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CMOD > Robbin's Autoimmune Diseases > Flashcards

Robbin's Autoimmune Diseases Flashcards

1
Q

autoimmunity is carried out by what two major mechanisms

A

Auto-antibodies

self reactive t cells

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2
Q

Systemic diseases that are caused by autoantibodies and immune complexes are primarily characterized as

A

connective tissue disorders
or
collagen vascular disorders
*even though autoantibodies are not directly targeting blood vessels or connective tissue

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3
Q

What is immunologic tolerance

A

unresponsiveness to an antigen that is induced by exposure specific lymphocytes to that antigen

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4
Q

antigen-induced deletion of self-reactive t and b cells during maturation

A

central tolerance

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5
Q

Gene responsible for self tolerance in central tolerance

A

AIRE

*defect is APCED or something

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6
Q

Which cells can undergo receptor editing

A

B cells that were initially self-reactive

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7
Q

Mechanisms by which Self reactive T cells are deleted from the repertoire in the periphery

A

Anergy
Suppression by TREGS
Activation induced cell-death

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8
Q

Define Anergy

A

Anergy–> lack of B7 costimulatory signal–> T cell then undergoes apoptosis

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9
Q

what goes on in Suppression by TREGS

A

t regs bind to self antigens–> they supress cd4/cd8 t cells that are binding locally to self-peptides on MHC molecules by secreting IL-10 and TGF-Beta
end result is dampens t cell response, also competes for binding of B7 on apc

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10
Q

T regs express what?

A

CD25

*one of the Il-2 receptor subunits, require IL-2 for their proliferation and survivial

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11
Q

Tregs express what

A

Foxp3

*responsible for the development of regulatory cells

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12
Q

Mutations in Foxp3 result in what disease

A

IPEX

diabetes, diarrhea, eczematous dermatitis

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13
Q

WHat goes on in Activation induced cell death?

A

FasR (member of TNF family) is activated by FasL upon self-recognition–> apoptosis

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14
Q

Activation induced cell death is important for what?

A

deletion of self-reactive B cells by FasL expressed on T cells

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15
Q

defect in Fas, FasL or Caspase 10 results in what

A

ALPS

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16
Q

Most autoimmune disease cannot be explained by

A

defects in a single gene

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17
Q

several autoimmune disease are linked to the

A 
HLA locus--> especially class II gene
Dr and DQ
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18
Q

RA locus

A

HLA DR4 (HLA DRB1)

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19
Q

Ankylosing Spondylitis locus

A

HLA B27

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20
Q

Rheumatic heart disease is caused by

A

molecular mimicry of GAS to host tissue–> viruses and other microbes share cross-reactive antigens with host tissue–> in an attempt to make an immune response to the pathogen, the host is left with self-reactive antibodies that bind to “immitation” epitopes and recruit inflammatory cells–> damage of the tissue

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21
Q

epitope spreading

A

autoimmune attack only furthers the autoimmune response–> damage to self tissues uncovers host antigens in a newly immunogenic form–> body makes even more anti-self responses

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22
Q

Main causes/characterizations of of LUPUS

A

Loss of self tolerance mechanisms, leading to production of a large number of autoantibodies that can damage tissue directly or in the form of immune complex deposits

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23
Q

main areas that SLE attacks

A

skin, kidneys, serosal membranes, joints and heart

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24
Q

Lupus is associated with an enormous array of

A

Anti Nucelar Antibodies–this is very sensitive but 50/50 specific

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25
Q

ratio of SLE female:male

A

9:1

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26
Q

race most likely to have lupus

A

Black women

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27
Q

Loci for SLE

A

HLA DR2 and HLA DR3

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28
Q

Pathogenis of SLE involes

A
  1. susceptibility of genes

2 external TRIGGERS

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29
Q

genetic factors for SLE(3)

A

Familial association
HLA association
genetic deficiencies of C1q, C2, C4–>defective clearance of immune complexes and apoptotic cells debris

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30
Q

Environmental factors of SLE (4)

A

UV radiation
Smoking
Sex hormones
Drugs–> procainimide and hydralazine–> SLE-like disease without glomerulonephritis

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31
Q

Immunologic Abnormalities in SLE–>(3)

A
  1. Abnormally large amounts of Type I interferons– AB
  2. TLR signals TLR 9 DNA and TLR 7 RNA–> activate self-specific B cells
  3. Failure of B cell tolerance
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32
Q

