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Respiratory > Immunology > Flashcards

Immunology Flashcards

1
Q

What are the 4 hallmarks of immune deficiency?

A 
SPUR
Serious infections
Persistent infections
Unusual infections
Recurrent infections
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2
Q

what defines a serious infection?

A

unresponsive to oral antibiotics

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3
Q

what defines persistent infections?

A

early structural damage

chronic infections

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4
Q

what defines unusual infections?

A

unusual organisms

unusual sites

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5
Q

what defines recurrent infections?

A

two major or one major + recurrent minor infections in one year

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6
Q

what 6 features apart from SPURS may be surggestive of primary immune deficiency?

A 
weight loss/ failure to thrive
severe skin rash
chronic diarrhoea
mouth ulceration
unusual autoimmune disease
family history
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7
Q

what 2 important infections lead to secondary immune deficiency?

A

HIV

measels

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8
Q

what 3 important treatment interventions lead to secondary immune deficiency?

A

immunosuppressive therapy
anti-cancer agents
corticosteriods

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9
Q

what type of malignancies can lead to secondary immune deficiency?

A

cancer of the immune system (lymphoma, myeloma, leukemia)

metastatic tumours

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10
Q

what biochemical/nutritional disorders can lead to secondary immune deficiency?

A

malnutrition
diabetes (Type 1 and 2)
dialysis

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11
Q

what is a major feature of phagocyte deficiencies?

A

recurrent infections

which can affect either common or unusual sites

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12
Q

what are the types of organisms typically infect patients with phagocyte deficiencies?

A

common bacteria (eg Staph sureus)
unusual bacteria (eg Burkholderia cepacia)
Mycobateria (TB and atypicals)
Fungi (candida, aspergillus)

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13
Q

what is the general life cycle of a phagocyte? (ie neutrophil

A
  1. mobilisation of phagocytes and precursos from bone marrow or within tissues
  2. upregulation of endothelial adhesion markers causing neutrophil adhesion and migration into tissues
  3. phagocytosis and killing of organism
  4. activation of other components of the immune system
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14
Q

what are the 2 reasons that can cause the failure of neutrophil production?
(and therefore neutropaenia and phacogyte deficiency)

A

failure of stem cells to differentiate along myeloid lineage

specific failure of neutrophil maturation

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15
Q

what is reticular dysgensis?

A

the failure of stem cells to differentiate along myeloid and lymphoid lineages. megakaryocyte-erythroid lineage is unaffected. most severe form of severe combined immunodeficiency (SCID)

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16
Q

when can the secondary defect which causes the failure of stem cells to differentiate along the myeloid lineage occur?

A

after stem cell transplantation

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17
Q

what are the 2 congenital causes of specific failure of neutrophil maturation? (and therefore cause neutropaenia)

A

Kostmanns syndrome

Cyclic neutropaenia

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18
Q

what is Kostmanns syndrome?

A

rare autosomal recessive disorder causing the specific failure of neutrophil maturation- leads to severe chronic neutropaenia

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19
Q

what is Cyclic Neutropaenia?

A

episodic neutropaenia every 4-6 weeks caused by the specific failure of neutrophil maturation

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20
Q

when do patients with Kostmanns syndrome usually present with recurrent infections?

A

within 2 weeks after birth

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21
Q

what is the supportive treatment of Kostmanns syndrome?

A

prophylactic antibiotics

prophylactic antifungals

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22
Q

what is the definitive treatment of Kostmanss syndrome?

A

stem cell transplantation

Granulocyte colony stimulating factor (G-CSF)

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23
Q

what is granulocyte colony stimulating factor (G-CSF)?

A

a growth factor which assists maturation of neutrophils

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24
Q

what rare, primary immunodeficiency is caused by the genetic defect in leukocyte integrins?

A

leukocyte adhesion deficiency

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25
Q

what does leukocyte adhesion deficiency result in?

A

failure of neutrophil adhesion and migration

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26
Q

what is the general clinical picture of leukocyte adhesion deficiency?

A

marked leukocytosis but with localised bacterial infections that are difficult to detect

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27
Q

what 2 receptors do phagocytes possess that bind to opsinons?

