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EMS: MoD > Lecture 3- immunodeficiency and autoimmunity > Flashcards

Lecture 3- immunodeficiency and autoimmunity Flashcards

1
Q

what are the general principles of the immune system?

A

-multi-layer defense
-network of pathogen recognition
effective inter-cellular communication
-many mechanisms for pathogen clearance
-adaptive response to changing pathogens
-self-regulation
-limitation of host damage

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2
Q

self-regulation of immune system

A

important in terms of causing disease. when self-regulation is not correct, disease results

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3
Q

innate immune system

A

neutrophils, macrophages produce simple chemicals that kill bacteria

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4
Q

adaptive immune system

A

more precise and potent but this needs regulation

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5
Q

what are PRR?

A

pattern recognition receptor. they recognise components of the microorganism

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6
Q

what are the different barrier components of the immune system

A

SKIN
tears
saliver

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7
Q

name the different mechanisms of the immune system

A
  • barrier and chemical mechanism
  • PRR
  • cellular-such as phagocytes/natural killer cells
  • humoral
  • cellular
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8
Q

where are the cells of immune system produced?

A

bone marrow from precursors

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9
Q

which precursors do innate immune cells come from

A

myeloid precursors

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10
Q

where do dendritic cells, macrophages, and neutrophils arise from?

A

common stem cell

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11
Q

what do lymphoid precursors divide into?

A

B and T cells

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12
Q

where do B cells mature?

A

initially mature in the bone marrow

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13
Q

where do T cells mature?

A

mature in the thymus.

when released into the periphery, it matures further into specialised TH1 and TH2 cells

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14
Q

autoimmune disease

A

range of conditions where own immune system attacks target or systemic autoimmunity.

has distinct clinical entities

breakdown of self-tolerance

environmental factors acting on favourable genetic background

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15
Q

requirements for autoimmune disease to develop

A

genetic predisposition
environment
immune regulation

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16
Q

what is HLA

A

human leukocyte antigen

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17
Q

where are HLA molecules found?

A

found at the surface of antigen presenting cells and can attach to variety of proteins

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18
Q

what causes rheumatoid arthritis?

A

shared epitopes, several HLA alleles that can present certain proteins in ways to cause the disease.

these are proteins that can be citronated and cause disease chain reaction

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19
Q

what are the risk factors of rheumatoid arthritis?

A

smoking –> cause citronisation more rapidly, leading to damaged proteins and initiate autoimmune response

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20
Q

what is HLA-DRB1?

A

The HLA-DRB1 gene provides instructions for making a protein that plays a critical role in the immune system. The HLA-DRB1 gene is part of a family of genes called the human leukocyte antigen (HLA) complex.

The protein produced from the HLA-DRB1 gene, called the beta chain, attaches (binds) to another protein called the alpha chain, which is produced from the HLA-DRA gene. Together, they form a functional protein complex called the HLA-DR antigen-binding heterodimer. This complex displays foreign peptides to the immune system to trigger the body’s immune response

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21
Q

which HLA-DRB1 is associated with tye 1 diabetes?

A

DR3,DR4

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22
Q

which HLA-DRB1 is associated with graves disease?

A

DR3

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23
Q

which HLA-DRB1 is associated with multiple sclerosis?

A

DR2

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24
Q

which HLA-DRB1 is associated with systemic lupus erythematosus (SLE)?

A

DR2,DR3

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25
Q

Rheumatoid arthritis

A

DR1, DR4

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26
Q

which genetic defect is linked with autoimmune polyendocrine sydrome type 1?

A

AIRE

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27
Q

what is the mode of inheritance for autoimmune polyendocrine syndrome type 1?

A

Autosomal recessive

28
Q

what is the mechanism of disease for autoimmune polyendocrine syndrome type 1?

A

adaptive immunity affected

defective thymic expression of self antigen

29
Q

which genetic defect is linked with immune dysregulation, polyendocrinopathy, enteropathy

A

FOXP3

30
Q

which genetic defect is linked with type 1 diabetes?

A

insulin gene

31
Q

which genetic defect is linked with type 1 diabetes, rheumatoid arthritis, autoimmune thyroid disease?

A

PTPN22

32
Q

which genetic defect is linked with type 1 diabetes, autoimmune thyroid disease, rheumatoid arthritis

A

CTLA4

33
Q

which genetic defect is linked with SLE, type 1 diabetes, rheumatoid arthritis?

A

PD1

34
Q

which genetic defect is linked with autoimmune polyendocrine syndrome type 2?

A

unknown

35
Q

which genetic defect is linked with SLE?

A

IRF5

complement deficiencies C1Q, C2, C4

36
Q

what is the mode of inheritance for diseases caused by FOXP3 gene?

A

X-linked recessive

37
Q

what is the mechanism of disease for diseases caused by FOXP3 gene?

A
  • adaptive immunity affected

- defective production of regulatory T cells

38
Q

what is the mechanism of disease for type 1 diabetes caused by deficiency in insulin gene?

