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Pathology > Hypersensitivity, Autoimmunity and Immunodeficiency Part 2 > Flashcards

Hypersensitivity, Autoimmunity and Immunodeficiency Part 2 Flashcards

1
Q

what are autoimmune diseases

A
  • immune reaction to self-antigens
  • organ or cell specific disorders
  • multi-system disorders (collagen vascular or connective tissue disease)
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2
Q

what are the different types of autoimmune disease

A
  • immunologic tolerance

- self tolerance (central tolerance or peripheral tolerance)

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3
Q

what is central tolerance

A
  • deletion of self reactive T and B cells during development
  • thymus-negative selection by apoptosis of T cell expressing receptor for autologous antigens
  • bone marrow-deletion of self reactive B cells by apoptosis
  • slippage occurs
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4
Q

what is peripheral tolerance

A

-self reactive T cell escape negative selection in thymus and must be deleted from periphery

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5
Q

what is anergy

A
  • encounter with Ag by APC lacking appropriate MHC molecule and costimulatory molecules for T cell
  • lack of specific T helper cell for B cell functional inactivation not death
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6
Q

peripheral suppression is mediated by

A

regulatory T cells which express CD25, a chain of the IL-2 receptor, which require IL-2 for generation and survival
-also express transcription factor FoxP3

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7
Q

mutation of FoxP3 gene are responsible for what

A

systemic autoimmune disease called immune dysregulation, polyendocrinopahy, enteropathy X-linked syndrome (IPEX)

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8
Q

peripheral suppression excrete what

A

immunosuppressive cytokines IL-10 and TGF-B

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9
Q

what is activation-induced cell death

A
  • apoptosis by Fas-Fas ligand system mutations of which cause lymphoproliferative syndrome
  • peripheral suppression by regulatory T cells
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10
Q

what are the mechanisms of autoimmunity

A
  • failure of tolerance:
  • single gene mutation
  • failure of activation - induced cell death
  • breakdown of T cell anergy
  • bypass of B cell requirement for T cell help
  • failure of T cell mediated suppression
  • molecular mimicry
  • polyclonal lymphocyte activation
  • release of sequestered antigens
  • exposure of cryptic self and epitope spreading
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11
Q

what genetic factors are involved in autoimmunity

A
  • Familial clustering

- Linkage with HLA antigens

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12
Q

a greater occurrence of familial clustering occur in

A

monozygotic than dizygotic twins

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13
Q

linkage of HLA antigens are common in what alleles

A

class II alleles (HLA-DR, HLA-DQ)

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14
Q

how do infections in autoimmunity act as triggers

A
  • cross reacting epitopes
  • bypass T cell tolerance by forming immunogenic units
  • act as non-specific polyclonal B cell or T cell mitogens
  • up-regulate co-stimulators via necrosis and inflammation
  • facilitate cryptic antigen presentation and induce epitope spread
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15
Q

what is systemic lupus erythematosis (SLE)

A

-systemic disorder primarily affecting skin, kidneys, serosal membranes, joints and the heart

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16
Q

what type of antibody is involved with SLE

A

ANA -autonuclear antibody

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17
Q

who does SLE affect

A
  • childbearing age
  • African American women
  • presents in second or third decade
    9: 1 female to male
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18
Q

what is the diagnostic criteria for SLE

A

Patient is said to have SLE if any 4 or more of the 11 are present serially or simultaneously during any interval of observation

  • macular rash
  • discoid rash
  • photosensitivity
  • oral ulcers
  • arthritis
  • serositis
  • renal disorder
  • neurological disorder
  • hematologic disorder
  • immunologic disorder
  • antinuclear antibod
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19
Q

SLE is caused by

A
  • defect in self tolerance

- antinuclear antibody (ANA)

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20
Q

what is ANA

A
  • anti DNA
  • anti histone
  • anti non histone proteins bound to RNA
  • anti nucleolar antigens
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21
Q

what are the patterns of immunofluorescence used to diagnosis SLE

A
  1. homogenous or diffuse
  2. Rim or peripheral
  3. Speckled
  4. Nucleolar
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22
Q

what is homogeneous or diffuse patterns of immunofluorescence

A

-chromatin, histones and DS DNA antibodies

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23
Q

what is rim or peripheral patterns of immunofluorescence

A

-double stranded DNA (ds DNA) antibodies

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24
Q

what is speckled patterns of immunofluorescence

A

-most common, histones and ribonucleoprotein antibodies

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25
Q

what are nucleolar patterns of immunofluorescence

A

nucleolar RNA antibodies

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26
Q

what is immunoluorescence

A
  • highly sensitive
  • not highly specific
  • patterns are not absolutely specific for types of antibody
  • antibodies to DS DNA and to Smith (Sm) antigen (non-DNA) are virtually diagnostic of SLE
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27
Q

