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Immunology & Serology (Lecture) > Autoimmunity > Flashcards

Autoimmunity Flashcards

1
Q

Paul Erlich (1900s) introduced the phenomenon he called “__________” or “_________”

A

“horror autotoxicus” or “fear of self-poisoning”

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2
Q

The immune system could attack the very host it was intended to protect

A

“horror autotoxicus” or “fear of self-poisoning”

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3
Q

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

A

immune response

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4
Q

Can be caused by T-cell–mediated immune
responses or autoantibodies that are directed
against host antigens

A

autoimmune diseases

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5
Q

The 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

Occurs in the central or primary lymphoid organs,
the thymus, and the bone marrow; Negative and
Positive Selection during T cell maturation; Receptor editing in B cells

A

central tolerance

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9
Q

Can result from anergy (a specific state of
unresponsiveness to the antigens) caused by the
absence of a costimulatory signal from an
antigen-presenting cell (APC) or binding of inhibitory receptors such as CTLA-4 (a molecule that prevents T-cell activation)

A

peripheral tolerance

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10
Q

A molecule that prevents T-cell activation

A

CTLA-4

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11
Q

More prevalent among family members and among monozygotic (genetically identical) twins than dizygotic (non-identical) twins or siblings

A

genetics

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12
Q

Women are 2.7 times more likely to acquire an
autoimmune disease than men; about 78% of
patients with autoimmune diseases are females; the stimulatory effects of female hormones may place women at a greater risk for developing autoimmune disease

A

hormonal influence

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13
Q

female hormones affected in autoimmune disease

A

Estrogen
Androgen
Prolactin

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14
Q

“Immunologic tolerance” some self-antigens may
be cryptic, or hidden within the tissues of the host. T and B lymphocytes are shielded from these
sequestered antigens and are not educated to
become tolerant to them

A

TISSUE TRAUMA AND RELEASE OF CRYPTIC ANTIGENS

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15
Q

TISSUE TRAUMA AND RELEASE OF CRYPTIC ANTIGENS
Tissue damage could be caused by factors such as:

A

infections
contact with environmental toxins
physical injury from exposure to ultraviolet (UV) radiation

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16
Q

Bacteria, viruses, and other infectious pathogens
may be able to trigger autoimmune responses

A

microbial infections

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17
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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18
Q

the microorganism can induce a local inflammatory response that recruits leukocytes and stimulates APCs to release cytokines that nonspecifically activate T cells; some of the T cells that are activated may have specificity for self-antigens

A

bystander effect

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19
Q

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

A

superantigens

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20
Q

can cause polyclonal activation of B cells

A

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

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21
Q

_______ refers to modifications in gene expression that are not caused by changes in the original DNA sequence.

A

epigenetics

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22
Q

can induce epigenetic changes by increasing or decreasing methylation of cytosine bases, modifying histones, and causing abnormal regulation by microRNAs; underexpression of certain genes in the immune system may result in
homeostatic imbalances and a breakdown of
self-tolerance, leading to autoimmunity

A

Triggered by exposure to environmental
toxins, ingestion of harmful foods or drugs,
or the aging process

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23
Q

Exposure to environmental factors can lead
to changes at the protein level; post-translational modifications and may involve biochemical processes such as:

A

acetylation
lipidation
citrullination
glycosylation

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24
Q

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

complex interactions between genetic and environmental factors

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25
Q

Chronic
Peak age of onset: 20-40 years old
W:M – 9:1, 5 yr
Survival rate – 90%

A

SLE

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26
Q

Complex interactions between environmental
factors, genetic susceptibility, and abnormalities
within the immune system; genetic defects in genes coding for HLA-A1, B8, and DR3

A

SLE

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27
Q

increases the chance of developing lupus; polymorphisms in genes, genes coding for various cytokines, and genes involved in signaling of innate immune responses - result in uncontrolled autoreactivity of T and B cells, which leads to the production of numerous autoantibodies

A

HLA-A1
B8
DR3

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28
Q

Antibodies to double-stranded DNA (dsDNA),
histones, and other nuclear components, as well as
autoantibodies to lymphocytes, erythrocytes,
platelet, phospholipids, ribosomal components, and endothelium

