6/29 - Diseases of Immune System Flashcards

1
Q

what is the act of transferring cells, tissues, or organs from one site to another; typically between different individuals

A

transplantation

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2
Q

can organ system malfunction be corrected w/ transplantation of organ from a donor

A

YES

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3
Q

what is the most formidable barrier to transplantation as a routing medical treatment? this results in what?

A

immune system is a barrier - results in rejection of transplanted organs

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4
Q

what are the different graft types

A
  1. autograft
  2. isograft
  3. allograft
  4. xenograft
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5
Q

what is a graft in the same person from one part of body to area

A

autograft

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6
Q

what is the graft between identical twins

A

isograft

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7
Q

what is the graft between different members of the same species

A

allograft

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8
Q

what is the graft between two different species

A

xenograft

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9
Q

the ability of recipient T cells to recognize donor derived antigens is called what

A

allorecognition

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10
Q

what initiates allograft rejection

A

allorecognition

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11
Q

once recipient T cells become active, they under go clonal expansion, differentiate into effector cells, and ___

A

migrate into the graft where they promote tissue destruction

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12
Q

how to increase graft survival

A
  1. good MHC (HLA) matching of donor and recipient (MHC I and II)
  2. immunosuppression
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13
Q

when is immunosuppression not needed when grafting

A

with identical (monozygotic twins)

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14
Q

what occurs when immunologically compentent cells or their precursors are transplanted into immunologically crippled recipients

A

graft vs host disease (GVHD)

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15
Q

what occurs when transferred cells recognize alloantigens in the host and attack host tissues

A

GVHD

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16
Q

what is the pathophysiology of GVHD

A

The interaction of the donor T cell and the host APC leads to the activation of APC and immunogenic presentation of HLA class I-restricted minorhistocompatibility antigens (“license to kill”) to activate CD8 cells of the transplantation that can attack mHA on epithelial tissue or also on hematopoietic tissue

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17
Q

what is a major cause of morbidity and mortality in allogenic bone marrow transplantation, with increasing incidene of it corresponding to level of HLA mismatch between donor and host

A

GVHD

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18
Q

what causes epithelial cell neocrosis

A

GVHD

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19
Q

where does GVHD cause epithelial cell necrosis

A

liver, skin, GI tract

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20
Q

is GVHD lethal

A

yes, potentially lethal process

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21
Q

what are immune rxns against self antigens

A

autoimmune disorders

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22
Q

what is the phenomenon of unresposiveness to an antigen induced by exposure to lymphocytes to that antigen

A

immunologic tolerance

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23
Q

autoimmunity arises from combination of what

A
  1. inheritance of susceptibility genes
  2. environmental triggers
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24
Q

what contributes to the breakdown of self-tolerance

A

inheritance of susceptibility genes

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25
Q

what promote the activation of self-reactive lymphocytes, such as infections and tissue damage

A

environmental triggers

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26
Q

general features of autoimmune diseases

A
  1. chronic, w/ relapses and remissions, and damage is progressive
  2. manifestations determined by nature of underlying immune response
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27
Q

genetic factors in autoimmunity have an evidence of ___ clustering and linkage w/ ___

A

evidence of familial clustering and linkage w/ certain HLA types, especially class II antigens

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28
Q

who has a greater incidence of most autoimmune disorders

A

women

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29
Q

what are implicated in autoimmunity

A

bacteria, mycoplasmas, and viruses

30
Q

which diseases are exceptions to the concept that women have the greater incidence of autoimmune disorders?

A
  1. ulcerative colitis (50:50)
  2. diabetes mellitus (more men than women)
31
Q

what is the most common connective tissue disease in US

A

lupus erythematosus (LE)

32
Q

what are the 3 forms of LE

A
  1. Systemic Lupus erythematosus
  2. Chronic cutaneous erythematosus
  3. Subacute cutaneous erythematosus
33
Q

what disease results from increased activity of humoral limb (B lymph) of immune system

A

LE

34
Q

what disease results from abnormal function of T lymphocytes

A

LE

35
Q

do we know the exact pahtogenesis of LE

A

no

36
Q

does genetic and environmental factors play a role in LE

A

YES

37
Q

what disease exhibits butterfly rash

A

systemic lupus erythematosus

38
Q

what disease:

