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immunology and serology > Autoimmunity (Introduction, Systemic Autoimmune Disease) > Flashcards

Autoimmunity (Introduction, Systemic Autoimmune Disease) Flashcards

1
Q

introduced “horror autotoxicus” or
“fear of self-poisoning” in 1900s

A

Paul Ehrlich

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2
Q

Immune responses are targeted toward self-antigens resulting to organ and tissue damage

A

Autoimmune diseases

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3
Q

Autoimmune diseases can be caused by

A

T-cell–mediated immune responses
Autoantibodies directed against host antigens

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4
Q

ETIOLOGY of autoimmunity

A

Self-Tolerance
Genetics
Other endogenous & environmental factors

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5
Q

ability of the immune system to accept self-antigens and not initiate a response against them

A

Self-Tolerance

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6
Q

a state of immune unresponsiveness that is directed against a specific antigen, in this case, a self-antigen

A

Immunologic Tolerance

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7
Q

2 levels of Immune Tolerance

A

Central Tolerance
Peripheral Tolerance

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8
Q

immune tolerance that occurs in central or primary lymphoid organs (thymus & BM)

A

Central Tolerance

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9
Q

involves negative and positive selection during T cell maturation

A

Central Tolerance

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10
Q

involves receptor editing in B cells

A

Central Tolerance

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11
Q

result from anergy caused by absence of costimulatory signal from an antigen-presenting cell (APC) or binding of inhibitory receptors such as CTLA-4

A

Peripheral Tolerance

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12
Q

specific state of unresponsiveness to the antigens

A

anergy

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13
Q

molecule that prevents T-cell activation

A

CTLA-4

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14
Q

in terms of genetics, autoimmunity is prevalent in these groups

A

family members and among monozygotic (genetically identical) twins

than dizygotic (non-identical) twins or siblings

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15
Q

Other Endogenous and Environmental Factors

A

Hormonal Influence
Tissue Trauma and Release of Cryptic Antigens
Microbial Infections

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16
Q

women are ___ more likely to acquire an
autoimmune disease than men

A

2.7x

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17
Q

___ of pt. with autoimmune dse are females

A

78%

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18
Q

female hormones that may place women at a greater risk for developing autoimmune dse

A

estrogen, androgen, prolactin

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19
Q

“immunologic tolerance” some self-antigens
may be cryptic, or hidden within host’s tissue

A

Tissue Trauma and Release of Cryptic Antigens

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20
Q

tissue damage could be caused by factors:

A

▪ infections
▪ contact with environmental toxins
▪ physical injury from UV radiation exposure

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21
Q

Mechanisms of Microbes in triggering autoimmune response

A

1) Molecular mimicry
2) Bystander effect
3) Superantigens
4) Epigenetics and Modification of Self-Antigens
5) Interactions Between Factors

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22
Q

many bacterial or viral agents contain antigens that closely resemble the structure or amino acid
sequence of self-antigens

A

Molecular mimicry

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23
Q

example of molecular mimicry

A

gram-positive bacterium S. pyogenes and
rheumatic fever (scarlet fever/pharyngitis)

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24
Q

production of antibodies to the M protein and N-acetyl glucosamine components of the bacteria, which crossreact with cardiac myosin, causing damage to the heart

A

Molecular mimicry in scarlet fever or pharyngitis

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25
Q

microorganism can induce a local inflammatory
response that recruits leukocytes and stimulates
APCs to release cytokines that nonspecifically
activate T cells

A

Bystander effect

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26
Q

proteins produced by various microbes that bind to both class II MHC molecules and TCRs, regardless of their antigen specificity

A

Superantigens

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27
Q

example of superantigen

A

staphylococcal enterotoxins
Epstein-Barr virus (EBV) and cytomegalovirus (CMV)

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28
Q

can cause polyclonal activation of B cells

A

Epstein-Barr virus (EBV) and cytomegalovirus (CMV)

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29
Q

superantigens produced can act as potent T-cell mitogens by activating a large number of T cells with different antigen specificities

A

staphylococcal enterotoxins

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30
Q

modifications in gene expression that are not caused by changes in original DNA sequence

