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MOD Final > Diseases of Immunity > Flashcards

Diseases of Immunity Flashcards

1
Q

What is central tolerance and where does it occur?

A

it is learned tolerance prior to release from generative organs; occurs in the thymus and in the bone marrow

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2
Q

What happens if B cells recognize and react to their own self antigens?

A

they have the opportunity for receptor editing or they undergo apoptosis

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3
Q

What are the mechanisms of peripheral tolerance?

A

T regulatory suppression and anergy

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4
Q

How does anergy occur?

A

T lymphocytes have inhibitory receptors; our self cells express ligands and if the T cells bind to these ligands on the self cell they undergo anergy

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5
Q

what are the inhibitory receptors that are found on T lymphocytes that are important in the process of anergy?

A

CTLA and PD-1

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6
Q

what do our self cells express that can bind to the T lymphocytes and induce anergy?

A

CD 80/86 or PDL1

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7
Q

What are three different mechanisms for autoimmune diseases?

A

susceptibility genes, molecular mimicry, and epitope spreading

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8
Q

what autoimmune diseases occur via the mechanism of susceptibility genes?

A

ankylosing spondylitis, rheumatoid arthritis, and Crohn’s disease

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9
Q

what autoimmune diseases occur via the mechanism of molecular mimicry?

A

rheumatic heart disease

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10
Q

what autoimmune diseases occur via the mechanism of epitope spreading?

A

oral lichen planus

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11
Q

What is the susceptible gene that is associated with ankylosing spondylitis?

A

B27

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12
Q

what is ankylosing spondylitis/ how does it present?

A

it is a hereditary inflammatory condition of the joints; young patients present with severe neck and back pain; bamboo spine

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13
Q

What is the susceptible gene associated with rheumatoid arthritis?

A

PTPN22 (protein tyrosine phosphatase)

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14
Q

what does the polymorphism of the PTPN22 gene lead to?

A

there is not adequate elimination of the self reactive clones, so you don’t suppress your T cell response; leads to increased inflammation; they have a direct influence on reducing B-cell and T-cell tolerance

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15
Q

What is the susceptible gene associated with Crohn’s disease?

A

NOD-2 gene

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16
Q

what happens in cases of polymorphisms of the NOD-2 gene?

A

paneth cells in the intestinal epithelium are ineffective at killing microbes; so there is a significant overgrowth of bacteria and an accompanying inflammatory response

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17
Q

what is molecular mimicry?

A

when there is antigenic similarity between true pathogens and native tissues leading to autoimmunity

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18
Q

What is the mechanism of rheumatic heart disease?

A

molecular mimicry

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19
Q

What occurs during epitope spreading?

A

some of our antigens are hiding intracellularly or they are just anatomically hidden; this means that if something happens to breakdown the intact structures or cells, we can spill out antigens that our immune system has never seen before

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20
Q

during oral lichen planus, what happens during the initial T response?

A

leads to keratotic lesions in the oral and conjunctival mucosa, which leads to basement membrane disruption exposing antigenic proteins (lichen planus)

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21
Q

what is the secondary disease that follows Lichen Planus?

A

Pemphigoid (a secondary B cell response)

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22
Q

If you suspect an autoimmune disease might be occurring in your patient, what would be one of the first test you order?

A

ANA (anti-nuclear antibody) test

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23
Q

if a patient tests positive for ANA, what test would you look for to be positive if they had lupus?

A

anti DS DNA or anti smith

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24
Q

if a patient tests positive for ANA, what test would you look for to be positive if they had Sjogren syndrome?

A

Anti Ro/ SS-A or Anti La/ SS-B

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25
Q

if a patient tests positive for ANA, what test would you look for to be positive if they had systemic sclerosis?

A

Anti DNA topoisomerase (Scl-70)

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26
Q

what is the staining pattern associated with systemic lupus erythematous?

A

homogenous

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27
Q

what is the staining pattern associated with systemic sclerosis?

A

nucleolar (or speckled)

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28
Q

what is the staining pattern associated with CREST syndrome?

A

centromeric

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29
Q

what is the genetic association with Lupus?

A

family patterns or HLA-DQ possibly

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30
Q

what is the gender bias associated with lupus?