SLE have antibodies to what

A

RBC
WBC
platlets

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28
Q

40-50% of patients with SLE have what type of antibodies

A

antiphospholipid antibodies

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29
Q

patients with SLE have false positive

A

syphilis serology

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30
Q

what syndrome do SLE patients have

A

lupus anticoagulant or the antiphospholipid antibody syndrome

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31
Q

what are the genetic factors of SLE

A

25% concordance in monozygotic twins vs. 1 to 3% in disygotic twins
-increases risk of developing disease in family members with 20% unaffected showing autoantibodies
-association of HLA-DQ locus and SLE
~6% have deficiencies of complement

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32
Q

what non-genetic factors affect SLE

A
  • drug induced lupus
  • sex hormones
  • UV exposure
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33
Q

what immunologic factors affect SLE

A

CD4 + T cell as effector cell

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34
Q

what is the mechanism of tissue injury in SLE

A
  • autoantibodies as mediator
  • visceral injury by type III hypersensitivity
  • RBC, WBC, and platelet injury by type III hypersensitivity
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35
Q

SLE has what types of bodies or hematoxylin bodies in tissue or cells in vitro

A

LE

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36
Q

SLE is associated with deposition of what

A

immune complexes

  • acute necrotizing vasculitis
  • chronic stages with fibrosis and luminal narrowing
37
Q

what does the skin look like in a SLE patient

A

-malar rash

38
Q

why does the skin in lupus have a malar rash

A
  • liquifactive degeneration of basal layer

- immune complexes and complement at dermal epidermal junction

39
Q

what happens to the joints of SLE patients

A

-swelling with mononuclear cell infiltrate without destruction

40
Q

how does SLE affect the CNS

A
  • focal neurological deficit or neuropsychiatric symptoms
  • intimal proliferation in small vessels due to antiphospholipid antibodies; anti-synaptic membrane protein antibodies have been found
41
Q

what does the spleen of a SLE patient look like

A
  • onion skin lesions

- splenomegaly with follicular hyperplasia and plasma cells in red pulp

42
Q

how does SLE affect serosal membranes

A
  • causes pericardium and pleura
  • serous effusions
  • fibrinous exudates
  • fibrous obliterations of space
43
Q

what effects does SLE have on the heart

A

can cause:

  • pericarditis
  • myocarditis
  • Libman-Sacks endocarditis
  • Coronary artery disease
  • Hypertension
44
Q

what is the most common cause of death in SLE patients

A

renal failure

45
Q

what are the different glomerulonephritis in SLE

A

Class I - normal by LM, EM, IF (rare)
Class II - mesangial lupus nephrtis (20%)
Class III focal proliferative glomerulonephritis (25%)
Class IV - diffuse proliferative glomeruloephritis (~50%) MOST SERIOUS
Class V - membranous glomerulonephritis (15%)

46
Q

what is the clinical course in SLE

A
  • clinical manifestations
  • difficult to diagnose in many cases
  • protean organ and system involvement
47
Q

what are the course variable in SLE

A
  • benign, indolent
  • malignant, rapid
  • remissions and relapses
  • Rx steroids/immunosuppressive
  • 90% 5 year survival
  • 80% 10 year survival
48
Q

see clinical manifestations

A

see clinical manifestations

49
Q

what are the major causes of death in SLE

A
  • renal failure
  • intercurrent infections
  • diffuse CNS involvement
50
Q

what is Sjogren Syndrome

A
  • dry eyes (keratoconjunctivitis sicca)
  • dry mouth (xerostomia)
  • immune mediated destruction of lacrimal and salivary glands (ductal epothelial cells are primary target)
51
Q

what is the primary form of Sjogren syndrome

A

sicca syndrome

52
Q

what is the secondary form of Sjogren Syndrome

A

-associated w/ other autoimmune disorder esp. RA, SLE polymyositis, systemic sclerosis, vasculitis or thyroiditis in ~60% of patients

53
Q

the autoantibodies of Sjogren syndrome are to

A

SS-A (Ro), SS-B (La) RNP ags

54
Q

Sjogren syndrome are associated with what

A

systemic disease and high anti SS-A

55
Q

RF is present in Sjogren syndrome in the absence of

A

RA

56
Q

what cells proliferate in Sjogren syndrome

A

initial polyclonal B cell proliferation

57
Q

what genetic factors affect Sjogren syndrome

A
  • inheritance of certain MHC II molecules

- loss of tolerance of CD4 + T cells

58
Q

what glands are affected in Sjogren syndrome

A

-lacrimal, salivary and other secretory glands

59
Q

what is the morphology of Sjogren syndrome

A
  • intense lymphocyte and plasma cell infiltrates with germinal center formation
  • loss of normal architecture
  • mucosal atrophy
  • ulceration or perforation of nasal septum
  • bronchitis, laryngitis, pneumonia
60
Q

