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PBD I Exam 2 > Diseases of the Immune System III > Flashcards

Diseases of the Immune System III Flashcards

1
Q

self-tolerance

A

normal state of non-responsiveness to ones own antigens

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2
Q

requirements for autoimmunity

A

1 immune specific reaction to autoantigen
2 evidence it is not due to tissue damage
3 absence of another cause

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3
Q

type I DM

A

autoreactive T cells and antibodies - against pancreatic beta cells

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4
Q

multiple sclerosis

A

autoreactive T cells against CNS myelin

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5
Q

goodpasture syndrome

A

autoreactive antibodies against BM of lung and kidney

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6
Q

SLE

A

autoreactive antibodies against DNA, platelets, RBCs, protein phospholipid complexes

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7
Q

central tolerance

A

T and B cells in central lymph organs

T cells - negative selection in thymus
-AIRE - stimulates expression of self-antigens in thymus

B cells - receptor editing in bone marrow

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8
Q

peripheral tolerance

A

T and B cells in peripheral tissues

anergy, T-reg cell suppression, deletion

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9
Q

anergy

A

T cells - don’t get costimulator CD28

  • lose ability to signal
  • also express CTLA-4 and PD-1
  • inhibitory signals

B cells - don’t get T cell help
-become unable to respond

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10
Q

CTLA-4 and PD-1

A

inhibitory signal to T cell activity

-knocked out of this gene - autoimmunity

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11
Q

CD28

A

costimulator for T cell activation

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12
Q

suppression by T-reg cells

A

CD4 T cells with CD25 and FoxP3

-develop in thymus in response to self antigen

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13
Q

Foxp3

A

on T-reg cells
-mutation - autoimmune disease
IPEX

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14
Q

CD25

A

on T-reg cells

-polymorphisms - MS and other autoimmunities

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15
Q

deletion by activation induced cell death

A

CD4 T cells that recognize self antigen - apoptosis

  • proapoptotic Bim - mito patway
  • also Fas-FasL death receptor pathway
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16
Q

FAS

A

mutation - autoimmune lymphoproliferative syndrome

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17
Q

immune-privileged sites

A

antigens hidden from immune syndrome
-testis, eyes, brain

released with trauma - elicit immune response

post-traumatic orchitis and uveitis

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18
Q

mechanisms of autoimmunity

A

genes and environmental triggers

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19
Q

AIRE

A

expression of self-antigens in thymus

-aid with central tolerance

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20
Q

PTPN-22

A

genetic association with autoimmunity

  • rheumatoid arthritis, type I DM
  • inability to control tyrosine kinase
  • excessive lymphocyte activation
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21
Q

NOD-2

A

genetic association with autoimmunity

  • crohns disease
  • cytoplasm sensor for microbes
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22
Q

B cell negative feedback

A

Fc receptor for IgG antibodies

-switches off antibody production

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23
Q

infection and autoimmunity

A

may upregulate costimulators on APCs

also, some microbes may have antigens that are similar to self-antigens (molecular mimicry)

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24
Q

molecular mimicry

A

rheumatic heart disease

-streptococcal proteins similar to myocardial proteins

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25
Q

general features of autoimmunity

A

progressive

Th1 - M0 / Th17 - neutrophils and monocytes

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26
Q

systemic lupus erythematous

A

SLE

  • autoantibodies
  • injury to skin, joints, kidney, serosa, etc.

predominantly in woman of childbearing age
usually arises in 20s or 30s

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27
Q

autoantibodies in SLE

A

diverse

  • ANAs - antinuclear antibodies
  • against DNA, histones, non-histones bound to RNA, nucleolar antigens

-also - to blood cells
-antiphospholipid
-

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28
Q

patterns of nuclear fluorescence

A

1 - homogenous diffuse nuclear staining
2 - peripheral staining
3 - speckled pattern
4 - nucleolar pattern