A

SLE

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29
Q

_______ are present in 70% of patients with lupus and are highly specific for the disease

A

Antibodies to dsDNA

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30
Q

________ and _________ have been found in immune complexes that are deposited in organs such as the kidneys and skin

A

Anti-dsDNA and complement proteins

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31
Q

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

A

Leukocytes

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32
Q

Antibodies to RBCs

A

hemolytic anemia

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33
Q

Antibodies to platelets

A

thrombocytopenia

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34
Q

inflammation of the blood vessels and vascular
damage in lupus, which may be responsible for
the vasculitis and neuropsychiatric symptoms

A

Antibodies to endothelial cells

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35
Q

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

A

Phospholipid antibodies

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36
Q

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

A

neonatal lupus

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37
Q

neonatal lupus antigens

A

SS-A/Ro
SS-B/La

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38
Q

SLE
fatigue, weight loss, malaise, fever, and anorexia are often the first to appear

A

Nonspecific symptoms

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39
Q

SLE
______ and ______ in 90% of patients
(arthritis is symmetric and involves the small joints of the hands, wrists, and knees)

A

Polyarthralgias or arthritis

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40
Q

________ may appear on any area of the
body exposed to UV light in 80% of patients
(butterfly rash across the nose and cheeks)

A

Erythematous rash

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41
Q

drug-induced lupus

A

procainamide, hydralazine, chlorpromazine, isoniazid, quinidine, anticonvulsants such as methyldopa, and possibly oral contraceptives

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42
Q

Acute cutaneous lupus, chronic cutaneous lupus,
oral ulcers, non-scarring alopecia (thinning or
fragility of the hair), synovitis, serositis, renal
involvement, neurological symptoms, hemolytic
anemia, leukopenia, and thrombocytopenia

A

clinical criteria

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43
Q

Elevated antinuclear antibody titer, elevated
anti-dsDNA titer, presence of antibody to the Sm
nuclear antigen, presence of antiphospholipid
antibody, low complement levels, and positive direct Coombs’ test in the absence of hemolytic anemia

A

immunologic criteria

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44
Q

A patient must satisfy at least __ of the 17 criteria,
including at least one clinical criterion and one
immunologic criterion, to be classified as having
SLE

A

4

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45
Q

SLE
For mild symptoms, a high dose of _______ or
other anti-inflammatory drug may bring relief

A

aspirin

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46
Q

SLE
For skin manifestations, antimalarials such as
__________ or ________ and _________ are often prescribed

A

hydroxychloroquine
chloroquine
topical steroids

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47
Q

The antimalarial drugs are thought to inhibit
signaling of _____, __, and __

A

TLR 7, 8, and 9

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48
Q

used for acute fulminant (severe and sudden) lupus, lupus nephritis, or central nervous system (CNS) complications because these suppress the immune response and lower antibody titers

A

Systemic corticosteroids

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49
Q

laboratory diagnosis of SLE

A

Complete blood count (CBC), platelet count, and
urinalysis, quantification of complement
proteins (C3) and the detection of specific
autoantibodies

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50
Q

Autoantibodies that are directed against antigens in the nuclei of mammalian cells

A

ANTI-NUCLEAR ANTIBODIES (ANAs)

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51
Q

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

A

DOUBLE-STRANDED DNA (dsDNA) ANTIBODIES

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52
Q

ANTIHISTONE ANTIBODIES
_______ are nucleoproteins that are essential components of chromatin

A

histones

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53
Q

ANTIHISTONE ANTIBODIES
five major classes of histones:

A

H1, H2A, H2B, H3, and H4

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54
Q

ANTIHISTONE ANTIBODIES:
Antibodies to ____ and ___ can be detected in
almost all patients with drug-induced lupus

A

H2A and H2B

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55
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

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56
Q

antihistone antibodies pattern in IIF assay

A

homogeneous pattern

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57
Q

Stimulated by DNA-histone complexes;
nucleosomes, or deoxyribonucleoprotein (DNP)

A

nucleosome antibodies

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58
Q

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

A

nucleosome antibodies

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59
Q

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

A

nucleosome antibodies

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60
Q

nucleosome antibodies pattern in IIF assay

A

homogeneous

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61
Q

associated with uridine-rich RNA

A

extractable nuclear antigens (ENA)

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62
Q

Found in only 20% to 40% of patients with SLE,
depending on the race of the population

