Chapter 6 Flashcards

1
Q

What type of cells are activated in a type I HS?

A

CD4+ TH2 responses

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2
Q

What are the roles of TH2 T cells

A

produce cytokines

IL4 and IL13 which lead to increase B cell IgE synthesis

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3
Q

what chemokines can cause mast cell degranulation

A

IL8 and adenosine

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4
Q

what drugs can cause mast cell degranulation

A

codein, morphine

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5
Q

what phsyical stimuli can cause mast cell degranulation

A

sunlight, trauma, heart/cold

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6
Q

What are released in the initial response of type I HS

A

histamine, chymase.tryptase

heparin

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7
Q

what are released in the delayed phase type I HS

A
lipid mediators
LKT B4, C4, D4, E4
Porstaglandin D2
PAF
TNF and IL1
IL4 for + feedback
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8
Q

What cells are responsible for the Ab-dep cell-mediated cytotoxicity in type II HS

A

NK

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9
Q

What are common diseases that are type II HS

A

Good pastures (Ab agaainst alveolar and glomerular BM)
Myasthenia Gravis
DM II

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10
Q

What type of HS is SLE

A

III IC

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11
Q

What type of HS is post-streptococcal glomerulonephritis

A

III IC

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12
Q

What is a disease type III HS from infectious agent

A

polyareteritis nodosa

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13
Q

what type of rxn is acute serum sickness

A

type III

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14
Q

Describe injury causes by IC deposition

A

activates C’

can have fibrinoid necrosis from protein accumulation

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15
Q

What is type IV HS

A

T cell mediated CD8

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16
Q

describe the CD4 rxn in type IV HS

A

DTH
TH1 with macrophages
TH17 with neutrophils

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17
Q

What causes CD4 switch to TH1

A

IL-12, then TH1 makes IFNy which increases TH1

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18
Q

what causes CD4 witch to TH17

A

IL 1,6,23 and TGFb

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19
Q

what is the role of IFNyin DTH

A

Macrophage activation increasing TNF and IL1 and IL12

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20
Q

what is the role of TH17 cells in DTH

A

recruit, activate neutrophils and monocytes

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21
Q

What does a granuloma look like

A

not around central blood vessel and lots of epithelial histiocytes

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22
Q

types of type IV HS

A
RA
DM I
MS
Geullain Barre
Crohns
contact dermatitis
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23
Q

describe CTL component of type IV HS

A

granzymes and performs

CTL don’t need prior recognition of tumors or viruses

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24
Q

What type of HS reaction is graft rejection

A

type IV DTH

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25
Q

What is AIRE

A

autoimmune regulator, important in central tolerance

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26
Q

Where are T cells found in thymus

A

paracortical regions

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27
Q

What is clonal deletion

A

the method of peripheral tolerance for B and T cells with high affinity to self

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28
Q

What cytokines are responsible for anergy

A

B7-CD28 whcih irreversible inactivate T cells when recognize self

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29
Q

What is PTPN-22 encode and what autoimmune diseases are related

A

tyrosine phosphotase

DM and RA

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30
Q

What is NOD2 endode and what AI disease

A

sensing IC microbes

IBD

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31
Q

What IL polymorphisms are assoc with MS

A

2 and 17

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32
Q

What are characteristics of SLE

A

auto Ab to ds DNA, smith Ab and ANAs detected by immunofleuorescence

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33
Q

antiphospholiipid Ab to cardiolipin indicates what

A

+syphilis, seen in SLE

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34
Q

how can SLE cause recurrent thrombosis

A

lupus anticoagulants

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35
Q

What C’ parts are deficient withs ome SLE

A

C2, C4, C1q–> IC aggregation

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36
Q

What immune cells are targeted in SLE

A

B cells because self react

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37
Q

What are included in criteria for SLE

A

malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neuro disorder, hematologic disorder, immuno disorder and ANAs

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38
Q

What happens to kidney in SLE

A

increase cell infiltration, microvascular thormbosis, vas wall deposition, increased heamturia, proteinuria, HTN, renal insufficiency

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39
Q

What is typical of joints in SLE

A

non-erosive synovitis

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40
Q

What type of necrosis occurs in heart in SLE

A

fibrinoid

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41
Q

What are verrucuos, Libman-sacks endocarditis

A

assoc with SLE

small vegetations sup and inverior of heart valves

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42
Q

What occurs in spleen in SLE

A

follicular hyperplasia (onion skin appearance)