A

Epigenetics

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31
Q

citrullination of collagen leads to

A

RA

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32
Q

glycosylation of myelin leads to

A

Multiple Sclerosis

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33
Q

complex interactions between genetic and environmental factors may cause break in
immunologic tolerance, autoreactive T cells recognize and proliferate in response to self-antigens and B cells develop into plasma cells that secrete autoantibodies

A

Interactions Between Factors

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34
Q

a chronic systemic inflammatory disease

A

SLE

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35
Q

peak age of onset of SLE

A

20-40 yo

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36
Q

W:M of SLE

A

9:1

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37
Q

5 yr survival rate of SLE

A

90%

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38
Q

etiology of SLE

A

complex interactions between environmental factors, genetic susceptibility, and abnormalities within immune system

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39
Q

genetic defects in genes coding for these increases the chance of developing lupus

A

HLA-A1, B8, DR3

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40
Q

result in uncontrolled autoreactivity of T and B cells, which leads to production of numerous autoantibodies in SLE

A

polymorphisms in genes
genes coding for various cytokines
genes involved in signaling of innate immune response

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41
Q

What will be the result if there is a hidden or cryptic self antigens?

A

T and B lymphocytes are shielded and not educated to become tolerant. (No recognition since hidden)

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42
Q

changes at protein level during epigenetics and modification of self-antigens

A

post translational modification
biochemical processes:
acetylation
lipidation
citrullination
glycosylation

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43
Q

Associated autoantibodies in SLE

A

Ab to dsDNA, histones, and other nuclear components
Autoantibody to lymphocytes
Autoantibody to erythrocytes
Autoantibody to platelets
Autoantibody to ribosomal components
Autoantibody to endothelium
Phospholipid Ab
RF

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44
Q

present in 70% of patients with lupus

A

Antibodies to dsDNA (AntidsDNA)

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45
Q

Ab highly specific for SLE

A

Antibodies to dsDNA (AntidsDNA)

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46
Q

found in immune complexes that are deposited in
organs such as kidneys and skin of patient with SLE

A

AntidsDNA
complement protein

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47
Q

most pathogenic, forms intermediate size complexes that become deposited in glomerular basement membrane (GBM)

A

Accumulation of IgG to dsDNA

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48
Q

activate complement and initiate an inflammatory response

A

Immune complexes

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49
Q

attracted to sites of inflammation and release cytokines that perpetuate the response, resulting in tissue damage

A

Leukocytes

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50
Q

Antibodies to RBCs indicates

A

hemolytic anemia

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51
Q

Antibodies to platelets indicates

A

thrombocytopenia

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52
Q

Antibodies to endothelial cells indicates

A

inflammation of blood vessels and vascular damage in lupus, vasculitis and neuropsychiatric symptoms

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53
Q

Phospholipid antibodies indicates

A

increased miscarriage, stillbirth, and preterm delivery in pregnant women with lupus

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54
Q

occurs in up to 8% of babies born to pregnant women with SLE, associated with antibodies to the nuclear antigens, SS-A/Ro and SS-B/La

A

Neonatal lupus

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55
Q

nuclear antigens where neonatal lupus antibodies are associated

A

SS-B/La
SS-A/Ro

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56
Q

serious complication that occurs in 2% of fetuses whose mothers have anti–SS-A antibodies

A

In utero heart block

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57
Q

clinical symptom in 90% of patients with SLE

A

polyarthralgias or arthritis

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58
Q

symmetric and involves small joints of the hands,
wrists, and knees

A

arthritis

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59
Q

may appear on any area of the body exposed to UV light in 80% of SLE patients

A

erythematous rash or butterfly rash

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60
Q

found in 50% of SLE patients

A

renal involvement leading to renal failure

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61
Q

end stage renal dse, deposition of immune complexes in subendothelial tissue and thickening of basement membrane

A

nephritis, diffuse proliferative glomerulonephritis

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62
Q

symptoms usually disappear once the drug is discontinued; milder form of the disease; manifested as fever, arthritis, or rashes; kidneys are rarely involved