A 
Fc receptors (for antibody Fc)
Complement receptor 1 (CR1- for binding to complement which are bound to the antigen)
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28
Q

what 2 defect can cause decreased efficiency of opsonisation? (and therefore a phagocyte deficiency)

A

defect of complement/antibody production

defect in opsonin receptor

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29
Q

what is chronic granulomatous disease?

A

a phagocyte deficiency due to failure of oxidative killing mechanisms

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30
Q

in chronic granulomatous disease what causes the failure of oxidative killing mechanisms?

A

inability to generate oxygen free radicals and so impaired killing of intracellular organisms

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31
Q

why are granulomas formed in chronic granulomatous disease?

A

inability to clear organisms leads to excessive inflammation and persistent accumulation of neutrophils, activated macrophages and lymphocytes. all leading to a granuloma

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32
Q

in chronic granulomatous disease what features would you find on an full abdominal exam?

A

lymphadenopathy

hepatosplenomegaly

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33
Q

how do you investigate chronic granulomatous disease?

A

NBT test

nitroblue tetrazolium

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34
Q

how does a NBT test work?

A

you feed patients neutrophils a source of E-coli
add a dye sensitive to H202
if hydrogen peroxide is produced by neutrophils (meaning oxidative killing is function) the dye will change colour showing patient doesn’t have chronic granulomatous disease

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35
Q

what is the supportive treatment for chronic granulomatous disease?

A

prophylactic antibiotics

prophylactic antifungals

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36
Q

what type of cells do mycobacteria reside within?

A

macrophages

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37
Q

what network is activated when a macrophage becomes infected with mycobacteria?

A

IL12-gIFN network

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38
Q

what are the 6 steps in the IL12-gIFN network?

A
  1. infected macrophages stimulated to produce IL 12
  2. IL 12 induces T cells to secrete gIFN
  3. gIFN feeds back to macrophages and neutrophils
  4. stimulation of TNF within the infected macrophage
  5. activation of NADPH oxidase within the macrophage
  6. TNF and NADPH oxidase stimulate oxidative pathways
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39
Q

what is the definitive treatment for chronic granulomatous disease?

A

stem cell transplantation
(gene therapy)
gIFN therapy

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40
Q

what 3 single gene defects are associated with suscpetibility to intracellular bacteria such as mycobacteria infection and salmonella?

A

gIFN receptor deficiency
IL 12 deficiency
IL 12 receptor deficiency

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41
Q

what can happen when anti-TNF drugs are used in the treatment of inflammatory diseases?

A

reactivation of latent intracellular bacteria such as tuberculosis

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42
Q

compare congenital neutropaenia, leukocyte adhesion deficiency and chronic granulomatous disease with regards to neutrophil count?

A

conginital neutropaenia: absent
leukocyte adhesion defect: increased during infection
chronic granulomatous: normal

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43
Q

compare congenital neutropaenia, leukocyte adhesion deficiency and chronic granulomatous disease with regards to the ability to form pus?

A

congenital neutropaenia- no
leukocyte adhesion defect- no
chronic granulomatous- yes

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44
Q

compare congenital neutropaenia, leukocyte adhesion deficiency and chronic granulomatous disease with regards to leukocyte adhesion markers?

A

congenital neutropaenia- normal
leukocyte adhesion defect- abnormal
chronic granulomatous- normal

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45
Q

compare congenital neutropaenia, leukocyte adhesion deficiency and chronic granulomatous disease with regards to oxidative killing?

A

congenital neutropaenia- absent
leukocyte adhesion defect- normal
chronic granulomatous- absent

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46
Q

how is reticular dysgenesis treated?

A

fatal unless corrected with bone marrow transplantation

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47
Q

what is severe combined immunodeficiency?

A

failure of lymphocyte production (failure of lymphocyte precursors)

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48
Q

what are the 6 typical features of a child with severe combined immunodeficiency?

A 
unwell by 3 months of age
persistent diarrhoea
failure to thrive
infections of all types
unusual skin disease
family history of early infant death
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49
Q

what is the unusual skin disease usually present in a child with severe combined immunodeficiency?

A

graft versus host disease

caused by colonisation of the infants empty bone marrow by maternal lymphocytes

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50
Q

what is the period of time called where even normal babies get infections (3-4 months) if there immune system is slow to mature?