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29
Q

homogenous diffuse nuclear staining

A

chromatin/histones

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30
Q

peripheral staining

A

double strand DNA

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31
Q

speckled pattern staining

A

non-DNA nuclear constituents

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32
Q

nucleolar pattern staining

A

RNA

-systemic sclerosis

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33
Q

criteria for SLE

A

must meet 4 of 11

  • malar rash - butterfly rash on cheeks
  • discoid rash
  • photosensitivity
  • oral ulcer
  • arthritis
  • serositis (pleuritis / pericarditis)
  • renal disorder
  • neuro disorder
  • hematologic disorder
  • immuno disorder
  • antinuclear antibodies
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34
Q

diagnostic for SLE

A

autoantibodies to smith antigen and double strand DNA

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35
Q

false positive for syphilis

A

SLE patients

-autoantibody for beta-2 glycoprotein complex also binds cardiolipin (in syphilis test)

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36
Q

antiphospholipid antibodies

A

in 40-50% SLE patients
-epitopes of plasma proteins revealed when proteins in complex with phospholipids

lupus anticoagulant (in vitro)
-however, individuals in hypercoagulable state**

results in secondary antiphospholipid antibody syndrome

37
Q

genetics of SLE

A

defect in B cell tolerance

also, deficiency in complement components favoring tissue deposition

38
Q

UV light

A

exacerbates SLE

39
Q

sex hormones

A

influence on SLE

40
Q

drugs that induce SLE response

A

hydralazine
procainamide
D-penicillamine

41
Q

tissue injury in SLE

A

type III - antibody-mediated

type IV - immune complex mediated

42
Q

LE cell

A

any phagocytic leukocyte that has engulfed the denatured nucleus of injured cell
-seen in SLE

43
Q

characteristic lesions in SLE

A

blood vessels, kidneys, CT, skin

44
Q

blood vessels and SLE

A

fibrinoid depositi in walls

45
Q

kidney and SLE

A

lupus nephritis

-immune complexes in glomeruli

46
Q

5 patterns of lupus nephritis

A 
class 1 - minimal mesangial
class 2 - mesangial proliferative
class 3 - focal proliferative
class 4 - diffuse proliferative
class 5 - membranous
47
Q

mesangial lupus glomerulonephritis

A

no or slight (class 1) or moderate (class 2) mesangial proliferation with deposits of Ig and complement

no involvement of capillaries

48
Q

focal proliferative glomerulonephritis

A

class 3
fewer than 50% involvement of glomeruli
-crescent formation, fibrinoid necrosis, endothelial/mesangial proliferation
-hematuria/proteinuria

49
Q

diffuse proliferative glomerulonephritis

A

class 4
greater than 50% involvement of glomeruli
-crescent formation, fibrinoid necrosis, endothelial/mesangial proliferation
-hematuria/proteinuria

50
Q

membranous glomerulonephritis

A 
class 5
thickened capillary walls

severe proteinuria and nephrotic syndrome

51
Q

subepithelial deposits of antibody/complement in glomeruli

A

membranous glomerulonephritis

between BM and visceral epithelial cells

52
Q

subendothelial deposits of antibody/complement in glomeruli

A

proliferative glomerulinephritis
-wire loops

between endothelium and BM

53
Q

wire loops

A

subendothelial deposits create thickened capillary wall

54
Q

skin and SLE

A

facial malar area - butterfly

also bullae, urticaria, ulcerations, maculopapular lesions

worsened with UV exposure

deposition of Ig and complement

55
Q

joints and SLE

A

nonerosive synovitis

56
Q

CNS and SLE

A

antibodies against synaptic membrane

57
Q

serosa and SLE

A

acute - fibrous exudate on surface

later - thickened, opaque and fibrous

58
Q

cardiovascular and SLE

A

non-bacterial verrucous endocarditis

  • affects valves
  • 1-3mm warty deposits on any heart valve
59
Q

infective endocarditis

A

larger deposits than SLE

60
Q

spleen and SLE

A

splenomegaly
capsular thickening
follicular hyperplasia

61
Q

lungs and SLE

A

pleuritis and pleural effusion

62
Q

clinical features of SLE

A

typically young woman

  • butterfly rash on face, fever, pain in joints
  • photosensitivity
  • ANAs found
  • anemia / thrombocytopenia
  • mental aberrations
  • CAD
63
Q

course of SLE

A

rare/acute - die within weeks
-with therapy - flare ups and remission

  • during flare-up - hypocomplementemia
  • Tx - corticosteroids (immunosuppressant)

