Diseases of Immunity Pt 2 Flashcards

1
Q

Requirements to characterize autoimmune disease

A

presence of immune reaction specific for self ag or tissue, evidence that a rxn is not secondary to tissue damage but is of pathogenic significance, absence of another well-defined cause of disease

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2
Q

Immunologic tolerance

A

unresponsiveness to ag induced by exposure of lymphocytes to ag

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3
Q

Self tolerance

A

lack of responsiveness to individual’s own ag

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4
Q

Central tolerance

A

learned tolerance prior to release from generative lymphoid organs

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5
Q

Negative selection

A

process responsible for eliminating self-reactive lymphocytes from T-cell pool

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6
Q

AIRE

A

autoimmune regulator, protein that stimulates expression of some “peripheral-tissue-restricted” self ags in thymus and is critical for deletion of self-reactive T cells

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7
Q

Receptor editing

A

reactivation of B cell machinery to create a new antigen receptor gene rearrangement, not specific for self antigens

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8
Q

Peripheral tolerance

A

ongoing regulation in peripheral tissues

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9
Q

Anergy

A

lymphocytes rendered functionally unresponsive

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10
Q

Inhibitory T cell receptors

A

CTLA, PD-1; downregulate T cells when self ags are present

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11
Q

Most common regulatory T cell features

A

induced by TGF-B, CD4+, express CD25 and FOXP3

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12
Q

Actions of regulatory T cells

A

cytokine immunosuppression (IL-10, TGF-B), CTLA-4 inhibition

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13
Q

Mutations in AIRE cause what?

A

autoimmune polyendocrinopathy

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14
Q

IPEX

A

Immune dysregulation, polyendocrinopathy, enteropathy, X-linked; systemic autoimmunity

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15
Q

IPEX mutation

A

FOXP3

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16
Q

What factors contribute to the genesis of autoimmune diseases?

A

susceptibility genes, infection, damaged tissues

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17
Q

Pathology of ankylosing spondylitis

A

inflammatory condition of joints that leads to degeneration and fusion of vertebrae

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18
Q

Etiology of ankylosing spondylitis

A

typically hereditary, associated with Class I HLA allele B27

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19
Q

Etiology of Crohn disease

A

polymorphisms in NOD-2 gene render paneth cells in intestinal epithelium ineffective at microbe killing

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20
Q

Pathogenesis of Crohn disease

A

defective killing and clearance allows accumulation of bacteria and an exaggerated immune response

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21
Q

Pathogenesis of oral lichen planus

A

epitope spreading; initial T cell response leads to keratotic lesions in oral mucosa, basement membrane disruption exposes antigenic proteins, secondary B cell response occurs and results in blistering

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22
Q

Autoimmune diseases with anti-nuclear antibody

A

SLE, Sjögren syndrome, systemic sclerosis

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23
Q

Sensitivity of ANA test

A

sensitive for multiple diseases, will be negative the patient does not have the disease, will prompt more specific testing

