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Immunopathology II Flashcards

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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
What is characteristic of both the butterfly rash, and the discoid rash seen in SLE?
Photosensitive
26
What is the malar rash in SLE?
fixed erythema, flat or raised over the malar eminences tending to spare the nasolabial folds
27
What is the discoid rash in SLE?
Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur
28
What are the neurological disorderes with SLE?
Szs or psychosis
29
What are the hematologic disorder that occurs in 100% of pts with SLE?
hemolytic anemia leukopenia Lymphopenia
30
What is the serositis in SLE?
Pleuritis or pericarditis document by ST elevation in all leads
31
What is the renal disorder seen in SLE?
Proteinuira
32
What are the immunologic disorders with SLE?
Anti-dsDNA, anti SM + anti phospholipid abs
33
What is the genetic locus associated with SLE? What does this code for?
HLA-DQ MHC class II
34
What are the environmental facotors associated with SLE?
UV light Viruses Drugs hormones
35
What is the immune response to SLE?
Self reactive helper T cells escapse tolerlance drie antibody production by B cells
36
What type of autoimmunity if SLE?
II, III (antibodies and immune complexes)
37
What is the immune response to SLE?
Self reactive helper T cells escape tolerance drive autoantibody production by B cells
38
What are the common symptoms with SLE? (5)
``` renal disease Arthritis Fever Fatigue weight loss ```
39
What causes the autoimmunity of SLE?
Increased burden of apoptotic bodies, thus increased nuclear antigen
40
What is the hallmark of SLE, and is used in diagnosis?
Antinuclear antibodies (ANA), reflecting a loss of tolerance
41
What are the 4 categories of Ag that ANAs are directed against in SLE?
DNA HIstones Proteins bound to RNA Nuclear Ag
42
What is the diagnostic technique used in SLE?
Immunofluorescent staining
43
What is ANA testing?
Uses human tissue cell culture nuclei as a substrate for diagnostic screening and toerh types of nuclei
44
What are the two self antigens that are attacked in SLE?
Double stranded DNA | Histones
45
What is the self antigen attacked in Diffuse scleroderma? Limited?
``` Diffuse = Topoisomerase I Limited = Centromere proteins ```
46
Why can you be totally healthy, but still have reactive self antibodies in your serum?
Damaged tissue is targeted
47
What is central tolerance?
Destruction of T cells from binding too well to MHC cells in the thymus
48
What are the three MOA of peripheral tolerance?
1. No B7 2. Th suppression of self reactive T cells 3. Apoptosis
49
What three bits of information are obtained from ANA testing?
Positive/negative Titer Pattern of staining
50
What does the peripheral rim staining pattern of ANA indicate?
Indicates Ab to ds-DNA Seen in SLE
51
Homogenous staining of ANA is found for what three antibodies? Which two disorders is this particularly found in?
Ab to DNP, ds-DNA, histones Seen in RA and SLE
52
Speckled ANA staining pattern occurs for what four antibodies?
Anti-sm Anti-ro and La anti-Scl 70
53
Nucleolar ANA staining pattern is found for what antibody?
Anti-centromere
54
Speckled pattern with anti- Sm and RNP indicates what?
Sjogren's syndrome
55
Speckled anti Scl-70 indicates what?
PSS (systemic)
56
Speckled anti-centromere indicates what?
PSS (CREST)
57
Speckled anti-nucleolar indicates what?
SLE and PSS
58
Of the following diseases: - SLE - Drug-induced LE - Systemic sclerosis (diffuse) - Limited scleroderma (CREST) - Sjogren's, which has "many nuclear antigens"?
They all do, but SLE and drug induce SLE >95%
59
Of the following diseases: - SLE - Drug-induced LE - Systemic sclerosis (diffuse) - Limited scleroderma (CREST) - Sjogren's, which has anti ds-DNA abs?
SLE
60
Of the following diseases: - SLE - Drug-induced LE - Systemic sclerosis (diffuse) - Limited scleroderma (CREST) - Sjogren's, which has anti-histones?
>95% drug induces SLE
61
Of the following diseases: - SLE - Drug-induced LE - Systemic sclerosis (diffuse) - Limited scleroderma (CREST) - Sjogren's, which has anti-nuclear RNP?
SLE (30-40%)
62
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)?
sjogren
63
Of the following diseases: - SLE - Drug-induced LE - Systemic sclerosis (diffuse) - Limited scleroderma (CREST) - Sjogren's, which has anti DNA topoisomerase I (Scl-70)?
Systemic sclerosis (22-36%)
64
Of the following diseases: - SLE - Drug-induced LE - Systemic sclerosis (diffuse) - Limited scleroderma (CREST) - Sjogren's, which has anti-smith?
SLE
65
Of the following diseases: - SLE - Drug-induced LE - Systemic sclerosis (diffuse) - Limited scleroderma (CREST) - Sjogren's, which has anti-centromeric?
Limited scleroderma (CREST)
66
Homogenous ANA pattern = ?
RA or drug induced SLE
67
Rim pattern ANA pattern = ?
SLE active flares
68
Centromere ANA pattern = ?
CREST syndrome
69
What are the specific Lupus ANAs?
dsDNA Anti-Smith RBCs Phospholipids
70
Hyper or hypo coagulability in SLE? Why?
Hypocoag d/t antibodies against Phospholipids
71
What are the hypersensitivities seen in SLE?
II, III
72
True or false: any organ can be involved in SLE
True
73
What causes the arthralgias of SLE?
Immune complexes
74
What are the histological characteristics of SLE skin rash?
Immune cells beneath the dermis
75
What are the vascular changes in SLE?
Immune complexes deposit in vascular beds leading to vasculitis
76
What are the histological characteristics of the vascular changes seen in SLE?
Layered, fibrous thickening (onion staining)
77
What is the MOA of renal damage in SLE?
Immune complex deposition in the glomeruli
78
What are the cells that make up the ECM in glomeruli?
Mesangial cells
79
What is the acute form of serosal membranes in SLE? Chronic?
``` Acute = exudation of fibrin Chronic = Proliferation of fibrous tissue ```
80
What are the heart defects in SLE? (2)
Endocarditis (libman and Sacks) | Pericarditis
81
Small, warty vegetations along lines of closure of valve leaflets in the heart = ?
Rheumatic heart disease
82
Large, irregular destructive masses on valve cusps that can extend to the chordae = ?
Infective endocarditis
83
Small, bland vegetations at the line of closure =?
Nonbacterial thrombotic endocarditis
84
Small-medium sized vegetations on either/both sides of valve leaflets =?
Libman-Sacks endocarditis
85
What are the lung changes seen in SLE? Acute, chronic ?
``` Acute = pneumonitis Chronic = vascular/pulmonary fibrosis ```
86
What are the CNS symptoms seen in SLE? MOA?
Focal deficits + Szs MOA= ?
87
What are the three major causes of death with SLE?
Renal failure Infection CAD
88
How often does SLE develop in chronic discoied LE?
5-10%
89
What is subacute cutaneous LE?
Rash, mild systemic disease
90
How often does SLE develop in chronic discoid LE?
5-10%
91
What is the treatment for drug induced LE?
Cessation of drug
92
What are the drugs involved in drug induced Lupus? (4)
D-penicillamine Procainamide Hydralazine Isoniazid
93
What is Drug induce SLE?
Lupus -like syndrome without Renal or CNS involvement

Pathology (32 decks)

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