A

antibody to Sm antigen

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63
Q

anti-Sm antibody pattern in IIF

A

coarse speckled pattern of nuclear fluorescence

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64
Q

anti-RNP antibody pattern in IIF

A

coarse speckled pattern

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65
Q

anti-RNP antibody meaning

A

anti-ribonucleoproteins antibody

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66
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 antibody

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67
Q

Appears in approximately 24% to 60% of patients
with SLE and has been closely associated with the
presence of nephritis, vasculitis,
lymphadenopathy, photosensitivity, and
hematologic manifestations such as leukopenia

A

ANTI-SS-A/Ro

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68
Q

Found in only 9% to 35% of patients with SLE and
all of these have anti–SS-A/Ro; most often found in
patients who have cutaneous manifestations of
SLE, especially photosensitivity dermatitis

A

ANTIBODIES TO SS-B/La

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69
Q

Can cross the placenta and have been associated
with neonatal lupus

A

ANTIBODIES TO BOTH SS-A/Ro and SS-B/La

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70
Q

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

A

nucleolus

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71
Q

Common in systemic sclerosis (also known as
scleroderma)

A

ANTIBODY TO FIBRILLARIN

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72
Q

ANTIBODY TO FIBRILLARIN pattern in IIF

A

clumpy nucleolar fluorescence

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73
Q

Associated with scleroderma

A

ANTIBODIES TO RNA POLYMERASE

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74
Q

ANTIBODIES TO RNA POLYMERASE pattern in IIF

A

speckled nucleolar pattern

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75
Q

Also known as PM/Scl

A

ANTIBODIES TO PM-1 ANTIGEN

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76
Q

Found in polymyositis and systemic sclerosis;
homogeneous staining of the nucleolus

A

ANTIBODIES TO PM-1 ANTIGEN

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77
Q

Bind to proteins in the middle region of a
chromosome where the sister chromatids are joined; directed against three centromere antigens of molecular weights 16kDa, 80kDa, and 120kDa

A

ANTICENTROMERE ANTIBODIES

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78
Q

3 centromere antigens molecular weights:

A

16kDa
80kDa
120kDa

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79
Q

Found in 50% to 80% of patients with the CREST
syndrome

A

ANTICENTROMERE ANTIBODIES

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80
Q

CREST syndrome:

A

Calcinosis, Raynaud’s
phenomenon, Esophageal dysmotility,
Sclerodactyly, Telangiectasia

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81
Q

anticentromere antibodies pattern

A

discrete speckled staining in the nuclei
of the cells

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82
Q

methods of ANA detection

A

IIF, immunoperoxidase staining,
enzyme-linked immunosorbent assay (ELISA),
microsphere multiplex immunoassays (MIA),
radioimmunoassay (RIA), immunodiffusion,
immunoblotting (Western blot), dot blot,
immunoelectrophoresis, and microarray

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83
Q

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

A

ANTIPHOSPHOLIPID ANTIBODIES

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84
Q

Associated with deep-vein and arterial thrombosis
and with recurrent pregnancy loss

A

antiphospholipid antibodies

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85
Q

Found in up to 60% of patients with lupus

A

antiphospholipid antibodies

86
Q

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

A

antiphospholipid antibodies

87
Q

APTT may be prolonged, but it 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

88
Q

Affects about 0.5% to 1.0% of the adult
population; age: 25 and 55; W:M – 3:1

A

rheumatoid arthritis

89
Q

Can be characterized as a chronic, symmetric, and
erosive arthritis of the peripheral joints that can also affect multiple organs such as the heart and the lungs

A

rheumatoid arthritis

90
Q

HLA-DRB1 alleles or PTPN22 gene
polymorphisms

A

rheumatoid arthritis

91
Q

Strongest environmental risk factor of RA

A

cigarette smoking

92
Q

Caused by an inflammatory process that results in
the destruction of bone and cartilage

A

rheumatoid arthritis

93
Q

Lesions show an increase in cells lining the synovial membrane and formation of a pannus, a sheet of inflammatory granulation tissue that grows into the joint space and invades the cartilage

A

rheumatoid arthritis

94
Q

RHEUMATOID ARTHRITIS:
Balance between proinflammatory and
anti-inflammatory cytokines _______

A

IL-1, IL-6, IL-17, and TNF-α

95
Q

these enzymes degrade important structural proteins in the cartilage

A

metalloproteinases

96
Q

Normally, there is a good balance between
bone production and destruction. However, in
RA, the osteoclasts become overly activated in
the inflammatory environment of the joints.