common cause of death - renal failure

64
Q

chronic discoid lupus erythematous

A

skin manifestations of SLE - but no systemic problems

skin plaques - face and scalp
positive ANA test

rarely have double strand DNA antibodies

65
Q

subacute cutaneous lupus erythematous

A

predominant skin involvement
-widespread and nonscarring

mild systemic symptoms

antibodies to SS-A
HLA-DR3 genotype

66
Q

drug-induced lupus erythematous

A

hydralazine, procanamide, isonaizid, d-penicillamine

-development of ANAs

renal and CNS involvement are rare

lots of antibodies for histones

HLA-DR4 genotype

67
Q

rheumatoid arthritis

A

chronic inflammatory
-affects joints
-also skin, vessels, lungs, heart
-

68
Q

sjogren syndrome

A

keratoconjunctivitis sicca (dry eyes) and xerostomia (dry mouth)

destruction of salivary and lacrimal glands

autoantibodies against SS-A and SS-B
-both are ribonucleoprotein antigens

often associated rheumatoid arthritis

69
Q

sicca syndrome

A

decreased saliva

70
Q

rheumatoid factor

A

antibody against IgG

71
Q

lymphoma vs. intense reactive chronic inflammation

A

look at clonality of cells present

  • monoclonal - lymphoma
  • polyclonal - inflammation
72
Q

clinical features of sjogren syndrome

A

women aged 50-60

keratoconjunctivitis - causes blurry vision, burning, itching
xerostmia - difficulty swallowing food, decreased taste, cracks in mouth

parotid gland enlargement

rare - glomerular lesions
-but do see phosphaturia, uricosuria, renal tubular acidosis

73
Q

mikulicz syndrome

A

combination of lacrimal and salivary gland inflammation

74
Q

diagnosis of sjogren syndrome

A

Bx of lip - minor salivary glands

75
Q

node in sjogren

A

initially T and B cells
tendency to more B cells

can lead to marginal zone lymphoma (B cell malignancy)

76
Q

systemic sclerosis

A

chronic inflammation
widespread damage to small vessels
progressive interstitial and perivascular fibrosis in skin

excessive fibrosis throughout body

77
Q

slceroderma

A

aka systemic sclerosis

78
Q

diffuse scleroderma

A

widespread skin involvement at onset and progression to visceral involvement early

79
Q

limited scleroderma

A

skin involvement only fingers, forearms, face
-visceral involvement late

some patients develop CREST

80
Q

CREST syndrome

A

associated with limited scleroderma

calcinosis - deposits in soft tissue
raynauds phenomenon - reduced color in digits/toes
esophageal dysmotility - difficulty swallowing
sclerodactylyl - local thickening of skin of digits/toes
tenalgiectasia - blood vessels near surface of skin

81
Q

pathology of scleroderma

A

CD4 T cell response to antigen in skin

ANAs - against DNA topoisomerase I (pulmonary fibrosis) and anticentromere antibody (CREST)

82
Q

marfan syndrome

A

mutation of fibrillin I gene

83
Q

skin and scleroderma

A

begins in fingers goes centrally
-edema and perivascular infiltrates with CD4 cells

fibrosis of dermis
-subQ calcifications

clawlike hands

84
Q

alimentary tract and scleroderma

A

fibrosis of muscles in esophagus

GERD can occur

85
Q

lungs and scleroderma

A

pulmonary HTN and interstitial fibrosis

86
Q

clinical features of scleroderma

A

50-60 year old
-more likely female

skin thickening, raynauds phenomenon, dysphagia, respiratory difficulty

CREST - in limited scleroderma
-pts with CREST usually have limited involvement of skin - only fingers, forearms, face

87
Q

inflammatory myopathies

A

inflammation of skeletal muscles

dermatomyositis
polymyositis
inclusion-body myositis

88
Q

mixed CT disease

A

mix of SLE, systemic sclerosis, and polymyositis

ANAs to U1 ribonucleoprotein

89
Q

polyarteritis nodosa

A

necrotizing inflammation of vessels

PBD I Exam 2 (18 decks)

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