

  • SLE
  • Drug-induced LE
  • Systemic sclerosis (diffuse)
  • Limited scleroderma (CREST)
  • Sjogren’s,

which has anti SS-A(Ro) or SS-B(La)?

A

sjogren

63
Q

Of the following diseases:

  • SLE
  • Drug-induced LE
  • Systemic sclerosis (diffuse)
  • Limited scleroderma (CREST)
  • Sjogren’s,

which has anti DNA topoisomerase I (Scl-70)?

A

Systemic sclerosis (22-36%)

64
Q

Of the following diseases:

  • SLE
  • Drug-induced LE
  • Systemic sclerosis (diffuse)
  • Limited scleroderma (CREST)
  • Sjogren’s,

which has anti-smith?

A

SLE

65
Q

Of the following diseases:

  • SLE
  • Drug-induced LE
  • Systemic sclerosis (diffuse)
  • Limited scleroderma (CREST)
  • Sjogren’s,

which has anti-centromeric?

A

Limited scleroderma (CREST)

66
Q

Homogenous ANA pattern = ?

A

RA or drug induced SLE

67
Q

Rim pattern ANA pattern = ?

A

SLE active flares

68
Q

Centromere ANA pattern = ?

A

CREST syndrome

69
Q

What are the specific Lupus ANAs?

A

dsDNA
Anti-Smith
RBCs
Phospholipids

70
Q

Hyper or hypo coagulability in SLE? Why?

A

Hypocoag d/t antibodies against Phospholipids

71
Q

What are the hypersensitivities seen in SLE?

A

II, III

72
Q

True or false: any organ can be involved in SLE

A

True

73
Q

What causes the arthralgias of SLE?

A

Immune complexes

74
Q

What are the histological characteristics of SLE skin rash?

A

Immune cells beneath the dermis

75
Q

What are the vascular changes in SLE?

A

Immune complexes deposit in vascular beds leading to vasculitis

76
Q

What are the histological characteristics of the vascular changes seen in SLE?

A

Layered, fibrous thickening (onion staining)

77
Q

What is the MOA of renal damage in SLE?

A

Immune complex deposition in the glomeruli

78
Q

What are the cells that make up the ECM in glomeruli?

A

Mesangial cells

79
Q

What is the acute form of serosal membranes in SLE? Chronic?

A 
Acute = exudation of fibrin
Chronic = Proliferation of fibrous tissue
80
Q

What are the heart defects in SLE? (2)

A

Endocarditis (libman and Sacks)

Pericarditis

81
Q

Small, warty vegetations along lines of closure of valve leaflets in the heart = ?

A

Rheumatic heart disease

82
Q

Large, irregular destructive masses on valve cusps that can extend to the chordae = ?

A

Infective endocarditis

83
Q

Small, bland vegetations at the line of closure =?

A

Nonbacterial thrombotic endocarditis

84
Q

Small-medium sized vegetations on either/both sides of valve leaflets =?

A

Libman-Sacks endocarditis

85
Q

What are the lung changes seen in SLE? Acute, chronic ?

A 
Acute = pneumonitis
Chronic = vascular/pulmonary fibrosis
86
Q

What are the CNS symptoms seen in SLE? MOA?

A

Focal deficits + Szs

MOA= ?

87
Q

What are the three major causes of death with SLE?

A

Renal failure
Infection
CAD

88
Q

How often does SLE develop in chronic discoied LE?

A

5-10%

89
Q

What is subacute cutaneous LE?

A

Rash, mild systemic disease

90
Q

How often does SLE develop in chronic discoid LE?

A

5-10%

91
Q

What is the treatment for drug induced LE?

A

Cessation of drug

92
Q

What are the drugs involved in drug induced Lupus? (4)

A

D-penicillamine
Procainamide
Hydralazine
Isoniazid

93
Q

What is Drug induce SLE?

A

Lupus -like syndrome without Renal or CNS involvement

Pathology (32 decks)

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