A

local bone erosion

97
Q

TNF-α, in conjunction with other cytokines and
a molecule called ___________, induces the differentiation of osteoclasts and inhibits
bone formation

A

RANKL (receptor activator of nuclear factor kappa-B ligand)

98
Q

second major type of antibody associated
with RA

A

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

99
Q

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

A

Citrulline

100
Q

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

A

Nonspecific symptoms

101
Q

Can progress to the larger joints, often affecting the knees, hips, elbows, shoulders, and cervical spine

A

RA

102
Q

(T/F)
Inflammation if left untreated, may lead to
permanent joint dysfunction and deformity

A

true

103
Q

RA
Extra-articular manifestations include the:

A

formation of subcutaneous nodules, pericarditis,
lymphadenopathy, splenomegaly, interstitial lung
disease, or vasculitis

104
Q

RA
Some have nodules over the:

A

bones, myocardium, pericardium, heart valves, pleura, lungs, spleen, and larynx

105
Q

RA
Most common cause of death is _______ due to the acceleration of arteriosclerosis by proinflammatory cytokines released during the disease process

A

cardiovascular disease

106
Q

tretament for RA

A

Disease-modifying anti-rheumatic drugs
(DMARDs)
methotrexate

107
Q

act by inhibiting adenosine metabolism and T-cell activation

A

methotrexate

108
Q

RA (TREATMENT):
monoclonal antibodies to TNF-α

A

infliximab, adalimumab, certolizumab, golimumab

109
Q

RA (TREATMENT)
TNF-α receptors fused to an IgG molecule

A

etanercept

110
Q

_____ is the antibody that is most often tested to aid in making the initial diagnosis (is an autoantibody, usually of the IgM class, that reacts with the Fc portion of IgG; 70% to 90% of patients with RA test positive for RF)

A

Rheumatoid Factor (RF)

111
Q

RF can be found in patients with other
connective tissue diseases such as _________, and
_________, as well as in people with some _______

A

SLE
Sjögren’s syndrome
scleroderma
mixed connective tissue disease
chronic infections

112
Q

Manual agglutination tests using ______ or _____ particles coated with IgG have been used for many years to detect RF

A

charcoal or latex

113
Q

RF detection

A

ELISA, chemiluminescence immunoassay, and nephelometric methods

114
Q

RA
Laboratory testing for antibody to:

A

cyclic citrullinated peptides (anti-CCP) – ELISA

115
Q

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

A

GRANULOMATOSIS WITH POLYANGIITIS

116
Q

GRANULOMATOSIS WITH POLYANGIITIS aka _______

A

WEGENER’S GRANULOMATOSIS

117
Q

Usually begins with a localized inflammation of
the upper and lower respiratory tract.

A

GRANULOMATOSIS WITH POLYANGIITIS

118
Q

General symptoms of granulomatosis with polyangiitis

A

fever, malaise, arthralgias, anorexia, and weight loss.

119
Q

The majority of patients progress to develop a more systemic form of the disease that can affect any organ system

A

granulomatosis with polyangiitis

120
Q

Patients can experience coughing, shortness of
breath, chest pain, or hemoptysis (coughing up
blood)

A

granulomatosis with polyangiitis

121
Q

GRANULOMATOSIS WITH POLYANGIITIS
(T/F)
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; pain and arthritis of the large joints, which is usually symmetric but not deforming; skin lesions; ocular manifestations that can potentially lead to vision loss

A

true

122
Q

________ has been found to have a strong association with GPA in Caucasian patients

A

HLA-DPB1*0401 allele

123
Q

________ and ________ are associated with increased risk in Asian and African American populations

A

HLA-DRB10901 and HLA-DRB1501 alleles

124
Q

(T/F)
Chronic nasal infection with Staphylococcus aureus
bacteria has been associated with a greater rate of
relapse in patients with WG

A

true

125
Q

(T/F)
Chronic nasal infection with Staphylococcus aureus
bacteria has been associated with a greater rate of
relapse in patients with WG

A

true

126
Q

GP:
S. aureus may induce molecular mimicry because it contains peptides that bear similarity to the _______

A

proteinase 3 (PR3) autoantigen

127
Q

RISK FACTORS of GP

A

Exposure to silica or to certain drugs such as
hydralazine and penicillamine

128
Q

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

A

PR3

129
Q

Autoantibodies that are produced against proteins
that are present in the neutrophil granules