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43
Q

What are the top 10 clinical manifestations of SLE

A

hematologic, arthritis, skin, fever, fatigue, weight loss, renal, neuropshychiatric, pleuritis, myalgia

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44
Q

What causes chronic discoid lupus erythematous

A

deposition of Ig C3 at dermal epidermal junction

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45
Q

Ab to SS-A and HLA-DR3 are assoc with what

A

subacute cutaneous lupus erythematous

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46
Q

what drugs can induce SLE

A

hydralazine procainamide, Isonizid, D-pennicliamine

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47
Q

What are the recombination genes for T cells

A

Rag 1 and 2

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48
Q

What are the components of the TCR complex

A

CD3 and zig

CD4,8,2,28

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49
Q

What R are on B cells that are essential

A

Fc, C’ and CD40

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50
Q

Without CD40 what happens

A

no Ab production because B cells cannot interact with T cells CD40L
hyper IgM

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51
Q

What is the R for EBV

A

CR2 or CD21

52
Q

What type of R are on dedritic cells

A

TLRs and mannose R for microbes

53
Q

Where are follicular DC found

A

germinal centers in spleen and lymph nodes

54
Q

What R do follicular DC have

A

FcR for IgG and R for C3b

55
Q

What is the key effector cell to eliminate IC microbes

A

macrophages

56
Q

What CD are used to identify Nk cells

A

16 and 56

57
Q

What R inhibit NK cells

A

Ig-like, CD94 family of lectins

58
Q

what cytokines do macrophages secrete

A

TNF IL1

59
Q

What is related with HLA b27

A

ankylosing spondylitis

60
Q

What locus of HLA are autoimmune related to

A

DR

61
Q

HLA-A is associ with what disease

A

hereditary hemachromatosis

62
Q

What are the costimulators for APCs on T cells

A

B7

63
Q

what are the costimulators for APCs on naive T cells

A

CD28

64
Q

during earliest response to microbe in cell mediated immunity what cells and IL are released

A

CD4 and IL2

65
Q

Where does isotype switching of Ig take place

A

in germinal centers formed by proliferating B cells

66
Q

what Ig activate C’

A

IgG and IgM

67
Q

What tissues are involved in RA

A

joints, skin vessels, lungs and heart

68
Q

What is primary form sjogren

A

sicca which is fibrosis lacrimal and salivary glands

69
Q

What disease is assoc with Ab SS-A Ro and SS-B La

A

sjogren syndrome, sicca

70
Q

lymphocytic infiltration salivary glands, ductal lining hyperplasia, atrophy acini and hyalinizaiton and drying of corneal epithelium point to what

A

sicca syndrome

71
Q

what is essential for Dx of sicca, or primary sjogren

A

lip biopsy

72
Q

what are the clinical features of sjogren syndrome

A

keratoconjuntivitis, xerostomia, parotid gland enlargement, synovitis, other AI disorders

73
Q

What is systemic sclerosis from

A

chornic disease with chronic inflmmation from AI with widespread damage and progressive fibrosis

74
Q

What is diffuse scleroderma

A

widespread skin involvement with rapid progression

75
Q

what is limited scleroderma

A

skin involvement is on fingers toes, face

CREST

76
Q

What ANAs are found assoc with diffuse scleroderma

A

DNA topoisomerase I leading to pulm fibrosis and peripheral vasc disease

77
Q

what Ab are found assoc with CREST

A

anti centromere

78
Q

What can happen with scleroderma in alimentary track

A

progressive atrophy with Barret metaplasia and loss of microvilli leading to malabsorption

79
Q

What type of necrosis occurs in kidney with scleroderma

A

fibrinoid, death from renal failure common

80
Q

can you Dx patient based on CREST symptoms

A

no, need Ab against centromeres

81
Q

What are examples of inflammatory myopathies

A

dermatomyositis, polymyositis, inclusion-body myositis

82
Q

how do you Dx inflammatory myopathies

A

muscle biopsy

83
Q

high titers of Abs to ribonucleoprotein particle containing U1 RNP is assoc with what

A

mixed CT syndrome

84
Q

if patient has less than 4 criteria SLE than what is high on Ddx

A

mixed CT

85
Q

mutations on Xq21.22 is probably causing what

A

well its bruton tyrosine kinase, so failure development B cells.

86
Q

What type of infections are people with Burtons Agammaglobulinemia prone to

A

respiratory

viral–> giardia resisted usually IgA

87
Q

what are histological appearance of burtons agammaglobulinemia

A

underdevelopled germinal centers of lymph nodes, peyers patches, appendix tonsils
no plasma cells

88
Q

what type of inheritance is burtons agammaglobulinemia

A

x linked recessive

89
Q

Normal # B cells, no plasma cells, recurrent sinopulmonary infections, recurrent herpes, persistent diarrhea
hyperplastic B cell areas
probably what?