A

drug-induced SLE

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63
Q

Systemic Lupus International Collaborating Clinics criteria (2012)

A

Clinical criteria
Immunologic criteria

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64
Q

give examples of clinical criteria and immunologic criteria

A

Clinical criteria
▪ acute cutaneous lupus
▪ chronic cutaneous lupus
▪ oral ulcers
▪ non-scarring alopecia (hair thinning/fragility)
▪ synovitis
▪ serositis
▪ renal involvement
▪ neurological symptoms
▪ hemolytic anemia
▪ leukopenia
▪ thrombocytopenia

Immunologic criteria
▪ elevated antinuclear antibody titer
▪ elevated anti-dsDNA titer
▪ presence of antibody to the Sm nuclear antigen
▪ presence of antiphospholipid antibody
▪ low complement levels
▪ + direct Coombs’ test in hemolytic anemia absence

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65
Q

what is needed to be classified as having SLE

A

4 of the 17 criteria, including at least 1 clinical criterion and 1 immunologic criterion

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66
Q

treatment for SLE with mild symptoms

A

high dose of aspirin (brings relief)

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67
Q

treatment for skin manifestations of SLE? what is the action?

A

Antimalarials (hydroxychloroquine/chloroquine and topical steroids)

Antimalarial drugs – inhibit signaling of TLR 7, 8, and 9

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68
Q

treatment for acute fulminant (severe & sudden) lupus, lupus nephritis, or CNS complications because these suppress immune response and lower antibody titers

A

Systemic corticosteroids

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69
Q

key or goal to successful treatment of SLE

A

prevent organ damage
achieve remission

70
Q

SLE Laboratory Diagnosis

A

complete blood count (CBC)
platelet count
urinalysis
quantification of complement proteins (C3) - low
detection of specific autoantibodies

71
Q

autoantibodies directed against antigens in the nuclei of mammalian cells

A

Anti-Nuclear Antibodies (ANA)

72
Q

12 types of ANA

A
  1. Double-stranded DNA (dsDNA) antibodies
  2. Antihistone Antibodies
  3. Nucleosome antibodies
  4. Antibody to Sm antigen
  5. Anti-RNP (ribonucleoproteins) antibody
  6. Anti–SS-A/Ro
  7. Antibodies to SS-B/La
  8. Antibodies to both SS-A/Ro and SS-B/La
  9. Antibody to fibrillarin
  10. Antibodies to RNA polymerase
  11. Antibodies to the PM-1 antigen (aka PM/Scl)
  12. Anticentromere antibodies
73
Q

produce a peripheral or a homogeneous staining pattern on indirect immunofluorescence (IIF)

A

Double-stranded DNA (dsDNA) antibodies

74
Q

ANAs that produce homogenous IF pattern

A

Antihistone Antibodies
Anti-DNP
Anti-dsDNA
Nucleosome antibodies
Anti-PM-1 antigen (PM/Scl)

75
Q

nucleoproteins; essential components of chromatin

A

Histones

76
Q

5 major classes of histones

A

H1, H2A, H2B, H3, H4

77
Q

Antibodies to H2A and H2B is detected in almost all
patients with

A

drug-induced lupus

78
Q

supports the diagnosis of drug-induced lupus

A

antihistone antibody alone or combined with antibody to ssDNA

79
Q

Also found in RA, Felty’s syndrome, Sjögren’s syndrome, systemic sclerosis, and primary biliary cirrhosis, but the levels are usually lower

A

Antihistone Antibodies

80
Q

stimulated by DNA-histone complexes (nucleosomes, or deoxyribonucleoprotein [DNP])

A

Nucleosome antibodies

81
Q

directed only against the complexes and not against DNA or the individual histones

A

Nucleosome antibodies

82
Q

found in 85% of patients with SLE and their levels correlate with disease severity

A

Nucleosome antibodies

83
Q

associated with uridine-rich RNA

A

ENA – extractable nuclear antigens

84
Q

found in only 20 – 40% of patients with SLE, depending on the race of the population

