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Respiratory > Autoimmunity > Flashcards

Autoimmunity Flashcards

1
Q

what is autoimmunity

A

the presence of immune responses against self tissues/cells

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2
Q

when does auto immunity become dangerous

A

when there is HIGH titres of auto-antibodies or auto-reactive T cells

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3
Q

what can high titres of auto-antibodies or auto-reactive T cells cause

A

significant tissue/organ damage, chronic inflammation

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4
Q

what is the initiating event of autoimmune diseases

A

exposure to noxious substance or environmental factor

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5
Q

what determines whether a autoimmune reaction will be driven

A

genetic susceptibility- build up of many susceptible genes

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6
Q

what occurs after the initiating event

A

breakdown of self tolerance- loss of immune regulation- generation of auto-reactive B and T cells

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7
Q

what does the breakdown of self tolerance lead to

A

autoimmune phenomena/disease

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8
Q

what is a monogenic disorder

A

single gene defect causing autoimmune diseases that are rare

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9
Q

what is IPEX syndrome

A

a rare monogenic disorder of immune disregulation

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10
Q

what genes are commonly involved in the complex genetic interplay that results in most autoimmune diseases

A

HLA genes, sex genes, immune response genes

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11
Q

how does ipex syndrome present

A

overwhelming systemic autoimmunity in early childhood causing a wide range of symptoms

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12
Q

a mutation in what gene leads to IPEX syndrome and why

A

FOXP3, as essential for the development of regulatory T-cells

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13
Q

why are only males affected by IPEX syndrome

A

as it is X linked

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14
Q

what are T follicular helper cells

A

antigen experienced CD4+ T cells that produce interleukins that lead to B cell co stimulation

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15
Q

what is the role of T regulator cells

A

secrete anti inflammatory cytokines that can act on all of the other T and B cells supressing their activity (ymphocytes)

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16
Q

what does loss of balance of T Reg cells lead to

A

loss of control of T cell activation- uninhibited; loss of control of adaptive immune response = autoimmune disease

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17
Q

why do healthy T reg cells prevent autoimmune disease

A

as suppress hyperactive T cells

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18
Q

how do defective T Reg cells affect peripheral tolerance

A

leads to failure of peripheral tolerance= IPEX

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19
Q

what are tolerance mechanisms

A

control populations of T and B cells

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20
Q

describe central tolerance

A

deletion of self-reactive lymphocytes in primary lymphoid tissues

21
Q

describe peripheral tolerance

A

inactivation of self-reactive lymphocytes in peripheral tissues that escape central tolerance

22
Q

why must self reactive lymphocytes be deleted

A

as healthy T and B cells don’t recognise self antigen

23
Q

what is HLA

A

human leukocyte antigen- gene complex coding for MHC proteins in humans

24
Q

how can each individual express 12 MHC genes

A

2 variants of each HLA molecule type= 2 copies of chromosome 6 from parents

25
Q

why is different types of MHC molecules a good thing

A

as each better at presenting different peptides- able to present T cells with a wider range of peptides- polymorphisms makes all these genes differnt

26
Q

where do all nucleated cells express HLA class 1

A

cell surface

27
Q

how can sex differences affect autoimmune disease

A

sex bias in predisposing genetic factors, hormonal influences in lymphocyte function, pregnancy

28
Q

what is molecular mimicry

A

when pathogenic peptides mimic self antigens triggering a immune response targeting self antigens

29
Q

what environmental factors can contribute to autoimmune disease

A

infection- molecular mimicry

30
Q

give an example of molecular mimicry

A

acute rheumatic fever after streptococcal infection; strep. M5 protein v similar to cardiac tissue proteins, drives hypersensitivity reaction which targets self antigens

31
Q

what type of hypersensitivity reaction is acute rheumatic fever after streptococcal infection

A

type 2 as dealing with immune system vs self antigens bound to surface of cell

32
Q

what is reactive arthritis and what can it follow

A

sterile inflammation secondary to bacterial infection

33
Q

what is antigen sequestration

A

sequestered antigens are ones that cannot interact with the immune system during development so exposure to them due to damage (e.g to blood brain barrier, testes or eye) could drive autoimmune response

34
Q

what do super antigens cause

A

toxic shock syndrome

35
Q

what are two examples of super antigens

A

staphylococcal proteins, clostridium endotoxin

36
Q

why is immune regulation important in autoimmunity

A

as maintain self tolerance

37
Q

what is the clinical classification of autoimmune disease

A

organ-specific or non-organ specific/multi system diseases

38
Q

what is the pathological classification of autoimmune disease

A

gel and coombs classification (hypersensitivity reactions)

39
Q

what type of hypersensitivity reaction is not applicable to autoimmune conditions

A

type 1- immediate hypersensitivity (allergy)

40
Q

describe the mechanism of graves disease

A

mediated by type 2 mechanisms-

antibodies bind to TSH receptor (thyroid stimulating hormone)

41
Q

what is graves disease the leading cause of

A

hyperthyroidism

42
Q

describe goodpasture’s syndrome

A

mediated by type 2 mechanisms- antibodies bind to glomerular basement membrane in lungs and kidneys leading to bleeding from the lungs and kidney failure

43
Q

how are type 2 hypersensitivity reactions managed

A

plasmapheresis (removal of pathogenic antibody) immunosupression (switch off B cell production of antibody)

44
Q

give two example of type 2 mediated autoimmune diseases (bound anitgen)

A

graves disease and good pastures syndrome

45
Q

what is an example of an autoimmune disease mediated type 3 mechanisms

A

systemic lupus erythematosus (SLE)- auto-antibodies against nuclear antigens

46
Q

how are type 3 hypersensitivity reactions managed

A

control symptoms- limit sun exposure, analgesia, hydroxychloroquinine, decrease inflammation and production of auto-antibodies

47
Q

give three examples of type 4 hypersensitivity mediated autoimmune diseases (delayed hypersensitivity)

A

type 1 diabetes, coeliac disease, Rh arthritis

48
Q

what does an overwhelming og clearing mechanism mean

A

oxygen will not be cleared

Respiratory (26 decks)

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