A

transient hypogammaglobulinaemia of infancy

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51
Q

how long does maternal IgG protect any neonate?

A

3 months

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52
Q

what is the most common form of severe combined immunodeficiency?

A

X-linked SCID

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53
Q

what is the mutation in X-linked SCID?

A

mutation of component of IL 2 receptor

(IL2 is very important for T cell development

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54
Q

what are the levels of T and B cells in a child with SCID?

A

very low or absent T cells

normal or increased B cells (but poor response)

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55
Q

why do children with SCID have poorly developed lymphoid tissue and thymus?

A

not being stimulated by T cells

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56
Q

what is the prophylactic treatment of children with SCID?

A

hospitalised
avoid infections (prophylactic antibiotics, fungals, no vaccines)
aggressive treatment of existing infections
antibody replacement- IV Ig

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57
Q

what is the definitive treatment options of a child with SCID?

A

Stem cell transplant from HLA identical sibling if possible

Stem cell transplant from other sibling or parent of from matched unrelated donor

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58
Q

What causes DiGeorges syndrome?

A

deletion of chromosome 22q11

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59
Q

what is the phenotype of a child with DiGeorge syndrome? (5 things)

A 
Congenital heart defects
Abnormal facial features
Thymic hypoplasia
Cleft palate
Hypocalcaemia (due to hypoparathyroidism)
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60
Q

why is there a T cell deficiency in DiGeorges syndrome?

A

failure of thymic deveopment (nowhere for the T cells to mature)

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61
Q

what are the B cell, T cell and NK cell count like in DiGeorges Syndrome?

A

absent or very low T cells
normal or increased B cells (but poor response)
Normal NK cells

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62
Q

what is the management of DiGeorge Syndrome?

A

correct metabolic/cardiac abnormalities
prophylactic antibodies
early and aggressive treatment of infection
Ig replacement

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63
Q

why does T cell function improve with age in patients with DiGeorge syndrome?

A

extrathymic maturation of T cells

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64
Q

what type of disease happens when there is failure of normal apoptosis?

A

autoimmune syndromes

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65
Q

what are the 3 first line investigations of T cell deficiencies?

A

total white cell count differential
serum immunoglobulins and protein electrophoresis
quantitation of lymphocye subpopulation

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66
Q

what second line test is essential in investigating a T cell deficiency?

A

HIV test

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67
Q

what is the cause of Bruton’s X linked hypogammaglobulinaemia?

A

failure to produce mature B cells

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68
Q

what are the B cell levels like in Bruton’s X-linked hypogammaglobulinaemia?

A

no circulating B cells
no plasma calls
(no circulating antibody after first 6 months)

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69
Q

what is the name of the genetic condition in which IgM B cells can’t differentiate into IgA cells?

A

selective IgA deficiency

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70
Q

what are the symptoms of IgA deficiency?

A

2/3 asymp

1/3 recurrent respiratory tract infections

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71
Q

what is the name of the genetic condition in which there is low IgG, IgA and IgE?

A

common variable immune deficiency

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72
Q

what is the name of the genetic condition in which there is a failure of T cell co-stimulation of B-lymphocytes?

A

X-linked hyper IgM syndrome

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73
Q

what are the 3 main features of B cell deficiencies?

A

recurrent infections
opportunistic infections
antibody-mediated autoimmune disease

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74
Q

what are the 1st line investigations if there is a suspected B cell deficiency?

A

total white cell count and differential
serum immunoglobulins
serum and urine protein electrophoresis

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75
Q

how do you manage a B cell deficiency?

A

aggressive treatment of infection
immunoglobulin replacement
stem cell transplantation

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76
Q

what is a hypersensitivity reaction?

A

immune response that results in bystander damage to the self

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77
Q

what are the 4 types of hypersensitivity reactions?

A

Type 1: immediate hypersensitivity
Type 2: direct cell killing
Type 3: immune complex mediated
Type 4: delayed type hypersensitivity

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78
Q

what type of hypersensitivity reaction is an allergic disease?

A

type 1: immediate hypersensitivity

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79
Q

what is causes an immediate hypersensitivity reaction (allergy)?