Sjogren syndrome has a ~25% involvement of what

A

the CNS, skin, kidneys and muscle

61
Q

what is the kidney involvement in Sjogren syndrome

A

-usually mild interstitial nephritis

62
Q

Sjogren syndrome can also cause what

A

synovitis, pulmonary fibrosis, peripheral neuropathy

63
Q

Sjogren syndrome has a 40 fold increased risk of developing what

A

non-Hodgkin B cell lymphoma marginal zone lymphoma (MALT)

  • 90% of cases in women btwn 35-45 yrs of age
  • enlargement of salivary glands
  • present with dry mouth and lack of tears
64
Q

what are the different types of systemic sclerosis

A
  1. diffuse scleroderma
  2. limited scleroderma
  3. overlap syndromes
65
Q

what is diffuse scleroderma

A

-systemic widespread skin fibrosis, with rapid progression and early visceral involvement

66
Q

what is limited scleroderma

A
  • CREST syndrome

- calcinosis, Raynaud’s Esophageal dysmotility, Sclerodactyly, Telangiectasia

67
Q

what are overlap syndromes

A

-either diffuse or limited scleroderma with typical features of one or more other autoimmune diseases like MCTD

68
Q

what are the anti nuclear protein antibodies in systemic sclerosis

A
  1. Scl-70
    - Diffuse scleroderma (70%)
    - DNA topoisomerase 1
  2. centromere
    - limited scleroderma (90%)
69
Q

visceral involvement of systemic sclerosis includes

A

GI tract, lungs, kidneys, heart and skeletal muscle

70
Q

systemic sclerosis occurs most commonly in who

A

women in the 3rd to 5th decades

3:1 female to male

71
Q

what is the mechanism of systemic sclerosis

A
  • interaction of CD4+ T cells, endothelial injury and fibroblast activation by IL-1, TNF, PDGF, TGF-B and fibroblast growth factors
  • B cell activation with ANAs and hypergammaglobulinemia
  • microvascular disease present early on progressing to ischemic injury
72
Q

what are the effects of systemic sclerosis on the skin

A
  • diffuse sclerotic atrophy

- edema at first with ultimate claw like deformity

73
Q

what are the effects of systemic sclerosis on the GI tract

A

90% of patients esp. esophagus –> Barrett’s small bowel loss of villi and microvilli –> malabsorption

74
Q

what are the effects of systemic sclerosis on the musculoskeletal system

A
  • synovial hyperplasia and inflammation w/o deformity

- 10% get inflammatory myositis

75
Q

how does systemic sclerosis affect the lungs

A

50% of patients pulmonary HTN and/or interstital fibrosis

76
Q

how does systemic sclerosis affect the kidney

A

2/3 patients change in interlobular arteries similar to those of malignant HTN
-HTN occurs in 30% and 20% of those develop malignant HTN–> renal failure and death

77
Q

how does systemic sclerosis affect the heart

A
  • patchy fibrosis and thickening of intramyocardial arteries in 1/3 “cardiac Raynaud”
  • Cor pulmonale and RV hypertrophy due to lung changes
78
Q

what is the clinical course of systemic sclerosis

A
  • women 50-60 yrs of age
  • nearly all develop Raynaud’s phenomenon
  • hands atrophy and become immobile
  • dysphagia from esophageal involvement
  • malabsorption
  • dyspnea and chronic cough
  • pulmonary HTN with cor pulmonale
  • renal failure may lead to malignant HTN
79
Q

continuation of clinical course of systemic sclerosis

A

-most progress slowly and steadily downhill over many yrs
-life span normal if no renal involvement
-10 yr survival is 35-70%
CREST has much better prognosis
-begins frequently with Raynaud’s phenomenon with face and hand involvement only for many yrs

80
Q

what is inflammatory myopathies

A

-rare disorders with immune mediated muscle injury and inflammation which occur alone or with other disorder such as systemic sclerosis

81
Q

inflammatory myopathies can lead to

A
  • polymyositis
  • dermatomyositis - women have increased risk of developing visceral cancers (lung, ovary, stomach)
  • inclusion body myositis
82
Q

what is characteristic of inflammatory myopathies

A

-symmetric muscle weakness beginning in large muscles of trunk, neck and limbs with difficulty climbing stairs or rising from a chain

83
Q

what will be seen histologically in inflammatory myopathies

A

-histology lymphocytic infiltration and degenerative and regenerating muscle fibers

84
Q

what immunologic evidence in inflammatory myopathies

A
  • immunological evidence of antibody mediated injury in dermatomyositis
  • immunological evidence of T cell mediated injury in polymyositis and inclusion body myositis
85
Q

what antibody is characteristic of inflammatory myopathies

A

Jo-1 antibodies (tRNA synthetase)

86
Q

how do you diagnose inflammatory myopathies

A
  • diagnosis on clinical features
  • increase creatine kinase
  • EMG
  • biopsy
87
Q

patients with mixed connective tissue disease have symptoms of

A

-autoimmune disease, high tigers and antibodies to RNP antigen UIRNP

88
Q

mixed connective tissue disease responds well to what class of drug

A

corticosteroids

Pathology (18 decks)

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