A

Antibody to Sm antigen

85
Q

first described in a patient named Smith

A

anti-Sm antibody

86
Q

pattern of anti-Sm antibody in IIF

A

coarse speckled

87
Q

pattern of anti-RNP antibody in IIF

A

coarse speckled

88
Q

detected in 20% to 30% of patients with SLE, but are also found at a high titer in individuals with mixed connective tissue disease and in lower levels in patients with other autoimmune rheumatic diseases such as systemic sclerosis, Sjögren’s syndrome, and RA

A

Anti-RNP (ribonucleoproteins) antibody

89
Q

appears in 24 – 60% of patients with SLE

A

Anti–SS-A/Ro

90
Q

closely associated with presence of nephritis, vasculitis, lymphadenopathy, photosensitivity, and hematologic manifestations such as leukopenia

A

Anti–SS-A/Ro

91
Q

found in only 9 – 35% of patients with SLE and all of these have anti–SS-A/Ro

A

Antibodies to SS-B/La

92
Q

most often found in pt. w/ cutaneous manifestations of SLE, esp. photosensitivity dermatitis

A

Antibodies to SS-B/La

93
Q

pattern produced by SS-A/Ro and SS-B/La in IIF

A

finely speckled

94
Q

Ab that can cross placenta and associated with neonatal lupus

A

Antibodies to both SS-A/Ro and SS-B/La

95
Q

prominent structure within nucleus where transcription, processing of ribosomal RNA and assembly of ribosomes takes place

A

nucleolus

96
Q

Ab common in systemic sclerosis (aka scleroderma)

A

Antibody to fibrillarin

97
Q

pattern produced by Antibody to fibrillarin in IIF

A

clumpy nucleolar fluorescence

98
Q

Antibodies associated with scleroderma

A

Antibodies to:
RNA polymerase
fibrillarin
PM-1 antigen

99
Q

pattern produced by Antibodies to RNA polymerase in IIF

A

speckled nucleolar pattern

100
Q

Antibodies to the PM-1 antigen is aka

A

PM/Scl

101
Q

Ab found in polymyositis and systemic sclerosis

A

Antibodies to the PM-1 antigen (aka PM/Scl)

102
Q

bind to proteins in the middle region of a chromosome where sister chromatids are joined

A

Anticentromere antibodies

103
Q

directed against 3 centromere antigens of molecular weights 16kDa, 80kDa, and 120kDa

A

Anticentromere antibodies

104
Q

found in 50–80% of patients with CREST syndrome, a subset of scleroderma

A

Anticentromere antibodies

105
Q

5 major features of CREST syndrome

A

calcinosis
Raynaud’s phenomenon
esophageal dysmotility
sclerodactyly
telangiectasia

106
Q

pattern produced by Anticentromere antibodies in IIF

A

discrete speckled staining in cell nuclei

107
Q

Methods of ANA Detection

A
  1. IIF
  2. immunoperoxidase staining
  3. enzyme-linked immunosorbent assay (ELISA)
  4. microsphere multiplex immunoassays (MIA)
  5. radioimmunoassay (RIA)
  6. immunodiffusion
  7. immunoblotting (Western blot)
  8. dot blot
  9. immunoelectrophoresis
  10. microarray
108
Q

heterogeneous group of antibodies that bind to phospholipids alone or phospholipids complexed with protein

A

Antiphospholipid antibodies

109
Q

associated with deep-vein, arterial thrombosis and recurrent pregnancy loss

A

Antiphospholipid antibodies

110
Q

found in up to 60% of patients with lupus

A

Antiphospholipid antibodies

111
Q

identified by their ability to cause false-positive results in nontreponemal tests for syphilis, lupus anticoagulant assay, and immunoassays for antibodies to cardiolipin or other phospholipids

A

Antiphospholipid antibodies

112
Q

APTT may be prolonged, but is not corrected by mixing with normal plasma; factor assays may also need to be performed to rule out any factor deficiencies or factor-specific inhibitors

A

Antiphospholipid antibodies

113
Q

Rheumatoid Arthritis affects ___ of adult population

A

0.5 – 1.0%

114
Q

age affected by RA

A

25 and 55

115
Q

W:M of RA

A

3:1

116
Q

chronic, symmetric, and erosive arthritis of peripheral joints that can also affect multiple organs such as heart and lungs