Model fo SLE sequence

A
  1. UV radiation–>apoptosis
  2. defective clearance
  3. unable to maintain self-tolerance
  4. host picks up self nuclear-antigens and makes and immune response to it
  5. autoantibodies bind to self-tissue, activate compliment and phagocytes eat host–> more cell death
  6. net result is cyclic antigen release and immue activation resulting in the production of high-affinity autoantibodies
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33
Q

Auto Ab’s seen in SLE

A

ANA’s
Ab’s against RBC’s
Anti-phospholipid Ab’s

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34
Q

Types of ANA’s

A 
Ab's against
DNA
Histones
nonhistone proteins bound to RNA
nucleolar antigens
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35
Q

Are ANA’s diagnostic for SLE

A

NO
IFA testing is 95% senstive but only like 50% specfiic
ANA’s can be in ppl without SLE

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36
Q

Specific assays for SLE

A 
dsDNA
Smith antigens (Sm)
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37
Q

Most organ damage in SLE is caused by

A

Immune-Complex deposition

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38
Q

During a SLE flair pt.s will have decreased levels of what in their serum analysis

A

C3-C4–> because it is a Type III hypersensitivity

–> Ab-SelfAntigen complex is having compliment fixed on it so that is can be taken up by phagocytes

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39
Q

SLE, as it related to ORGAN DAMAGE can be classified as what type of Hypersensitivity

A

Type III– Immune Complex

40
Q

SLE as it related to DESTRCUTION OF RED BLOOD CELLS can be classified as what type of hypersensitivity

A

Type II- Altered Host

41
Q

Disease related to Anti-dsDNA

A

SLE diagnostic

42
Q

Anti-Sm

A

SLE diagnostic

43
Q

Anti-RNPU1

A

SLE, mixed connective tissue disorder

44
Q

Anti SS-A (Ro) and anti SS-B (La

A

Sjrogen, SLE

45
Q

Anti SCL 70

A

Systemic Sclerosis–> this is diagnostic

46
Q

Anti Topo 1

A

Scleroderma

47
Q

Anti histones

A

SLE

48
Q

Anti-centromere

A 
limited scleroderma (CREST), 
Systemic scleroderma--> diagnostic
49
Q

Anti phospholipid

A

SLE

50
Q

Autoantibodies against phospholipids result in SLE pt.’s to exhibit

A

–>spontaneous abortion

thrombotic episodes–> but actually this interferes with clotting tests and are called lupus anticoagulants

51
Q

Type II hypersensitivity involved with SLE

A

auto-ab’s to RBC’s, Leukocytes, and platelets==> resulting in many cytopenias

52
Q

LE bodies/Hematoxylin bodies

A

nuclei of damaged cells bound to ANA’s

53
Q

LE cells

A

nuclei + ANA that has been taken up by a phagocyte

54
Q

SLE–> can lead to what involving Blood Vessels

A

acute necrotizing vasculitis
–> ANY tissue, small arteries and arterioles
necrosis and fibrinois deposition of within vessel walls containing antibody, DNA, compliment frags, fibrinogen
also perivascular leukocyte infiltrate is commonly seen

55
Q

Most serious form of SLE renal lesions

35-60%

A 
Diffuse Lupus Nephritis (IV)-- over half glomeruli
>MARKED GLOBAL INCREASE IN CELLULARITY
> more than half glomeruli involved
>bowman's space is narrowed
>wire loops--> AB complexes
>hematuria, proteinuria
>renal insufficiency
>HYPERTENSION
56
Q

FOCAL LUPUS NEPHRITIS

20%-35%

A

NOT AS BAD–> PRESENT IN LESS THAN HALF OF THE GLOMERULI AND MAY BE RESTRICTED TO ONLY A PORTION OF A GIVEN GLOMERULUS

57
Q

MEMBRANOUS LUPUS NEPHRITIS

A

THICKENING OF THE CAPILLARY WALL DUE TO DEPOSITION OF SUB-ENDOTHELIAL IMMUNE COMPLEXES

58
Q

SLE NEPHRITIS IS MEDIATED BY WHICH AB ISOFORM

A

IgG

59
Q

Advancing sclerotic lupus nephritis

A

complete sclerosis of over 90% of the glomeruli

corresponds to clinical ESRD

60
Q

Skin finding with SLE

A

MALAR rash from photosensitivity

61
Q

Histo findings of SLE skin

A

> LIQUEFACTIVE DEGENERATION OF BASAL LAYER
EDEMA AND DERM-EPI JUNCTION
MONONUCLEAR INFILTRATES AROUNG BLOOD VESSELS AND SKIN APPENDAGES

62
Q

SLE findings in the spleen

A

ionion skin lesions–> thickening of penicilliary arteries and perivascular fibrosis