A

females get it more often than males

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31
Q

what environmental factor is associated with increased risk of developing lupus?

A

UV radiation

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32
Q

there are multiple immune mechanisms involved in systemic lupus erythematous, what are 2 examples?

A

B cells and CD-4 T cells, and immune complex formation (TYPE III HYPERSENSITIVITY)

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33
Q

what is the most sensitive/consistent thing you are going to see present in patients with lupus?

A

an abnormal CBC (could be a hemolytic anemia, thrombocytopenia, or leukopenia

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34
Q

What is the most common pattern of lupus nephritis?

A

diffuse lupus nephritis (class IV)

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35
Q

how do patients with diffuse lupus nephritis typically present? and why?

A

they present with complaints of proteinuria and hematuria; the immune complexes have landed in the glomerular capillary loop

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36
Q

how can you identify diffuse lupus nephritis on a regular microscope?

A

the mesangial cells are proliferating as the damage is occurring there; the glomeruli show increased cellularity

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37
Q

what does immunofluorescence of diffuse lupus nephritis show?

A

a granular pattern of IgG antibody-containing complexes ; tends to give a very patchy appearance

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38
Q

what is a cardiac complication associated with lupus?

A

Libman-Sacks endocarditis and CAD

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39
Q

what is libman-sacks endocarditis?

A

verrucous (warty) valve deposits compromised of fibrin; these are not infective and they can rarely embolize

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40
Q

what might cause lupus patients to be more susceptible for CAD?

A

the contribution from anti-phospholipid antibody syndrome

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41
Q

What histological feature is always associated with Lupus and what is this?

A

an L-E cell; what happens in lupus is your phagocytic cells (e.g. neutrophils) find damaged cells and swallow them; the neutrophils become so full that it had to squash its own nucleus

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42
Q

What is discoid lupus?/ how does discoid lupus present

A

discoid rashes on face and scalp; these patients don’t tend to have renal involvement or cardiac involvement; just the skin involvement

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43
Q

why is discoid lupus hard to make a diagnosis?

A

these patients often times do not have a positive ANA test and when they are positive they do not have specific markers

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44
Q

what is drug-induced lupus/ how does it present?

A

a very specific scenario; an older, cardiac patient that you put on a new drug and then they develop lupus; it is a medication induced breakdown of self-tolerance; they have arthralgia, fever, and cutaneous lesions

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45
Q

what do patients with drug-induced lupus test positive for?

A

ANA and anti-histone antibodies

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46
Q

what is sjogren syndrome?

A

an autoimmune disease resulting in the destruction of lacrimal and salivary gland tissue

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47
Q

what is the pathogenesis of sjogren syndrome?

A

b and t cell mediated inflammatory reaction to target tissues with inflammatory damage followed by fibrotic destruction

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48
Q

what is the clinical presentation of sjogren syndrome?

A

dry irritated eyes, dry mouth (xerostomia), and difficulty swallowing

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49
Q

how can you make the diagnosis of sjogren syndrome?

A

if anti-Ro/ss-a or anti-La/ss-B is present; or you can take a biopsy of the lip to look for inflammation of minor salivary gland tissue

50
Q

what are the complications associated with sjogren syndrome?

A

extraglandular disease: pulmonary fibrosis; or the lymphoid proliferation becoming clonal leading to lymphoma

51
Q

what test is used to evaluate dry eyes?

A

Schiermer’s test

52
Q

what can dry mouth lead to?

A

root caries and papillary atrophy (smooth tongue)

53
Q

what is systemic sclerosis?

A

fibrosis throughout the body; there is dense collagenous deposition consistent with subcutaneous fibrosis; you will seen vascular hyalinization (vascular changes)

54
Q

how does systemic sclerosis manifest? (7)

A

sclerodactyly: the hands get this contracted appearance and the skin has become thick and keratotic; you can also see features of Raynaud phenomenon; reflex, esophageal ulceration; renal crisis; pulmonary hypertension, or pulmonary fibrosis

55
Q

if you think someone has systemic sclerosis and you do the ANA IF test and it shows a centromeric pattern, what do they most likely have?

