Autoimmunity and immunodeficiency Flashcards

1
Q

what is autoimmunity

A

when the own body’s components are seen as a pathogen

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2
Q

what is immunodeficiency

A

a suppression of the immune system, such as medically induced.
there are situation when the immune system needs to be damped down e.g. when transplant patient is put on powerful immuno suppressants/drugs, or can be born with immunodeficiency or can acquire it

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3
Q

when can a person have immunodeficiency

A

when the immune system needs to be damped down e.g. when transplant patient is put on powerful immuno suppressants/drugs, or can be born with immunodeficiency or can acquire it e.g. via AIDS

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4
Q

what is the difference between the antigens in hypersensitivity and autoimmunity

A
  • hypersensitivity - the antigens which the immune system respond to are extraneous, e.g. pollen grains causes a typical allergic fashion
  • autoimmunity - its our own body’s components which are seen as foreign and antigenic, so the immune system is responding against itself
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5
Q

what does the bodies self tolerance mechanism distinguish and what for

A

between self and non-self, to avoid autoimmunity

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6
Q

what does the self tolerance mechanism by the body breakdown with

A

the production of autoantibodies and auto reactive T-cells

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7
Q

what do autoimmune mechanisms target

A

a variety or organs and tissues and underlie many diseases

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8
Q

what two types of autoimmune diseases are there

A
  • organ specific
    or
  • systemic (effects whole body)
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9
Q

where to T cells from the bone marrow go to

A

the thymus

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10
Q

what do T cells become in the thymus

A

matured into fully functional T cells and acquire their T cell receptor which is either a CD4 or CD8 receptor which determines whether they are T helper cells or T cytotoxic cells

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11
Q

how does the process in the thymus = self tolerance

A

the thymus shows the immature T cells e.g. MHC proteins in our body’s tissues to lymphocytes, & any lymphocyte that responds to them is immediately eradicated

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12
Q

what are many autoimmune diseases associated with

A

anterior & posterior uveitis

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13
Q

name some autoimmune diseases which are more prevalent in women

A
  • Graves disease (hyperthyroid)
  • Rheumatoid arthritis
  • Hashimoto’s thyroiditis (hypothyroid)
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14
Q

which type of diabetes is an autoimmune disease

A

type 1

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15
Q

which type of thyroid is not an autoimmune disease

A

type 2

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16
Q

which types of hypersensitivity mechanisms are involved with autoimmune diseases

A

types, 2, 3 & 4

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17
Q

which types of hypersensitivity mechanisms are involved in organ-specific

A

types, 2 & 4

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18
Q

which type of hypersensitivity mechanism is involved in non organ-specific autoimmune disorders

A

SLE type 3 reactions (deposition in immune complex mediated) predominate

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19
Q

what are the three ways in which diseases cause tissue destruction

A
  • complement activation by autoantibodies
  • autoantibody recognition of self antigens
  • auto reactive T cell recognition of self antigens
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20
Q

how does a disease cause tissue destruction via complement activation by autoantibodies

A

cells are targeted by autoantibodies
the antibody which is binding to some cell surface proteins & in doing so, activates the complement pathway which can cause cell lysis/breakdown
they are there to protect us as they breakdown bacterial cell walls, but can also damage tissues
the complement system attracts phagocytes to the inflammatory region

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21
Q

how does a disease cause tissue destruction via autoantibody recognition of self antigens

A

formation of immune complexes = cell antigen combining with the antibody & when present in excess, they deposit in cell tissues or blood vessel walls, lungs, kidneys etc

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22
Q

how does a disease cause tissue destruction via auto reactive T cell recognition of self antigens

A

cell mediated reactions
T cells are involved with surveillance e.g. looking for tumour cells & virally infected cells and are able to destroy these cells, but in the case of autoimmunity, they can damage our own cells

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23
Q

which factors play a role in the development of autoimmunity

A

genetic

autoimmune diseases tend to be associated with particular HLA specificies (i.e. inherit specific HLA/MHC proteins)

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24
Q

what is the major histocompatibility complex (MHC)

A

a gene locus that codes for histocompatibility antigens

present on every cell with a nucleus so not on red blood cells

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25
Q

what are histocompatibility antigens

A

cell surface glycoproteins that play important roles in interactions amongst immune cells

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26
Q

where are class 1 gene products found

A

on all nucleated cells

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27
Q

what do class 1 gene products participate in

A

antigen presentation to cytotoxic T cells (CD8+ve)

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28
Q

what are class 2 products found on

A

antigen presenting cells e.g. macrophages, have T 2 MHC proteins

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29
Q

what do class 2 products participate in

A

antigen presentation to T helper cells (CD4+ve)

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30
Q

what is the MHC locus designated in humans

A

in the human leukocyte antigen (HLA) locus

but is found on lots of cells, not just on leukocytes, its known as HLA as it first first studied on white blood cells