A

Rheumatoid Arthritis

117
Q

Etiology of RA

A

HLA-DRB1 alleles or PTPN22 gene polymorphisms
cigarette smoking

118
Q

strongest environmental risk factor of RA

A

cigarette smoking

119
Q

caused by inflammatory process that results in bone and cartilage destruction

A

RA

120
Q

lesions show an increase in cells lining the synovial membrane and pannus formation

A

RA

121
Q

sheet of inflammatory granulation tissue that grows into the joint space and invades the cartilage

A

pannus

122
Q

RA occur due to the balance between?

A

proinflammatory and anti-inflammatory cytokines;
IL-1, IL-6, IL-17, and TNF-α

123
Q

trigger the release of matrix metalloproteinases from fibroblasts and macrophages; these enzymes degrade important structural proteins in the cartilage.

A

Proinflammatory cytokines

124
Q

local bone erosion is found in this condition wherein osteoclasts become overly activated in inflammatory environment of the joints

A

RA

125
Q

receptor activator of nuclear factor kappa-B ligand

A

RANKL

126
Q

induces the differentiation of osteoclasts and inhibits bone formation in conjunction with other cytokines and RANKL

A

TNF-α

127
Q

second major type of antibody associated with RA

A

antibodies to cyclic citrullinated proteins (anticyclic citrullinated peptide antibody [anti-CCP or ACPA])

128
Q

modifies amino acid arginine by replacing an NH2 group with a neutral oxygen

A

citrulline

129
Q

nonspecific symptoms of RA such as malaise, fatigue, fever, weight loss, and transient joint pain that begin in the small joints of hands and feet (joints are typically affected in a symmetric fashion) are usually present in the

A

MORNING

130
Q

RA inflammation left untreated may lead to

A

permanent joint dysfunction and deformity

131
Q

extra-articular manifestations of RA

A

subcutaneous nodules formation
pericarditis
lymphadenopathy
splenomegaly
interstitial lung disease
vasculitis

132
Q

most common cause of death with RA

A

CV disease due to acceleration of arteriosclerosis by proinflammatory cytokines

133
Q

treatment for RA

A

disease-modifying anti-rheumatic drugs (DMARDs)
methotrexate

134
Q

inhibits adenosine metabolism and T-cell activation

A

methotrexate

135
Q

key therapies for RA that blocks the activity of cytokine, TNF-α is classified into 2 categories

A

monoclonal antibodies to TNF-α
TNF-α receptors fused to an IgG molecule

136
Q

treatment: monoclonal antibodies to TNF-α

A

▪ infliximab
▪ adalimumab
▪ certolizumab
▪ golimumab

137
Q

treatment: TNF-α receptors fused to an IgG molecule

A

etanercept

138
Q

antibody most often tested to make an initial diagnosis for RA

A

Rheumatoid Factor (RF)

139
Q

an autoantibody, usually IgM class, that reacts with Fc portion of IgG; 70% to 90% of patients with RA test positive with this

A

RF

140
Q

T/F
RF can be found in patients with other connective tissue diseases such as SLE, Sjögren’s syndrome, scleroderma, mixed connective tissue disease, some chronic infections

A

T

141
Q

used for many years to detect RF

A

Manual agglutination tests using charcoal or latex particles coated with IgG

142
Q

presence of this precedes the onset of RA by several years, making it a better marker for early disease

A

antibody to cyclic citrullinated peptides (anti-CCP) - ELISA

143
Q

About __ of RF-negative patients are positive for anti-CCP

A

20 – 30%

144
Q

Specificity of anti-CCP + RF testing for RA

A

98 – 100%

145
Q

enable more accurate diagnosis of RA by allowing better differentiation from other forms of arthritis

A

anti-CCP + RF testing

(rheumatologists recommended)

146
Q

CRP, ESR, and complement component levels in RA

A

CRP and ESR: elevated
Complement components: normal/elevated (due to increased acute-phase reactivity)

147
Q

type of hypersensitivity of SLE

A

3

148
Q

levels correlate well with RA disease activity because levels reflect the intensity of inflammatory response