A

IgE-mediated antibody response to external antigen

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80
Q

explain the prevalence of allergies?

A

increasing prevalence

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81
Q

what 2 factors can account for the increased prevalence of allergic disease?

A

genetic

environmental (eg hygiene hypothesis)

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82
Q

explain the hygiene hypothesis?

A

over the years there has been a general decrease in infectious burden during early life, this resulted in an underdeveloped immune response which predisposes to allergic conditions

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83
Q

what type of helper T cells (Th) cells are involved in the allergic response?

A

Th2

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84
Q

how quickly does the allergic attack occur after exposure to the allergen? (type 1: immediate hypersensitivity)

A

very quickly (minutes to 1/2 hours after exposure)

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85
Q

how can thresholds for allergic reactions be lowered?

A

by co-factors eg exercise, alcohol, infection

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86
Q

what does it mean by ‘type 1 immediate hypersensitivities are stereotyped’?

A

the same reaction occurs every time trigger is present

ie always a rash on exposure

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87
Q

name 6 common allergens?

A 
dust mite
pollen/animal dander
foods
drugs
latex
bee + wasp venom
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88
Q

what are the main immune cells involved in the type 1 immediate hypersensitivity response?

A

B lymphocytes
T lymphocytes
mast cells

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89
Q

what are the 2 functions of B cells within the allergic response?

A 
recognise antigen (allergen)
produce antigen-specific IgE antibody
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90
Q

what is the function of T cells within the allergic response?

A

provide help for B lymphocytes to make IgE antibody

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91
Q

what is the function of mast cells within the allergic response?

A

Fc receptors are binded to Fc of allergen specific IgE, when allergen antigen binds to variable part of the antibody, mast cells become active and release vasoactive substances

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92
Q

where are mast cells located?

A

resident in tissues, especially at interface with external environment

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93
Q

what vasoactive substances secreted by a mast cell are already preformed?

A

histamine
tryptase
heparin

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94
Q

what vasoactive substances secreted by a mast cell are synthesised on demand and aren’t preformed?

A

leukotrienes
prostaglandins
cytokines (including IL4 and TNF)

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95
Q

what are the 4 functions of vasoactive substances produced by a mast cell in response to an allergen?

A

increased blood flow
contraction of smooth muscle
increased vascular permeability
increased secretions at mucosal surface

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96
Q

what are the main 2 functions of a mast cell?

A

defence against parasites

wound healing

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97
Q

mast cells express Fc receptors on their surface that correspond to the Fc of what type of antibody?

A

IgE

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98
Q

what happens the first type the body encounters the allergen?

A

no allergic response

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99
Q

why is there no allergic response on first encounter with allergen?

A

B cells produce antigen-specific IgE antibody and allergen is cleared

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100
Q

on first encounter with allergen, once allergen has been cleared what happens to the residual IgE antibodies?

A

they bind to circulating mast cells via Fc receptors

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101
Q

what happens on re-encounter with an allergen (antigen)?

A

allergen binds to the IgE which is coating mast cells and disrupts cell membrane causing a release of preformed and newly synthesised vasoactive mediators (degranulation)

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102
Q

what is the name for allergic disease in the lung?

A

atopic asthma

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103
Q

what type of airway disease is asthma?

A

obstructive

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104
Q

how do you classify asthma? (3 questions)

A

early or late onset?
atopic or non-atopic?
extrinsic or intrinsic?

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105
Q

what is intrinsic asthma?

A

non-allergic asthma

not IgE mediated

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106
Q

what is extrinsic asthma?

A

response to external allergen

IgE mediated

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107
Q

name 3 common triggers of atopic asthma?

A

dust mite
grass pollen
animal dander

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108
Q

what is the clinical manifestation of muscle spasm caused by release of vasoactive substances in the lung?

A

wheeze (caused by bronchoconstriction)

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109
Q

what is the clinical manifestation of mucosal inflammation caused by release of vasoactive substances/inflammatory mediators in the lung?

A

sputum production (caused by mucosal oedema and increased secretions)

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110
Q

what is the clinical manifestation of the inflammatory cell infiltrate caused by release of vasoactive substances/inflammatory mediators in the lung?