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31
Q

where is HLA locus found

A

on the short arm of chromosome 6

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32
Q

what does the class 1 region of HLA consist of

A
HLA
A
B 
and C loci/proteins 
with subdivisions within those
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33
Q

what does the class 2 region of HLA consist of

A

divided into HLA-DP
DQ
and DR sub regions
they are particular types of protein and very variable, e.g. they must match HLA proteins with a donor & a host for e.g. an organ transplant)

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34
Q

what type of structure is HLA antigens

A

highly polymorphic with multiple allelic forms which differ from one person to another
eg HLA DR2, HLA DR4, HLA B27

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35
Q

what are HLA antigens important for

A

the ability of the T cells to differentiate between self and non-self (HLA antigens not possessed by an individual are seen as foreign)

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36
Q

what do class 1 proteins interact with and which in particular

A

interact with T cell receptors, particularly cytotoxic T helper cell receptors as they are proteins which show antigens to the immune system

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37
Q

what does the pocket of a class 1 protein hold

A

the antigen & shows it to the immune system i.e. immune surveillance

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38
Q

what do class 2 protein pockets hold on to

A

only to APC receptors

39
Q

what usually produces a single antigen receptor out of billions possible

A

each lymphocyte

40
Q

what are eliminated in the thymus

A

for developing T cells, the majority of self-reactive cells

41
Q

in the thymus, what are self peptides and MHC molecules displayed on

A

the surface of cortical thymic epithelial cells, and TCRs that bind to these ligands are eliminated

42
Q

what still occurs despite the fact that TCRs which bind to the ligands of the surface of cortical thymus epithelial cells are eliminated

A

a significant fraction of the receptors of mature T cells and B cells bind to one or more self components in the body

43
Q

in autoimmunity, what do T cells recognise their own cells as foreign through

A

MHC proteins

44
Q

what are the specificity on the T cells used to determine

A

if the cell is foreign or whether it belongs to our cells

45
Q

name and describe the three strategies used to deal with self reactive lymphocytes (when the process in the thymus is not perfect and allows some reactive T cells to go through)

A
  • a cell displaying a self-reactive receptor can be triggered to die by apoptosis
  • a self reactive receptor can be edited to display a different receptor that is not self reactive (i.e. can remodel T cell receptors & no longer reactive against self antigen)
  • intrinsic or extrinsic regulation can reduce the ability of the cell being activated by self reactive receptors (suppress themselves so they’re less likely to become activated)
46
Q

name 3 examples of autoimmune diseases (breakdown of self tolerance)

A
  • graves disease
  • rheumatoid arthritis
  • SLE - systemic lupus arithmetosis
47
Q

what does the development of lesions in rheumatoid arthritis involve

A

both cell mediated and antibody responses

48
Q

what are present in the synovium of patients diagnosed with rheumatoid arthritis

A

CD4+ve T cells
activated B lymphocytes
plasma cells

49
Q

what leads to inflamed synovium and formation of pannus (inflammatory granulation tissue) in rheumatoid arthritis

A

cytokine secretion by activated T cells

50
Q

what is the problem with in rheumatoid arthritis

A

the joint capsule where a normal joint has cartilage and one with rheumatoid arthritis has not
&
inflammation within the sinovium = deformation of granulous tissue

51
Q

what is graves disease a common cause of

A

hyperthyroidism (over production of thread hormone)

52
Q

what is graves disease associated with

A
  • enlargement of thyroid (goitre)
  • exophthalmos (ocular manifestation)
  • heat intolerance (cannot regulate body temp)
  • anxiety
53
Q

what are the hyperthyroidism and goitre of graves disease caused by

A

stimulation of the thyroid by thyroid stimulating hormone (TSH) receptor antibodies

54
Q

where does production of thyroid stimulating hormone receptor TSH antibodies occur in graves disease

A

within the thyroid gland itself

55
Q

in graves disease, which receptors do the TSH bind to

A

receptors of the follicular cells on the thyroid

56
Q

in graves disease, what do the TSH which bind to receptors of the follicular cells on the thyroid stimulate

A

thyroid cells to produce two types of thyroid hormone

T3 & T4

57
Q

in graves disease, what binds to TSH receptors and produces excess T3 & T4 thyroid hormone

A

autoantibodies

58
Q

in graves disease, what is the proposed hypothesis for the pathogenesis of ophthalmopathy

A

that an immune response to a TSH receptor - like protein in orbital connective tissue

59
Q

what happens to the extra ocular muscles in graves disease and what does it result in

A

they become enlarged and theres an increase in orbital fat
all this takes up space as bone is on either side it has to expand forward
eye don’t close which causes dry eye

60
Q

what does the forward expansion of the EOM’s in graves disease cause to the ONH

A

the forward expansion pulls on the slack of the optic nerve and the ONH gets stretched and loses vision

61
Q

what does autoimmunity and the eye tend to be associated with

A

particular HLA antigens/organ specific e.g. HLA B27

62
Q

what can ocular autoimmunity occur with the presence or absence of

A

systemic autoimmune disease

63
Q

what are the ocular autoimmune diseases in the absence of systemic involvement include