63
Q

SLE affects the heart mainly in the form of

A
  • -> pericarditis
  • ->libmann sacks endocarditis (non-bacterial verrucous endocarditis)
  • -> CAD–> from immune complexes lodging in arteries
64
Q

Most common CNS lesion involved with SLE

A

small vessel angiopathy with nononflammatory intimal proliferation–>sometimes there are neuropscyological symptoms

65
Q

anti-cd20 tx for SLE

A

rituximab

66
Q

pt.’s with lupus are at increased risk of

A

opportunistic infection

67
Q

Most common causes of death with lupus

A
  1. renal failure
  2. intercurrent infections
  3. CV disease
68
Q

Characteristics of Sjogren Syndrome
can occur by itself (sicca syndrome)
or in conjunction with another autoimmune disease– usually RA

A 
Dry eyes (keratoconjunctivitis)
Dry mouth (xerostomia)

*immune mediated destruction of lacrimal and salivary glands

69
Q

Most likely mode of pathogenesis for Sjogren Syndrome

A

CD4 t cells react against an unknown antigen in the ductal epithelial cells of he exocrine glands

70
Q

Ab’s associated with Sjogren

A

ANA, RF, SS-A, SS-B,

But none of this is diagnostic

71
Q

is there a viral cause suspected with sjogren

A

yes–> which one it is is unknown

72
Q

Histo findings of Sjogren

A

intense lymphocyte infiltration (CD4) of gland tissue and plasma cell infiltration
occasionally forming lymphoid follicles with a germinal center

73
Q

Sjogren pt.’s with primary Sicca syndrome and high levels of SS-A ab’s develop what

A

extraglandular problems involving skin, cns, kidneys etc.

74
Q

about 60% of pt.’s with Sjogren have what

A

another autoimmune disease

75
Q

Pt.’s with Sjogren’s also are susceptible to what

A

40 times more likely to develop non-hodgkin B cell lymphoma–> from robust polyclonal B cell proliferation

76
Q

general characteristics of Scleroderma

A
  1. excessive fibrosis of multiple tissue
  2. obliterative vascular disease
  3. production of many autoab’s
77
Q

2 main types of systemic scleroderma

A

> Diffuse

>Limited

78
Q

characteristics of diffuse scleroderma

A

> initial widespread skin involvement

>rapid visceral progression

79
Q

Characterstics of limited scleroderma

A

> mild skin involvement (fingers and face only)

>late visceral involvement

80
Q

Limited scleroderma is also known as

A

CREST syndrome

81
Q

findings of CREST syndrome

A 
Calcinosis
Raynaud phenomenon
Esophageal dysmotility
Sclerodactyly
Telangectasia
82
Q

what is thought to preclude Scleroderma pathogenic sequence

A

injury to endothelial cells–> migration of activated T cells to injured tissues

83
Q

domminant self-reactive cell type in scleroderma

A

> TH2–» responds to endothelial damage and recognizes self antigen
then releasesfcytokines–>induce alternative macrophage activation and collagen deposition by fibroblasts

84
Q

Cytokines released by Th2 cells involving scleroderma

A

IL 10 and TGFBeta–> tells fibroblasts to do their thang–> result in fibrosis

85
Q

Ultimate result of Scleroderma autoimmune sequence

A

ischemic injury

86
Q

what leads to ischemic injury in scleroderma

A

endothelial cell proliferation and intimal fibrosis–> leads to narrowing of the small vessels–> ischemic injury over time

87
Q

ANA’s unique to SS

A

anti topo 1 ab (anti Scl-70)

anticentomere ab

88
Q

ANA specific to CREST (limited ss)

A

anti CENTROMERE

89
Q

ANA ab specific to diffuse ss

A

anti topo 1

90
Q

sum up the skin histopath is SS

A

thickening of the dermis due to deposition of compact collagen and thinning of the epidermis, atrophy of dermal appendages

91
Q

loss of blood flow from SS can result in

A

autoamputation

92
Q

Effects of SS on GI tract

A

replacement of muscularis with collagen results in “rubber-hose” like loss of flexibility–> usualy the distal 1/3 of esophagus is affected and the result is esophageal dysmotility

93
Q

most diasgnostic indicator for SLE

A

CBC

94
Q

SS can lead to GERD and then

A

Barrett’s esophagus

squamous–>columnar

95
Q

secondary pulmonary hypertenison is associate with what disease

A

SS

96
Q

pt.s with Mixed connective tissue disease have high titers of what

A

U1RNP

97
Q

two other features of mixxed connective tissue diease

A
  1. scarcity of renal disease

2. great response to corticosteroids

CMOD (19 decks)

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