A

ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES (ANCAs)

130
Q

Strongly associated with three syndromes involving vascular inflammation: GPA or WG, microscopic polyangiitis (MPA), and eosinophilic
granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome) - collectively known as ANCA-associated vasculitides (AAV)

A

ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES (ANCAs)

131
Q

eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as ________

A

Churg-Strauss syndrome

132
Q

mainly directed against the PR3 antigen

A

GPA

133
Q

specific for myeloperoxidase (MPO)

A

MPA and EGPA

134
Q

ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES (ANCAs):
Fluorescence observed through IIF, using _______

A

ethanol-fixed leukocytes

135
Q

two patterns of fluorescence

A

Cytoplasmic or c-ANCA
Perinuclear or p-ANCA

136
Q

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

137
Q

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

A

perinuclear or p-ANCA

138
Q

Diseases in which the immune response is directed
against self-antigens that are mainly found in a
single organ or gland; systemic effects may
sometimes also occur

A

ORGAN-SPECIFIC AUTOIMMUNE DISEASES

139
Q

2 types of AUTOIMMUNE THYROID DISEASE (AITDS)

A

Hashimoto’s thyroiditis and Graves disease

140
Q

associated with inheritance of HLA antigens DR3, DR4, DR5, and DQ7

A

hashimoto’s thyroiditis

141
Q

HLA-DR3 is affected

A

graves’ disease

142
Q

________ are expressed on the surface of thyroid epithelial cells, perhaps increasing the autoimmune response. Mutations in the thyroglobulin gene may allow for interaction of
the protein with HLA-DR antigens, resulting in antithyroglobulin antibodies

A

HLA-DR antigens

143
Q

hashimoto’s thyroiditis aka _______

A

chronic lymphocytic thyroiditis

144
Q

he discovered hashimoto’s thyroiditis in japan (1912)

A

Dr. Hakaru Hashimoto

145
Q

HASHIMOTO’S THYROIDITIS
Most common autoimmune disease, 8 in every
1,000; W:M - ________

A

5-10:1

146
Q

Enlarged thyroid called a goiter; production of
thyroid-specific autoantibodies and cytotoxic T cells

A

hashimoto’s thyroiditis

147
Q

a state of decreased thyroid function due to immune destruction of thyroid gland

A

hypothyroidism

148
Q

dry skin, decreased sweating, puffy face with
edematous eyelids, pallor with a yellow tinge, weight gain, fatigue, and dry and brittle hair

A

hashimoto’s thyroiditis

149
Q

Characterized by hyperthyroidism, a state of
excessive thyroid function; manifested as
thyrotoxicosis, or an excess of thyroid hormones

A

graves’ disease

150
Q

nervousness, insomnia, depression, weight
loss, heat intolerance, sweating, rapid heartbeat, palpitations, breathlessness, fatigue, cardiac
dysrhythmias, and restlessness

A

graves’ disease

151
Q

GRAVES’ DISEASE
Another sign present in approximately 35% of
patients is ________, in which hypertrophy of
the eye muscles and increased connective tissue in
the orbit cause the eyeball to bulge out so that the
patient has a large-eyed staring expression.

A

exophthalmos

152
Q

treatment for GD

A

Daily oral thyroid hormone replacement therapy, with levothyroxine (T4)
radioactive iodine therapy
surgery to remove part of the thyroid

153
Q

GRAVES’ DISEASE
Measurement of circulating TSH levels –
chemiluminescent immunoassays that can detect
fewer than ______

A

0.1 mU/L

154
Q

An endocrine disorder characterized by
hyperglycemia (a high level of glucose in the blood); characterized by a complete or nearly complete deficiency in insulin

A

type 1 DM

155
Q

Involves selective destruction of the beta cells of the pancreas (located in clusters called the _________ and are responsible for the production and secretion of the hormone, insulin) resulting in
insufficient insulin production, hyperglycemia, and
toxic effects on the body

A

islets of Langerhans

156
Q

Long-term effects include cardiovascular complications, renal disease, nerve damage, blindness, and infections of the lower extremities, which can lead to amputation

A

type 1 DM

157
Q

Carry the HLA-DR3 or DR4 gene

A

type 1 DM

158
Q

treatment for type 1 DM

A

Daily injectable insulin
Transplantation of pancreatic beta islet cells (for patients who have poor glucose control)
Islet cell transplantation

