Immune Flashcards

(82 cards)

1
Q

REQUIREMENTS OF AUTOIMMUNITY

A

● The presence of an immune reaction specific for some self antigen or self tissue
● Not secondary to tissue damage but is of primary pathogenic significance
● Absence of another well-defined cause of the disease

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2
Q

Diseases mediated by antibodies

A
Hemolytic anemia
Thrombocytopenia
atrophic gastritis of pernicious anemia
Myasthenia gravis
Graves disease
goodpasture syndrome
SLE
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3
Q

Diseases mediated by T cells

A
T1 DM
Multiple sclerosis
Rheumatoid arthritis
Systemic sclerosis
Sjogen sydndrome
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4
Q

Diseases postulated to be autoimmune

A
IBD
Primary biliary cirrhosis
Autoimmune hepatits
Polyarteritis nodosa
Inflammatory myopathies
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5
Q

Phenomenon of unresponsiveness to an antigen
induced by exposure of lymphocytes to that
antigen

A

IMMUNOLOGIC TOLERANCE

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6
Q

CENTRAL TOLERANCE:

Originates in the_______

A

primary lymphoid organs

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7
Q

Process where your immature T cells
express receptors specific to self
antigen. When these proliferate, they are
self reactive. The body kills these cells
through apoptosis

A

Negative Selection or clonal deletion

T cells (thymus)

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8
Q

stimulates expression of
some peripheral tissue restricted self
antigens in the thymus and is thus critical
for deletion of immature T cells specific
for these antigens

A

AIRE (autoimmune

regulator)

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9
Q

Cells are still intact but the receptor is
changed into another type so that the
potentially reactive lymphocyte will no
longer react to its own tissue.

A

● Receptor Editing

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10
Q

This occurs when self-reactive cells escape the
central lymphoid organs reaching the periphery
and so the mechanisms are also different.

A

PERIPHERAL TOLERANCE

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11
Q

The best-defined regulatory T
cells are___ cells that express high
levels of CD25, the α chain of the IL-2
receptor, and FOXP3,

A

CD4

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12
Q

Activation of antigen-specific T cells

requires two signals:

A
recognition of 
peptide antigen in association with self 
MHC molecules on the surface of APCs 
and a set of costimulatory signals 
(“second signals”) from APCs
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13
Q

binds ligand of T cell from your APC.
If this signal is absent or is unresponsive
there is no binding. This is failure to
respond

A

co-stimulatory signal (such as CD28),

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14
Q

HLA associated diseases

A
RA
T1 DM
Multiple sclerosis
SLE
Ankylosing spondylitis (100-200)
Celiac disease
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15
Q

which
encode your tyrosine phosphatase and involved
in Rheumatoid Arthritis and Type 1 DM and
Inflammatory bowel disease

A

genes associated with your PTPN22

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16
Q

Best known for inflammatory bowel
disease.
- associated with crohn’s disease

A

NOD2

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17
Q

Polymorphisms in the gene encoding
the IL-2 receptor (CD25) are associated
with

A

Multiple sclerosis

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18
Q

antibodies against strep proteins
cross react with myocardial proteins and cause
myocarditis. The immune responses initially
directed against microbial antigens, also result in
activation of self-reactive lymphocytes. Aside
from attacking just the strep protein, it also
attacks own tissue

A

Rheumatic Heart Disease

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19
Q

Some viruses, such as EBV and HIV, cause _______, which may result
in production of autoantibodies.

A

polyclonal B-cell activation

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20
Q

T or F
infections promote low-level
IL-2 production, and this is essential for
maintaining regulatory T cells

A

T

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21
Q

In addition to infections, _____causes cell death and may lead to the
exposure of nuclear antigens, which elicit
pathologic immune responses in lupus; this
mechanism is the proposed explanation for the
association of lupus flares with exposure to
sunlight.

A

ultraviolet (UV)

radiation

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22
Q

T or F
Smoking is a risk factor for rheumatoid arthritis,
perhaps because it leads to chemical
modification of self antigens.

A

T

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23
Q

Autoimmune disease involving multiple organs,
characterized by a vast array of autoantibodies,
particularly antinuclear antibodies (ANAs), in
which injury is caused mainly by deposition of
immune complexes and binding of antibodies to
various cells and tissues

A

SYSTEMIC LUPUS ERYTHEMATOSUS

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24
Q

In SLE, injury to the _____________ is prominent.

