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
Q

Sjögren syndrome tests

A

anti Ro/SS-A, anti La/SS-B

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26
Q

Systemic sclerosis tests

A

anti DNA topoisomerase

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27
Q

Four staining patterns of ANAs

A

homogenous, speckled, nucleolar, centromere

28
Q

Etiology of SLE

A

genetic associations: family patterns, HLA-DQ, X chromosome (female bias)
Environmental influences: UV light
Combine to degrade self tolerance

29
Q

SLE criteria

A

malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neuro disorder, hematologic disorder, ANA, immunologic disorder

30
Q

When to consider/suspect SLE

A

arthritis, skin rashes, fever, fatigue, hemolytic anemia, edema

31
Q

What stage do people with SLE often present?

A

Class IV, patients are symptomatic (proteinuria, hematuria), glomeruli show increased cellularity, immune deposits in subendothelium, proliferation of endothelial, mesangial, and epithelial cells

32
Q

Immunofluorescence pattern in diffuse lupus nephritis

A

Granular pattern of IgG antibody-containing complexes

33
Q

Skin changes in SLE

A

basal layer degeneration of skin, vacuolate spaces between degenerating cells, positive immunofluorescence

34
Q

CV complications in SLE

A

Libman-sacks endocarditis (verrucous valve deposits of fibrin) and coronary artery disease

35
Q

L-E cell

A

neutrophil or macrophage that ingests nucleus of damaged cell

36
Q

Similarities between SLE and DiscoidLE

A

discoid rash, positive ANA, positive immunofluorescence

37
Q

Discoid lupus tests

A

positive ANA, negative anti-DS DNA

38
Q

Similarities between SLE and Drug-inducedLE

A

arthalgias, fever, positive ANA, discoid rash, hematologic disease, positive immunofluorescence

39
Q

Etiology of drug-induced lupus

A

medication-induced breakdown of self tolerance (procainamide, hydralazine)

40
Q

Drug-induced lupus tests

A

positive ANA, positive anti-histone Ab

41
Q

HLA risk linkage of drug-induced lupus in use of hydralazine

A

HLA-DR4 allele

42
Q

HLA risk linkage of drug-induced lupus in use of procainamide

A

HLA-DR6 allele

43
Q

Sjögren syndrome

A

autoimmune disease resulting in destruction of lacrimal and salivary gland tissue

44
Q

Pathogenesis of Sjögren syndrome

A

B and T cell mediated inflammatory reaction to target tissues with inflammatory damage followed by fibrotic destruction

45
Q

Sxs of Sjögren syndrome

A

dry eyes, dry mouth (resulting in root caries), smooth tongue(papillary atrophy)

46
Q

Clinical presentation of Sjögren syndrome

A

dry, irritated eyes, dry mouth, difficulty swallowing

47
Q

Diagnosis of Sjögren syndrome

A

anti-ro/ss-a and anti-la/ss-b, biopsy to look for inflammation of minor salivary gland tissue

48
Q

Possible complications of Sjögren syndrome

A

extraglandular disease, pulmonary fibrosis, lymphoma

49
Q

Systemic sclerosis

A

fibrosis throughout the body - skin, GI tract, kidneys, heart, lungs

50
Q

Skin morphology of systemic sclerosis

A

dense collagenous deposition consistent with subcutaneous fibrosis, vascular hyalinization, sclerodactyly, Raynaud’s phenomenon

51
Q

Manifestations of systemic sclerosis

A

GI reflux, esophageal ulceration, renal vascular disease, pulmonary hypertension, pulmonary fibrosis

52
Q

Immunofluorescence ANA pattern for systemic sclerosis

A

speckled, anti Slc-70

53
Q

Immunofluorescence ANA pattern for CREST syndrome

A

centromere, anticentromere antibody

54
Q

Crest Syndrome

A

unique form of limited sclerosis - calcinosis, raynaud’s phenomenon, esophageal dysfunction, sclerodactyly, telangestica

55
Q

Mixed connective tissue disease

A

symptoms of SLE, systemic sclerosis, polymyositis; not well understood, high titer for anti-RNP; most common feature is Raynaud phenomenon

56
Q

IgG4-related disease

A

group of diseases with common pathophys – autoimmune pancreatitis, Riedel thyroiditis, Mikulicz’s syndrome, idiopathic retroperitoneal fibrosis, inflammatory pseudotumors, inflammatory aortitis

57
Q

Pathophys of IgG4 related disorders

A

IgG4-producing plasma cells, t lymphocytes, fibrosis, fibrotic scarring and irreversible damage to involved areas

58
Q

Allorecognition

A

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
Q

Timing of rejection

A

hyperacute rejection, acute rejection, acute ab-mediated rejection, chronic rejection, chronic ab-mediated rejection

60
Q

Hyperacute rejection

A

occurs within minutes to hours, mediated by preformed antibodies, marked inflammation followed by thrombotic microvasculopathy

61
Q

Acute ab-mediated rejection

A

inflammation with complement C4d breakdown product

62
Q

Chronic ab-mediated rejection

A

fibrosis with primary effect on vessels

63
Q

Acute cellular rejection

A

occurs within days, months, years of transplant; T-cell mediated response

64
Q

Treatments for transplant rejection

A

corticosteroids (immunosuppressive), T lymphocyte-mediated cell rejection (Tacromilus), Ab-mediated rejection (immune globulin, rituximab

65
Q

Hematopoietic stem cell transplant

A

therapy for hematologic malignancies, ablative chemo and radiation destroys recipient marrow, stem cell transplant administered and recipient essentially has a new immune system

66
Q

Graft versus host disease

A

t-lymphocyte mediated response; skin rash, jaundice/cholestasis, bloody diarrhea->strictures