A

common variable immunodeficiency

90
Q

what descent is common to have isolated IgA deficiency

A

european

91
Q

what type of infections occur with isolated IgA deficiency

A

sinopulmonary and diarrhea

but usually asymptomatic

92
Q

What can cause hyper IgM syndrome

A

X linked CD40L on Xq26

auto recessive mutations in CD40 or cytosine deaminase

93
Q

What occurs clincally in hyper IgM syndrome

A

recurrent pyogenic infections

autoimmune hemolytic anemia, thrombocytopenia, neutropenia

94
Q

What is the result of a deletion 22q11

A

thymic hypoplasia, DiGeorge syndrome

lack of parathyroid, congenital heart defects, abnormal facies

95
Q

What causes x linked SCID

A

common y-chain subunit cytokine R deficient IL17 and 15

96
Q

what causes autosomal recessive SCID

A

adenosine deaminase deficiency

97
Q

what are histo findings in types of SCID

A

x linked: thymus has epithelial cells resembling fetal thymus
ADA deficiency has Hassall’s corpuscles

98
Q
decreased IgM
normal IgG
increased IgA IgE
thrombocytopenia and eczema
non-hodgkin B cell lymphoma
A

wiskott aldrich syndrome WASP Xp11.23

99
Q

What occurs with C3 deficiency

A

serious, pyogenic infections

incidence of IC mediated glomerulonephritis

100
Q

What are the types of amyloid

A

AL in plasma cell tumors
AA in secondary amyliodosis
B-amyloid protein in alzheimers

101
Q

What type of amyloid is a problem with hemodialysis

A

B2-microglobulin MHC class I

102
Q

what does disease does primary amyloidosis occur with

A

multiple myeloma

103
Q

bence jones proteins are assoc with what

A

primary amyloidosis

104
Q

What conditions are commonly assoc with reactive system amyloidosis

A

RA, heroin abusers
renal cell carcinoma
hodkin lymphoma

105
Q

what is familial mediterranean fever

A

autoinflamm
increased IL1 production
attachs of fever and inflammation of serosal surfaces via AA proteins

106
Q

what areas are assoc with familial med fever

A

armenian, sephardic jewish and arabic

107
Q

what is a sago spleen vs lauraceous spleen

A

sago: tapioca like granules on gross inspection
lauraceous: red pulp in splenic sinuses

108
Q

where do amyloids deposit in liver

A

space of Disse– hepatic parenchymal cells and sinusoids then kupffer cells

109
Q

what are clinical features amyloidosis

A

proteinuria

insidious CHF and arrythmias, chronic constrictive pericarditis, diarrhea

110
Q

What is classifcation A HIV

A

asymptomatic, acute. sometimes persistent generalized lymphadenopathy
A1 >500 cell A3 <200

111
Q

What is calssication B HIV

A

not A or C, symptomatic

B1 >500 B3<200

112
Q

what is classification C HIV

A

AIDS indicator conditions: constitutional disease, neuro disease or neoplasm

113
Q

Ab marker for drug induced SLE

A

antihistone

114
Q

Ab marker for polymyositis/dermatomyositis

A

Jo-1

115
Q

Ab mixed CT disease

A

Anti-U1-ribonucleotide protein

116
Q

x lnked SCID is mutations in what

A

y light chain of cytokine R

117
Q

most serious complication of diffuse scleroderma

A

malignant hypertension

118
Q

what cell is responsible for fibrotic chances in limited scleroderma

A

CD4

119
Q

HIV patients most at risk for what neoplasm

A

non-hodgkin B cell lymphoma

120
Q

what condition is most likely in someone with polymyositis, or Jo-1 Ab titers

A

myocarditis

121
Q

what other condition besides ankylosing spondylitis is assoc with HLA b27

A

psoriatic arthropathy

122
Q

what mutation in RNA HIV virus accounts for drug R

A

reverse transcriptase

123
Q

what pneumoniae is common in AIDS

A

pneumocystitis jiroveci

124
Q

what mutation is with SCID auto recessive

A

adenosine deaminase deficiency

125
Q

What Ab is in Goodpasture syndrome

A

type IV collagen so attacks BM

126
Q

What Ab assoc with sjogrens

A

SS-B

127
Q

what is a common cause senial cardiac amyloidosis

A

deposition transtheyretin