  • serious multisystem
  • variety of cutaneous and oral manifestations
  • difficult to diagnose at early stage
  • mainly women
A

systemic lupus erythematosus

39
Q

what is the average age of diagnosis of systemic lupus erythematosus

A

31 years

40
Q

what are common findings of systemic lupus erythematosus

A
  1. fever
  2. weight loss
  3. arthritis
  4. fatigue
  5. general malaise
41
Q

what percent of systemic lupus erythematosus has arthritis in multiple joints (polyarthritis)

A

95%

42
Q

what percentage of systemic lupus erythematosus has butterfly rash

A

40-50%

43
Q

does sunlight make systemic lupus erythematosis better?

A

NO! makes it worse

44
Q

kidneys are affected in ___% of patients in systemic lupus erythematosus

A

40-50%

45
Q

what is the most significant aspect of systemic lupus erythematosus

A

kidney failure

46
Q

is cardiac involvement common in systemic lupus? what percent?

A

common - 50% display vegetation in heart valves

47
Q

what is the type of cardiac involvement in systemic lupus erythematosus

A

Libman-Sacks endocartitis (vegetation affects heart valves) - antigen complexes coagulate in ehart

48
Q

can some patients w/ systemic lupus erythematosus develop superimposed bacterial endocartitis?

A

YES

49
Q

what percentage of patients with systemic lupus erythematosus has oral lesions? where?

A

5-25% of patients in palate, buccal mucosa, and gingiva

50
Q

what type of oral lesions found in systemic lupus erythematosus

A

lichenoid or granular or nonspecific

51
Q

what lupus has the involvement of the vermillion border of the lower lip

A

lupus cheilitis

52
Q

T/F: varying degrees of ulcerations, pain, erythema, and hyperkeratosis may be seen in systemic lupus erythematosis oral lesions

A

TRUE

53
Q

what disease:

  • pt has few or no symptoms
  • lesions limited to skin or mucosal surfaces
A

chronic cutaneous LE

54
Q

how does chronic discoid LE look

A

begin as scaly, erythematous patches often distributed on sun exposed skin

55
Q

chronic discoid LE healing process has what?

A

scarring and hypo or hyperpigementation

56
Q

chronic LE oral lesions are identical to what?

A

lesions of erosive lichen planus

57
Q

unlike oral lesions of lichen planus, oral lesions of CCLE seldom occur in absense of ___

A

skin lesions

58
Q

what has an ulcer in center and white radiating lines on side

A

lichenoid

59
Q

what characterizes oral lesions of CCLE

A

ulterated or atrophic, erythematous central zone, surrounded by find white radiating lines

60
Q

lesions in what disease may be painful when exposed to spicy or acidic food

A

CCLE

61
Q

what disease:
- clinical manifestation between SLE and CCLE
- skin lesions are most prominent feature
- present on sun exposed skin
- lesions do not show scarring

A

SCLE

62
Q

what disease:
direct immunofluorescent testing of lesional tissue shows deposition of immunoreactions in a shaggy or granular band at the basement of membrane zone

A

SLE

63
Q

what test for SLE

A

direct immunofluorescent testing

64
Q

what Ig to look for on positive lupus band test

A

IgG, IgM, or C3

65
Q

95% of SLE patients have antibodies against what

A

anti-nuclear antigens (ANAs)
(has nonspecific findins since seen in other autoimmune disease)

66
Q

70% of SLE patients have antibodies against what?

A

double stranded DNA

67
Q

30% of SLE patients have antibodies against what?

A

Sm - protein that is complexed w/ small nuclear RNA

68
Q

what antibodies are very specific against SLE

A

antibodies against Sm

69
Q

90% of SLE pt have ___ years to live, 60% are alive at __ years

A

5, 20

70
Q

T/F: prognosis of SLE depends on which organs are affected and how frequently the disease is reactivated

A

TRUE

71
Q

SLE treatment

A
  • avoid excess sun exposure
  • mild active disease: NSAIDs and antimalarial drugs
  • severe acute episodes: systemic corticosteroids, other immunosuppresants and immunomodulators
72
Q

CCLC/SCLE tx

A
  • topical corticosteroids
  • if no relief, systemic drugs like SLE