A

CRP

149
Q

aka Granulomatosis With Polyangiitis

A

Wegener’s Granulomatosis

150
Q

rare autoimmune disease involving inflammation of small- to medium-sized blood vessels, or vasculitis

A

Wegener’s Granulomatosis

151
Q

begins with localized inflammation of upper and lower respiratory tract

A

Wegener’s Granulomatosis

152
Q

majority of patients have renal involvement, which can range from mild glomerulonephritis with little functional impairment to severe glomerulonephritis that can rapidly lead to kidney failure

A

Wegener’s Granulomatosis

153
Q

involves pain and arthritis of the large joints, which is usually
symmetric but not deforming; skin lesions; ocular manifestations – potentially lead to vision loss

A

Wegener’s Granulomatosis

154
Q

Without treatment of this disease, more than 90% of patients will die within 2 years of diagnosis

A

Wegener’s Granulomatosis

155
Q

Etiology of Wegener’s Granulomatosis

A

HLA-DPB1 *0401 - Caucasian patients
HLA-DRB1 *0901 - Asian
HLA-DRB *1501 - African
chronic nasal infection with Staphylococcus aureus - WG relapse
Risk factors: exposure to silica or certain drugs (hydralazine and penicillamine)

156
Q

In WG, S. aureus induce molecular mimicry as it contains peptides similar to

A

proteinase 3 (PR3) autoantigen

157
Q

most have antibodies to neutrophil cytoplasmic antigens; in 80% of these, antibody is directed against an enzyme found in azurophilic granules of neutrophils called PR3

A

Wegener’s Granulomatosis

158
Q

enzyme found in azurophilic granules of neutrophils

A

PR3

159
Q

conditions to diagnose WG/GPA if 2 are met

A
  1. nasal or oral inflammation with oral ulcers or purulent or bloody nasal discharge
  2. abnormal chest x-ray, showing presence of nodules, fixed infiltrates, or cavities
  3. urinary sediment with microhematuria or RBC casts
  4. granulomatous inflammation on biopsy
160
Q

Ab where WG/GPA is positive

A

antineutrophil cytoplasmic antibody (ANCA)

161
Q

General laboratory findings of WG/GPA

A

▪ normochromic, normocytic anemia
▪ leukocytosis
▪ eosinophilia
▪ elevated ESR
▪ decreased albumin concentration in blood
▪ mild to severe renal insufficiency
▪ Urinalysis:
– Microhematuria
– Proteinuria
– Cellular casts

162
Q

Serological findings of WG/GPA

A

▪ elevated CRP
▪ elevated immunoglobulin levels
▪ positive ANCAs (c-ANCA pattern)
▪ other autoantibodies, such as RF and ANAs

163
Q

autoantibodies produced against proteins that are present in the neutrophil granules

A

Antineutrophil Cytoplasmic Antibodies (ANCAs)

164
Q

ANCA is strongly associated with 3 syndromes involving vascular inflammation:

A
  1. GPA or WG
  2. microscopic polyangiitis (MPA)
  3. eosinophilic granulomatosis with polyangiitis
    (EGPA; formerly known as ChurgStrauss syndrome)

*collectively known as ANCA-associated vasculitides (AAV)

165
Q

mainly directed against PR3 antigen

A

GPA

166
Q

specific for myeloperoxidase (MPO) Fluorescence observed through IIF, using ethanol-fixed leukocytes

A

MPA & EGPA

167
Q

As the cellular substrate; 2 patterns of fluorescence of ANCA

A

cytoplasmic or c-ANCA
perinuclear or p-ANCA

168
Q

appears as a diffuse, granular staining in the cytoplasm of neutrophils

A

cytoplasmic or c-ANCA

169
Q

staining is most intense in the center of the cell between the nuclear lobes and gradually fades at the outer edges of the cytoplasm

A

cytoplasmic or c-ANCA

170
Q

fluorescence surrounds the lobes of the nucleus, blending them together so that individual lobes cannot be distinguished

A

perinuclear or p-ANCA

immunology and serology (2 decks)

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