The disease is very heterogeneous, and any
patient may present with any number of these
clinical features

A

skin, joints, kidney, and serosal

membranes

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25
SLE is a Type___Hypersensitivity Reaction
III
26
Clinical/immunologic criteria: (this is important to | the diagnosis of SLE) cutaneous lupus ____(acute) & ____ (chronic).
malar rash; discoid rash
27
A RASH POINT MD
Mnemonic: A RASH POINT MD ● Arthritis ● Renal disease, ANAs, Serositis, Hematological disorders ● Photosensitivity, Oral Ulcers, Immunological Disorders, Neurological Disorders ● Malar Rash, Discoid ulcers
28
For a patient to be classified having SLE you need to have ______ both have clinical and immunological.
4/11 of criterion
29
The hallmark of SLE is the production of | autoantibodies, several of which (_________) are virtually diagnostic
antibodies to double-stranded DNA and the so called Smith [Sm] antigen
30
ANA’s. These are directed against nuclear Ag and can be grouped into four categories based on their specificity for:
○ DNA ○ Histones ○ Non histone proteins bound to RNA ○ Nucleolar Ag
31
is the most widely used method for detecting ANA, which can identify Ab that binds to a variety of nuclear Ag. The pattern of nuclear fluorescence suggests the type of Ab present in a patient's serum
Indirect Immunofluorescence
32
● Antibodies to chromatin, histones and occasionally, double stranded DNA ● Most specific
Homogenous or diffuse nuclear staining
33
Most often indicative antibodies to double | stranded DNA and nuclear envelope proteins
Rim or peripheral staining
34
● Presence of uniform or variable-sized speckles ● Most commonly observed patterns of fluorescence and therefore the least specific ● Antibodies to non-DNA nuclear constituent such as Sm antigen(SLE), ribonucleoprotein (SLE and others) and SS-A and SS-B (Sjogren)
Speckled pattern (
35
● Antibodies specific for centromeres (Systemic sclerosis, Sjogren) ● Stain Ab to centromere
Centromeric pattern
36
● Ab to RNA ● Presence of few discrete spots of fluorescence within the nucleus. ● Most often in patients with systemic sclerosis
Nucleolar pattern
37
SLE- most specific is _______ and also associated with _____. Generic ANA involved there are 95 to 100 percent positive for SLE, but the problem for this is not specific. Sm antigen there is 20-30 percent positive
Sm antigen; Antiphospholipid syndrome
38
is specific for CCP (cyclic | citrullinated peptides)
Rheumatoid arthritis
39
The fundamental defect in SLE is a failure of the | mechanism that maintains___________
self-tolerance
40
T OR F Lack of your complement may impair removal of circulating immune complexes by mononuclear phagocyte system and since they are not removed, favoring deposition in the tissues, leading to tissue anergy
T
41
T or F There is a higher rate of concordance (>20%) in monozygotic twins when compared with dizygotic twins (1% to 3%).
T
42
Specific alleles of the _______ locus have been linked to the production of anti–double-stranded DNA, anti-Sm, and antiphospholipid antibodies, although the relative risk is small
HLA-DQ
43
play a role in lymphocyte | activation in SLE.
Type I interferons -are antiviral cytokines that are normally produced during innate immune responses to viruses.
44
T or F The autoantibodies in SLE show characteristics of T cell-dependent antibodies produced in germinal centers, and increased numbers of follicular helper T cells have been detected in the blood of SLE patients.
T
45
TLR engagement by nuclear DNA and RNA | contained in immune complexes may activate______
B lymphocytes
46
Drugs such as ___________can induce an SLE- like | response in humans
hydralazine, procainamide, and | D-penicillamine
47
● Most common mechanism of tissue injury ● DNA and anti-DNA complexes deposit in the glomeruli and small blood vessels, and they go into tissues resulting in a low level of serum complement. Because they are deposited in glomeruli and other tissues
Deposition of Immune complexes (Type 3 | Hypersensitivity
48
``` Most common disorder caused is Immune Thrombocytopenic Purpura (ITP) ```
Autoantibodies specific for RBCs, WBCs and | platelets (Ab-mediated type 2 hypersensitivity)
49
● Secondary APS ● Venous and arterial thrombosis associated with recurrent spontaneous miscarraige and focal cerebral or ocular ischemia
Antiphospholipid antibody Syndrome (APS)
50
Antibodies cross the BBB and react with receptor or neuron for various neurotransmitter ● Cytokine may involved in cognitive dysfunction
Neuropsychiatric manifestations
51