159
Q

T1DM
a fasting glucose greater than ______ on more than one occasion (normal value is lower than 100 mg/dL)

A

126 mg/dL

160
Q

T1DM
a random plasma glucose level of _______ or more with classic symptoms of diabetes

A

200 mg/dL

161
Q

T1DM
an oral glucose tolerance test of ______ or more in a 2-hour sample with a 75g glucose load

A

200 mg/dL

162
Q

T1DM
a hemoglobin A1c value (HbA1c) greater than _____

A

6.5%

163
Q

a glycated form of hemoglobin that
is made when the RBC protein combines with
glucose in the blood.

A

HbA1c

164
Q

The HbA1c plasma level is proportional to the life span of the circulating RBCs (up to _______) and
reflects the average plasma glucose concentration over the previous ________

A

120 days
2 to 3 months

165
Q

T1DM
________ and _______ can be done to confirm the diagnosis

A

Antibodies to glutamic acid decarboxylase (GAD) and insulinoma-antigen 2 antibodies (IA-2A)

166
Q

An autoimmune disease affecting the small intestine and other organs; associated with a known environmental trigger—dietary gluten

A

celiac disease

167
Q

HLA-DQ2 ((90% to 95%) or HLA-DQ8
W:M - 2-3:1

A

celiac disease

168
Q

CELIAC DISEASE
diarrhea, abdominal distention, and
failure to thrive, but may also experience
vomiting, irritability, anorexia, and constipation

A

infants symptoms

169
Q

CELIAC DISEASE
classic symptoms of diarrhea and abdominal
pain or discomfort, but often have
extraintestinal manifestations that make the
condition difficult to diagnose

A

Older children, teenagers, and adults

170
Q

short stature, arthritis or arthralgia, osteoporosis,
neurological symptoms, iron-deficiency anemia, and dermatitis herpetiformis (a skin disorder with itchy blistering)

A

extraintestinal

171
Q

Treatment of celiac disease involves placing patients on a _________

A

gluten-free diet

172
Q

CELIAC DISEASE
Based on clinical symptoms, serological findings,
duodenal biopsy, and presence of the ______ or
_______

A

HLA-DQ2 or HLA-DQ8 haplotype

173
Q

AUTOIMMUNE LIVER DISEASES

A

Autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC)

174
Q

Formerly known as chronic active hepatitis, is an
immune mediated liver disease that can lead to
end-stage liver failure if left untreated

A

autoimmune hepatitis (AIH)

175
Q

Adults usually present with an unexpected onset of vague symptoms, including fatigue, nausea, weight loss, abdominal pain, itching, and maculopapular rashes

A

autoimmune hepatitis (AIH)

176
Q

2 types of AIH

A

AIH-1
AIH-2

177
Q

accounts for two-thirds of all AIH cases and has a female:male ratio of 4:1

A

AIH-1

178
Q

______ has a female:male ratio of 10:1 and is
seen mostly in children

A

AIH-2

179
Q

________ and ________ are associated with a higher risk of developing AIH

A

HLA-DRB1 and HLA-DQB1 alleles

180
Q

AIH treatment

A

Standard immunosuppressive treatment of prednisolone (+/–azathioprine)
liver transplant

181
Q

The most common autoimmune liver disease; W:M -10:1

A

PRIMARY BILIARY CIRRHOSIS (PBC)

182
Q

Genetic link with certain HLA-DRB1, HLA-DQA1,
HLA-DPB1, and HLA-DQB1 haplotypes

A

primary biliary cirrhosis (PBC)

183
Q

Involves progressive destruction of the intrahepatic bile ducts

A

primary biliary cirrhosis (PBC)

184
Q

A condition in which the flow of bile is slowed or
blocked), inflammation of the portal vein in the liver, and accumulation of scar tissue that can ultimately lead to cirrhosis and liver failure. Individual patients can be asymptomatic or have slowly or rapidly progressing disease

A

primary biliary cirrhosis (PBC)

185
Q

include fatigue, pruritus (itchy skin), abdominal
pain, and dry eyes and mouth; in the later stages, symptoms include jaundice, ascites, and greasy
stools

A

primary biliary cirrhosis (PBC)

186
Q

PBC treatment

A

Ursodeoxycholic acid (UDCA)
liver transplant

187
Q

a bile acid that helps move bile through the liver

A

Ursodeoxycholic acid (UDCA)