A

CREST syndrome

56
Q

what is CREST syndrome?

A

it is like systemic sclerosis but unique; it has a better prognosis than diffuse sclerosis

57
Q

what are the symptoms/manifestations of CREST syndrome?

A

C: calcinosis R: Raynaud’s phenomenon; E: esophageal dysfunction; S: sclerodactyly; T: telangiesctasis

58
Q

what is telangiectasias?

A

dilation of capillaries causing red marks on the surface of the skin

59
Q

how might a patient present with complaints of calcinosis?

A

hard skin nodules, calcium deposits, feels like marbles under skin

60
Q

what do patients with CREST syndrome have a high titer of?

A

anti-ribonucleoprotein (RNP)

61
Q

what is IgG4 related disease?

A

a constellation of disorders characterized by tissue infiltrates dominated by IgG4 antibody producing plasma cells and fibrosis

62
Q

what diseases are tied together in one disease known as IgG4 related disease?

A

autoimmune pancreatitis, riedel thyroiditis, mikulicz’s syndrome, idiopathic retroperitoneal fibrosis

63
Q

what could be seen of the bile duct in an IgG4 related disease?

A

the bile duct with sclerosing cholangitis

64
Q

what is involved in the direct pathway of allorecognition?

A

an APC from the donor presents the antigen to the host’s T cell; no humeral response involved in the direct pathway

65
Q

What is involved in the indirect pathway of allorecognition?

A

an APC of the recipient goes into the graft and pulls out the antigens from the graft and shows them to the recipient’s T cells

66
Q

which pathway in allorecognition involves both T cell responses and humeral response?

A

the indirect pathway

67
Q

Which types of rejection are cell-mediated?

A

acute cellular rejection and chronic cellular rejection

68
Q

which types of rejection are humoral?

A

hyperacute rejection, acute antibody-mediated rejection, chronic antibody-mediated rejection

69
Q

What is hyperacute rejection mediated by?

A

pre-formed antibodies

70
Q

what is hyperacute rejection marked inflammation followed by?

A

thrombotic microvasculopathy

71
Q

why would a recipient receive a graft for the first time and already have antibodies against it?

A

blood group antigens- we are constantly exposed to blood group antigens through bacteria

72
Q

what is chronic cellular rejection attacking?

A

the arteries and it creates arteriosclerosis

73
Q

what is specific to chronic rejection?

A

fibrosing/ sclerosing process

74
Q

How do we tell the difference between the antibody-mediated type of rejection and other forms pf cellular rejection?

A

a C4d stain

75
Q

What are transplant patients who are on immunosuppressive medications at risk for?

A

infections; viral: polymavirus and cytomegalovirus; fungal infections, and bacteria infections; also at risk for tumors

76
Q

What tumors are transplant patients who are on immunosuppressive medications at risk for?

A

viral induced tumors: lymphomas, Kaposi sarcomas; squamous carcinomas

77
Q

What occurs during a graft versus host disease?

A

T-lymphocyte mediated attack on host after transplantation

78
Q

what are common transplants that have graft versus host reactions?

A

bone marrow, thymus, and liver

79
Q

What are the manifestations of graft versus host disease?

A

skin: rash–> desquamation; liver: jaundice–> cholestasis; intestines: bloody diarrhea–> strictures

80
Q

What is leukocyte adhesion deficiency type 1?

A

defective integrins that help it anchor

81
Q

what is leukocyte adhesion deficiency type 2?

A

lack of selectin ligand so it can’t initially stop in the circulation

82
Q

what causes LAD type 2?

A

there is no fucosyl transferase

83
Q

what is the inheritance pattern of chediak higashi syndrome?

A

autosomal recessive

84
Q

what occurs during Chediak higashi syndrome?

A

there is failure of phagolysosomal fusion

85
Q

What are some of the clinical presentations of Chediak higashi syndrome?

A

easy bleeding/ bruising from platelet dysfunction, grey hair, albinism

86
Q

What is chronic granulomatous disease?

A

group of genetic disorders; any genetic disorder that affects the effective killing by ROS; the killing is so inadequate that the body is launching a plan B, which is to basically wall off the infection with granulomas

87
Q

What do LAD, Chediak-Higashi syndrome, and Chronic granulomatous disease all have in common?