A
  • moorens ulcer
  • sympathetic ophthalmia
    both only exclusive to the eye
64
Q

what is moorens ulcer

A

a degenerative condition of the cornea
thinning of the cornea around the arc near the limbus
shows a deep ridge near the limbus and extremely thin cornea = sight threatening

65
Q

what is moorens ulcer treated with

A

immunosuppression (damped down)

66
Q

what is sympathetic ophthalmia

A

can occur following trauma to the eye weeks or months later can develop severe inflammation to the other eye which was not effected in the trauma and can lose sight

67
Q

list the ocular autoimmune diseases with systemic involvement, i.e. which can cause anterior or posterior uveitis or both

A
  • reiter’s disease
  • ankylosing spondylitis
  • rheumatoid arthritis
  • behcet’s disease
  • SLE
  • sjogren’s syndrome
  • sarcoidosis
  • cicatricial pemphigoid
68
Q

what 4 things can primary immunodeficiency effect and what can these lead to

A
  • B cell deficiencies lead to recurrent bacterial infections
  • T cell deficiencies lead to recurrent viral, fungal and protozoal infections
  • phagocytic deficiency leads to recurrent bacterial infections
  • complement deficiency leads to recurrent bacterial infections and defects in immune complex clearance
69
Q

what was AIDS which was first described in the early 1980’s as a disease effecting

A
  • homosexual men
    &
  • iv drug users (injecting drugs into the vein)
70
Q

what are previously healthy AIDS sufferers presented with

A

opportunistic infections such as:
- pneumocystis carinii pneumonia
&
- toxoplasmosis

71
Q

what is pneumocystis carinii pneumonia caused by from AIDS sufferers

A

inhaling fungal spores

72
Q

which virus is AIDS caused by

A

HIV-1 infection

73
Q

what is HIV and what does it infect specifically

A

retrovirus
infects T-helper (CD4+ve) lymphocytes (by binding to receptor of T helper cell & depletes it) which causes profound immunodeficiency

74
Q

what do T helper cells produce and what does it regulate

A

produce cytokines

regulates immune mechanisms

75
Q

in HIV what is there initially a fall in and what does this cause

A

fall in T helper cells causes a primary infection, but soon recover

76
Q

in the first few weeks of HIV/AIDS, whilst the T helper cells have fallen, what has risen and what does this cause

A

rise of HIV RNA, causes acute HIV syndrome wide dissemination of virus seeding of lymphoid organs, our immune system then kicks in following this

77
Q

what happens during the clinical latency period of HIV/AIDS (weeks-years)

A

person doesn’t experience symptoms when the T helper cells are in excess over the virus/HIV RNA, many only experience flu like symptoms

78
Q

what happens 8-9 years down the line during HIV/AIDS

A

T helper cells decline and opportunistic infections arise as the HIV RNA increases followed by death

79
Q

list some infections/diseases caused by AIDS

A
  • pneumonia - lungs congested
  • sight threatening retinitis - virus from herpes
  • skin cancer - kaposi’s sarcoma
  • candita infection - thrush of oral mucosa
  • raised lesions around the eye - molluscum loads (sign of an asymptomatic AIDS sufferer)
80
Q

what type of tissue transplant is well tolerated

A

when tissue is transplanted from an individual to another location within the same individual - autografts

81
Q

what type of tissue transplant has high success rates

A

organs transplanted between identical twins

82
Q

when are grafts transplanted between genetically different individuals (allografts) tolerated

A

only when MHC antigens are matched

83
Q

what type of testing attempts to match alleles between an donor and recipient during transplants

A

histocompatibility testing of class 1 and class 2 antigens

84
Q

when can other cell surface markers still possibly induce rejection for transplants

A

even with a high degree or HLA similarity

85
Q

what are transplant patients routinely prescribed

A

immunosuppressant drugs e.g. cyclotron, tacrolimus, corticosteroids

86
Q

how can the blood system cause a mechanism of transplant rejection

A

blood system is essential to maintain host tissue but via the blood supply is where immune cells are bought into the vicinity so T cells which belong to the donor which are put into the body with the transplant (recipient) can attack the host tissue (called a graft vs host disease)

87
Q

what type of transplant disease is more common and needs to be controlled, than the graft vs host disease

A

when the host immune system is attacking the donor tissue

88
Q

what is the area where the blood is between the host and donor tissue called

A

interphase

89
Q

when is a corneal transplantation required

A

in cases of corneal opacification

90
Q

why do corneal transplants have high success rates

A

due to avascularity of cornea and lack of immune activation

91
Q

when is the success rate reduced for a corneal transplant

A

in high risk patients e.g. neovascularisation
where immune system responds to attack foreign (donor) antigens, T cells and macrophages infiltrate the tissue and secrete a variety of cytokines and corneal graft rejection can occur

92
Q

what two strategies are used in high risk corneal grafts

A
  • HLA matching of donor and recipient

- immunosuppressive therapy

93
Q

when can a corneal transplant cause astigmatism

A

when the suture is too tight, it can flatten the cornea in one meridian

94
Q

what is used to control the immune system in high risk corneal grafts

A

steroid eyedrops