188
Q

LAB DIAGNOSIS OF PBC

A

Anti-mitochondrial antibodies (AMAs)

189
Q

IIF and ELISA, serum alkaline phosphatase level elevated at least 1.5 times the upper limit of normal for 6 months or more and liver biopsy showing nonsuppurative destructive cholangitis and interlobular bile duct injury

A

anti-microbial antibodies (AMAs)

190
Q

An autoimmune disorder involving inflammation and destruction of the CNS

A

multiple sclerosis

191
Q

MULTIPLE SCLEROSIS
Most closely associated with inheritance of a
particular HLA molecule coding for the beta chain of the DR subregion, namely ________

A

DRB1*1501

192
Q

Environmental factors that appear to be associated with MS include _________

A

reduced exposure to sunlight, vitamin D deficiency, and cigarette smoking

193
Q

Characterized by the formation of lesions called
plaques in the white matter of the brain and spinal
cord, resulting in the progressive destruction of the myelin sheath of axons

A

multiple sclerosis

194
Q

Visual disturbances, weakness or diminished
dexterity in one or more limbs, locomotor
incoordination, dizziness, facial palsy, and numerous

A

multiple sclerosis

195
Q

MS
Sensory abnormalities such as tingling or _________that run down the spine or extremities, as well as flashes of light seen on eye movement;

A

“pins and needles”

196
Q

MS ratio

A

W:M - 2:1

197
Q

treatment for MS

A

Corticosteroids
IFN-β1a and IFN-β1b
natalizumab

198
Q

to reduce inflammation

A

corticosteroids

199
Q

downregulate MHC molecules on APCs, decreasing production of proinflammatory cytokines, and upregulating anti-inflammatory cytokines

A

IFN-β1a and IFN-β1b

200
Q

a humanized monoclonal antibody directed against an adhesion molecule of lymphocytes, preventing them from binding to endothelial cells and crossing the blood–brain barrier

A

natalizumab

201
Q

An autoimmune disease that affects the
neuromuscular junction; characterized by weakness and fatigability of skeletal muscles

A

MYASTHENIA GRAVIS (MG)

202
Q

Antibody-mediated damage to the acetylcholine
receptors in skeletal muscle or to other proteins in
the neuromuscular junction leads to progressive
muscle weakness

A

MYASTHENIA GRAVIS (MG)

203
Q

S/S: ptosis (drooping of the eyelids); diplopia
(double vision); and the inability to retract the
corners of the mouth, often resulting in a snarling
appearance; muscle weakness is most noticeable in the upper limbs; some have difficulty in speaking, chewing, and swallowing and may be unable to maintain support of the trunk, neck, or head

A

MYASTHENIA GRAVIS (MG)

204
Q

treatment for MYASTHENIA GRAVIS (MG)

A

Anticholinesterase agents
acetylcholine
thymectomy
plasmapheresis or intravenous immunoglobulin
monoclonal antibodies or fusion proteins

205
Q

to prevent destruction of the neurotransmitter

A

Anticholinesterase agents

206
Q

should be performed on patients who have a thymoma

A

thymectomy

207
Q

targeted to specific components of the immune system involved in the pathogenesis of MG

A

monoclonal antibodies or fusion proteins

208
Q

Characterized by the presence of autoantibody to an antigen in the basement membranes in the glomeruli of the kidneys and alveoli of the lungs

A

GOOD PASTURE’S SYNDROME

209
Q

GOOD PASTURE’S SYNDROME
Identified by ______ in _______; affects
two age groups—men in their 30s and men and
women in their 60s and 70s

A

Ernest Goodpasture in 1919

210
Q

_________ is a rare disorder that is found mainly in Caucasians of European origin

A

goodpasture’s syndrome

211
Q

S/S: fatigue and malaise followed by clinical signs of kidney involvement such as edema and hypertension, which can rapidly progress to acute
renal failure if left untreated; 60% to 70% of patients have pulmonary involvement and exhibit symptoms such as cough, shortness of breath, and hemoptysis

A

goodpasture’s syndrome

212
Q

treatment for goodpasture’s syndrome

A

high dose of corticosteroids
immunosuppressive drugs (cyclophosphamide)
plasmapheresis
hemodialysis
kidney transplantation

Immunology & Serology (Lecture) (8 decks)

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