A
  • adaptive immunity affected
  • reduced thymic expression of insulin
  • reduced negative selection of T cells
39
Q

what is the mechanism of disease for diseases caused by deficiency in PTPN22 gene?

A
  • adaptive immunity affected
  • reduced thymocyte responsiveness to negative selection
  • reduced regulatory T cell function
40
Q

what is the mechanism of disease for diseases caused by deficiency in CTLA4?

A
  • adaptive immunity affected
  • reduced regulatory T cell function
  • inadequade negative signalling in effector T cells
41
Q

what is the mechanism of disease for diseases caused by deficiency in PD1?

A
  • adaptive immunity affected

- inadequate negative signalling in effector T cells

42
Q

what is the mechanism of disease for autoimmune polyendocrine syndrome type 2

A
  • adaptive and innate immunity affected

- reduced regulatory T cells function

43
Q

what is the mechanism of disease for SLE caused by deficiency in IRF5 gene?

A
  • innate immunity affected

- altered signalling thresholds in TLR(toll-like-receptor) pathway

44
Q

what is the mechanism of disease for SLE caused by deficiency in complement C1Q, C2, C4?

A
  • adaptive and innate immunity affected
  • altered pathogen recognition
  • altered effector functions (immune complex clearance)
45
Q

what happens if the thymus doesnt have AIR gene?

A

it cannot remove potentially self-reactive T cells

46
Q

what are FOXP3 genes important in?

A

development of T regulatory cells, which is produced centrally in thymus

47
Q

what are other causative associations of autoimmune disease?

A
  • sex –> hormonal influence. woman&raquo_space; men
  • age –> more common in elderly
  • environmental triggers –> infection, trauma-tissue damage, smoking
48
Q

autoreactive B cells and autoantibodies are…

A
  • directly cytotoxic
  • activation of complement
  • interfere with normal physiological function
49
Q

autoreactive T cells are…

A
  • directly cytotoxic

- inflammatory cytokine production

50
Q

what is organ specific autoimmune disease?

A
  • affect a single organ
  • autoimmunity restricted to autoantigens if that organ
  • overlap with other organ specific diseases
  • autoimmune thyroid diseases is typical
51
Q

what is systemic autoimmune diseases?

A
  • affect several organs simultaneously
  • autoimmunity associated with autoantigens found in most cells body
  • overlap with other non-organ specific diseases
  • connective tissue diseases are typical
52
Q

what is hashimotos thyroiditis?

A
  • destruction of thyroid follicles by autoimmune process
  • associated with auto-antibodies to thyroglobulin and thyriod peroxidase
  • leads to hypothyrodism
53
Q

what is Grave’s disease?

A

inappropriate stimulation of thyroid gland by anti-TSH-auto-antibody
-leads to hyperthyrodism

54
Q

list connective tissue autoimmune diseases

A
  • SLE
  • scleroderma
  • polymyositis
  • sjogrens syndrome
55
Q

name a non-specific diagnostic test used for auto-immune disease

A

inflammatory markers

56
Q

name a disease specific diagnostic test used for auto-immune disease

A
  • auto-antibody testing

- HLA typing

57
Q

why measure autoantibdies

A
  • diagnostic
  • early diagnosis
  • pathogenic
  • subtyping of patients
  • mointoring of exacerbation/remission
  • exclusion of diagnosis
  • cost of treatment
58
Q

what is immunosupression?

A

a natural or artificial process which turns off the immune respnse, partially or fully, accidentally or on purpose
-can result in immunodeficiency

59
Q

what is immunodeficiency?

A

the lack of an efficient immune system-susceptibility to infections

60
Q

what are the causes of immunodeficiency?

A
  • infection –> the result of transient immunodeficiency
    -secondary to effects of external factors
    -primary immunodeficiency –> caused by genetic defects in individual components of immune system
    -
61
Q

what the causes of secondary or acquired immunodeficiencies?

A
  • stress
  • surgery/burns
  • malnutrition
  • cancer –> esp. lymphoproliferative disease
  • immunosupressive effect of drugs inc. cancer therapy
  • irradiation (clinical or other)
  • AIDS
  • other infection e.g. measles, TB
62
Q

when are primary immunodeficiency often diagnosed?

A

childhood - recurrent infection often suggests immunological problem

63
Q

what does SCID stand for?

A

severe combined immunodeficiency syndrome

64
Q

what happens if there is a defect in both B and T cells?

A
  • SCID
  • combined immunodeficiency syndrome
  • bone marrow transplantation curative
  • gene therapy
65
Q

what are symptoms of defects in T cells?

A

recurrent infection with opportunistic infection, bacteria, viruses, fungi (candida), protozoa (pneimocystis)

66
Q

why are defects in T cells more severe than defects in just B cells?

A

because T cells are needed for normal functioning of B cells

EMS: MoD (3 decks)

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