A

they all share recurrent bacterial infections

88
Q

what type of infections are LAD, CGD, and Chediak higashi susceptible to?

A

catalase negative bacteria: streptococcus, enterococcus, staphylococcus aureus

89
Q

Without the MAC complex, what are patients more susceptible to?

A

Neisseria meningitis

90
Q

what happens if you don’t have C1-INH?

A

unregulated Kallikrein and complement pathways result in severe kinin (bradykinin-) mediated edema; hereditary angioedema

91
Q

what is the inheritance pattern of hereditary angioedema?

A

autosomal dominant

92
Q

What is the inheritance pattern of SCID?

A

x-linked

93
Q

What is ADA deficiency?

A

adenosine deaminase deficiency; accumulation of toxic purine metabolites

94
Q

What is the effect of ADA deficiency?

A

it blocks T lymphocyte formation, some B cell influence but lack of helper T cells suppress humoral immune function

95
Q

What are the disorders of leukocyte function?

A

leukocyte adhesion deficiency, Chediak Higashi, Chronic granulomatous disease

96
Q

what are the disorders of complement function?

A

Membrane attack complex deficiency and hereditary angioedema

97
Q

what are the disorders of lymphocyte maturation?

A

SCID syndrome, X-linked agammaglobulinemia, DiGeorge Syndrome

98
Q

What are the disorders of lymphocyte function?

A

Hyper IgM syndrome, common variable immunodeficiency, isolated IgA deficiency, and X-linked immunoproliferative disorders

99
Q

what are the immunodeficiencies associated with systemic disease?

A

Wiskott Aldrich syndrome and Ataxia-Telangiectasia

100
Q

What is the main cause of DiGeorge syndrome?

A

22q11 deletion

101
Q

what all can be seen in DiGeorge syndrome?

A

facial and palatal abnormalities, cardiac abnormalities, tetany due to hypocalcemia, immune deficiency due to thymus aplasia

102
Q

what is x-linked agammaglobulinemia?

A

a defect in the bruton tyrosine kinase (BTK) gene on the X chromosome

103
Q

what is the effect of x-linked agammaglobulinemia?

A

inability of pre-B cells to mature (light chains cannot be rearranged); risk for infection increases

104
Q

What is a classic presentation of agammmaglobulinemia?

A

encapsulated bacteria are not phagocytized; so patients come in with recurrent infections of encapsulated bacteria: streptococcus pneumoniae, Haemophilius influenza type B, streptococcus pyogenes, pseudomonas aeruginosa, staph aureus, and protozoa: giardia

105
Q

What causes hyper- IgM syndrome?

A

CD40/CD40L mutations leads to the interference of T cells helping B ells class switch

106
Q

where is CD40L found?

A

on T cells

107
Q

where is CD40 found?

A

on B cells

108
Q

How do you treat hyper IgM syndrome?

A

IVIg or stem cell transplantation

109
Q

What is unique about Common variable Immunodeficiency?

A

you actually get increased autoimmunity

110
Q

What is the effect of IgA deficiency?

A

less defense against inhaled and ingested pathogens: more sinus and respiratory infections, urinary bladder infections, GI infections

111
Q

What is a possible first indication of IgA deficiency?

A

transfusion-related anaphylaxis

112
Q

Why is anal receptive sexual intercourse a common route of transmission of HIV?

A

because the mucosal barrier is very thin

113
Q

what effect would a primary chancre of syphillis have on HIV transmission?

A

it would have a synergistic effect

114
Q

what is the most common form of parenteral transfer of HIV?

A

IV drugs

115
Q

what region of HIV initiates transcription and binds transcription factors?

A

LTR

116
Q

what region of HIV encodes for the proteins inside of the virus?

A

gag

117
Q

what region of HIV encodes for the surface glycoproteins?

A

env

118
Q

what region of HIV encodes for the viral enzymes?

A

pol

119
Q

how does HIV bind to a CD4 T cell?

A

gp 120 binding

120
Q

how does HIV drill down into the CD 4 T cell?

A

gp 41

MOD Final (11 decks)

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