Nuclei of damaged cells react with your ANAs, they lose their chromatin and become a homogenous material to produce_______
LE body
52
Usually seen in blood vessels and usually manifested as thrombosis (venous or arterial) or vasculitis. Mechanism for patients with recurrent miscarriages because of antiphospholipid antibodies.
Antinuclear Antibodies
53
In SLE, the most clinical involvement is the ____
Kidney (50% of SLE patients)
54
Class 1 glomerular patterns -is very uncommon and is characterized by immune complex deposition in the mesangium
Minimal mesangial lupus nephritis -No infiltrates yet but Mesangial deposits are present by IgG (granular type)
55
Class 2 glomerular patterns
MESANGIAL PROLIFERATIVE LUPUS | NEPHRITIS
56
Class 1 are identified by ______
IF and EM
57
● Mesangial proliferation often with accumulation of mesangial matrix and granular mesangial deposits of immunoglobulins and complement ● No involvement of capillaries
Class 2 Mesangial proliferative lupus nephritis
58
Class 3 glomerular patterns
Focal lupus nephritis
59
● <50% glomeruli involved (segmental-portion or global-affects whole) ● Endothelial and mesangial cell proliferation ● Leukocyte accumulation ● Capillary necrosis and hyaline thrombi ● Extracapillary proliferation ● Crescent formation ● With hyaline thrombi (pink material) meaning there is involvement of capillaries
Class 3: Focal lupus nephritis
60
The clinical presentation ranges from mild hematuria and proteinuria to acute renal insufficiency.
Class 3: Focal lupus nephritis
61
Class 4 glomerular patterns
Diffuse lupus nephritis
62
● Most common type ● Severe form ● >50% glomerular involvement
CLASS 4 : DIFFUSE LUPUS NEPHRITIS
63
● Endothelial, mesangial and epithelial cell proliferation ● Crescent formation ● “Wire loop” lesion - subendothelial deposits
CLASS 4 : DIFFUSE LUPUS NEPHRITIS
64
Class 5 glomerular patterns
MEMBRANOUS LUPUS NEPHRITIS
65
● Same with class 4 ● PAS stain, capillaries are well distinct because of thickening ● This patient has severe proteinuria ● Diffuse thickening of capillary walls due to subepithelial deposits
CLASS 5: MEMBRANOUS LUPUS NEPHRITIS
66
The immune complexes are usually accompanied by increased production of basement membrane-like material.
Class 5: membranous lupus nephritis
67
Class 6 glomerular patterns
ADVANCED SCLEROSING NEPHRITIS
68
``` ● >90% glomerular involvement ● End-stage renal disease ● With lots of fibrosis and fibrinoid necrosis of tubules and arterioles, ● Kidneys are not functional anymore ```
CLASS 6: ADVANCED SCLEROSING NEPHRITIS
69
TUBULOINTERSTITIAL LESION
● Immune complexes in tubular or peritubular capillaries ● Well-organized B cell follicles in the interstitium
70
Thick capillary loop no longer delicate so this produces characteristic wire loop lesions so as you can see here is quietly thick already
SLE
71
In SLE, there is vacuolar degeneration of basal layer in this area
Epidermis
72
In SLE, variable edema and perivascular | inflammation occur in this area
Dermis
73
endocarditis associated with the | use of steroids
Libman sacks endocarditis
74
T or F Vegetation in infective endocarditis are smaller and confined to the lines of closure of the valve leaflets
False Vegetation in Rheumatic heart disease are smaller and confined to the lines of closure of the valve leaflets
75
In SLE, Central penicilliary arteries show concentric intimal and smooth muscle cell hyperplasia producing _________
Onion-skin lesions
76
● Skin manifestations of SLE but rare systemic manifestations ● Skin plaques with varying degrees of edema, erythema, scaliness, follicular plugging and skin atrophy surrounded by an erythematous border ● (+) generic ANAs, (-) anti-dsDNA
CHRONIC DISCOID LUPUS ERYTHEMATOSUS
77
CHRONIC DISCOID LUPUS ERYTHEMATOSUS: IF: _____ deposition at dermoepidermal junction
Ig and C3
78
● Intermediate between SLE and chronic discoid lupus ● Predominant skin involvement ● Skin rash is widespread, superficial and nonscarring ● Mild systemic symptoms consistent with SLE ● (+) anti-SSA and HLA-DR3 phenotype
SUBACUTE CUTANEOUS LUPUS | ERYTHEMATOSUS
79
● Induced by: Hydralazine, procainamide, isoniazid and D-penicillamine ● Anti-TNF therapy + anti-histone antibodies
DRUG-INDUCED LUPUS ERYTHEMATOSUS
80
● chronic inflammatory disease that involves the joints with subsequent involvement of the extra articular tissues such as the skin, blood vessels, lungs, and heart
RHEUMATOID ARTHRITIS
81
● Chronic disease characterized by: | 1) dry eyes (keratoconjunctivitis sicca (2) dry mouth (xerostomia)
SJOGREN SYNDROME
82
aka Sicca syndrome
Primary sjogen syndrome