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24
Q

SLE tests

A

Anti DS DNA, Anti smith

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25
Sjögren syndrome tests
anti Ro/SS-A, anti La/SS-B
26
Systemic sclerosis tests
anti DNA topoisomerase
27
Four staining patterns of ANAs
homogenous, speckled, nucleolar, centromere
28
Etiology of SLE
genetic associations: family patterns, HLA-DQ, X chromosome (female bias) Environmental influences: UV light Combine to degrade self tolerance
29
SLE criteria
malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neuro disorder, hematologic disorder, ANA, immunologic disorder
30
When to consider/suspect SLE
arthritis, skin rashes, fever, fatigue, hemolytic anemia, edema
31
What stage do people with SLE often present?
Class IV, patients are symptomatic (proteinuria, hematuria), glomeruli show increased cellularity, immune deposits in subendothelium, proliferation of endothelial, mesangial, and epithelial cells
32
Immunofluorescence pattern in diffuse lupus nephritis
Granular pattern of IgG antibody-containing complexes
33
Skin changes in SLE
basal layer degeneration of skin, vacuolate spaces between degenerating cells, positive immunofluorescence
34
CV complications in SLE
Libman-sacks endocarditis (verrucous valve deposits of fibrin) and coronary artery disease
35
L-E cell
neutrophil or macrophage that ingests nucleus of damaged cell
36
Similarities between SLE and DiscoidLE
discoid rash, positive ANA, positive immunofluorescence
37
Discoid lupus tests
positive ANA, negative anti-DS DNA
38
Similarities between SLE and Drug-inducedLE
arthalgias, fever, positive ANA, discoid rash, hematologic disease, positive immunofluorescence
39
Etiology of drug-induced lupus
medication-induced breakdown of self tolerance (procainamide, hydralazine)
40
Drug-induced lupus tests
positive ANA, positive anti-histone Ab
41
HLA risk linkage of drug-induced lupus in use of hydralazine
HLA-DR4 allele
42
HLA risk linkage of drug-induced lupus in use of procainamide
HLA-DR6 allele
43
Sjögren syndrome
autoimmune disease resulting in destruction of lacrimal and salivary gland tissue
44
Pathogenesis of Sjögren syndrome
B and T cell mediated inflammatory reaction to target tissues with inflammatory damage followed by fibrotic destruction
45
Sxs of Sjögren syndrome
dry eyes, dry mouth (resulting in root caries), smooth tongue(papillary atrophy)
46
Clinical presentation of Sjögren syndrome
dry, irritated eyes, dry mouth, difficulty swallowing
47
Diagnosis of Sjögren syndrome
anti-ro/ss-a and anti-la/ss-b, biopsy to look for inflammation of minor salivary gland tissue
48
Possible complications of Sjögren syndrome
extraglandular disease, pulmonary fibrosis, lymphoma
49
Systemic sclerosis
fibrosis throughout the body - skin, GI tract, kidneys, heart, lungs
50
Skin morphology of systemic sclerosis
dense collagenous deposition consistent with subcutaneous fibrosis, vascular hyalinization, sclerodactyly, Raynaud's phenomenon
51
Manifestations of systemic sclerosis
GI reflux, esophageal ulceration, renal vascular disease, pulmonary hypertension, pulmonary fibrosis
52
Immunofluorescence ANA pattern for systemic sclerosis
speckled, anti Slc-70
53
Immunofluorescence ANA pattern for CREST syndrome
centromere, anticentromere antibody
54
Crest Syndrome
unique form of limited sclerosis - calcinosis, raynaud's phenomenon, esophageal dysfunction, sclerodactyly, telangestica
55
Mixed connective tissue disease
symptoms of SLE, systemic sclerosis, polymyositis; not well understood, high titer for anti-RNP; most common feature is Raynaud phenomenon
56
IgG4-related disease
group of diseases with common pathophys -- autoimmune pancreatitis, Riedel thyroiditis, Mikulicz's syndrome, idiopathic retroperitoneal fibrosis, inflammatory pseudotumors, inflammatory aortitis
57
Pathophys of IgG4 related disorders
IgG4-producing plasma cells, t lymphocytes, fibrosis, fibrotic scarring and irreversible damage to involved areas
58
Allorecognition
direct pathway: donor APC in the graft triggers a rejection response to donor tissues Indirect pathway: recipient APC presents graft cell ag and triggers antibody production and macrophage activation
59
Timing of rejection
hyperacute rejection, acute rejection, acute ab-mediated rejection, chronic rejection, chronic ab-mediated rejection
60
Hyperacute rejection
occurs within minutes to hours, mediated by preformed antibodies, marked inflammation followed by thrombotic microvasculopathy
61
Acute ab-mediated rejection
inflammation with complement C4d breakdown product
62
Chronic ab-mediated rejection
fibrosis with primary effect on vessels
63
Acute cellular rejection
occurs within days, months, years of transplant; T-cell mediated response
64
Treatments for transplant rejection
corticosteroids (immunosuppressive), T lymphocyte-mediated cell rejection (Tacromilus), Ab-mediated rejection (immune globulin, rituximab
65
Hematopoietic stem cell transplant
therapy for hematologic malignancies, ablative chemo and radiation destroys recipient marrow, stem cell transplant administered and recipient essentially has a new immune system
66
Graft versus host disease
t-lymphocyte mediated response; skin rash, jaundice/cholestasis, bloody diarrhea->strictures