A

yellow sputum (caused by infiltration of lymphocytes and eosinophils into bronchioles)

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111
Q

what 2 factors caue the narrowing of the airways during an asthma attack?

A

excess mucus

contracted brochiole smooth muscle (in spasm)

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112
Q

what is the clinical name for ‘hives’?

A

urticaria

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113
Q

what is angioedema?

A

self-limited, localised swelling of subcutaneous tissues or mucous membranes
(non pitting oedema)

114
Q

what are the respiratory signs/symptoms of anaphylaxis?

A

bronchoconstriction causing wheeze

laryngeal obstruction causing stridor

115
Q

what are the cardiovascular signs/symptoms of anaphylaxis?

A

hypotension
cardiac arrhythmias
MI

116
Q

what are the GI signs/symptons of anaphylaxis?

A

oral itching
vomiting
diarrhoea
abdominal pain

117
Q

what can occur in the eyes, nose, lips during anaphylaxis?

A

conjuctival injection
rhinnorrhea
angioedema of lips and mucous membranes

118
Q

where can become itchy during anaphylaxis?

A

palms
soles of feet
genitalia
mouth

119
Q

what is the pathophysiology of non-allergic mast cell degranulation?
(ie non IgE mediated allergic-type reactions)

A

spontaneous mast cell degranulation

due to mast cells becoming twitchy

120
Q

what 2 types of drugs can cause spontaneous mast cell degranulation?

A

opiates

NSAIDS

121
Q

what is physical urticaria?

A

urticaria in response to pressure or heat

non IgE allergic-type reaction

122
Q

what type of drug can commonly cause spontaneous mast cell degranulation in the lungs? (induces asthma, but non IgE mediated)

A

aspirin induced asthma

(also other NSAIDs eg diclofenac,

123
Q

what is samters triad?

A

a patient who has asthma, nasal polyps and salicylate sensitivity (very severe aspirin sensitivity)

124
Q

how do you investigate a food allergy?

A

oral challenge

125
Q

what 3 elective investigations can confirm an allergy?

A

skin prick tests
specific IgE test (RAST test)
challenge test

126
Q

what is a challenge test?

A

supervised exposure to the putative antigen

127
Q

during an acute allergic reaction, how do you confirm anaphylaxis?

A

check serum mast cell tryptase levels

shows evidence of widespread mast cell degranulation

128
Q

what is the gold standard investigation for confirming an allergy?

A

skin-prick tests

129
Q

what does a positive skin-prick test show?

A

local wheal and flare response

130
Q

what type of drugs should be discontinued for at least 48 hours before a skin-prick test?

A

antihistaines

corticosteriods do not influence skin prick tests

131
Q

what are the 4 advantages of skin prick testing?

A

cheap
quick (15 mins)
unrivalled sensitivity of most allergens
patient can see the result

132
Q

what are the 2 disadvantages of skin prick testing?

A

requires experience for interpretation

very rarely may induce anaphylaxis

133
Q

what is a RAST test?

A

a specific IgE test

134
Q

how does a specific IgE test work?

A

you measure the amount of IgE in serum directed against specific allergen

135
Q

when is a specific IgE test used over a skin -prick tests?

A

for patients who are on antihistamines

136
Q

what is a better indicators of allergy? (total IgE or specific IgE)

A

specific IgE

many other causes of an elevated IgE

137
Q

in what type of allergic reactions do tryptase levels rise in?

A

only anaphylaxis

not in local levels

138
Q

what are the 6 ain ways to manage an IgE mediated allergic disorder?

A
  1. avoidance of allergen
  2. block mast cell activation
  3. prevent effects of mast cell activation
  4. anti-inflammatory agents
  5. management of anaphylaxis
  6. immunotherapy
139
Q

what is sodium cromoglycate?

A

a mast cell stabiliser

140
Q

how does sodium cromoglycate work in the treatment of allergies?

A

stabilise mast cell membranes and so block mast cell activation and degranulation

141
Q

how is sodium cromoglycate administered?

A

topical (nasal) spray

poor oral absorption

142
Q

how do anti-histamines work in the treatment of allergies?

A

a H1 receptor antagonist which blocks effects of histamine to prevent the effects of mast cell activation and degranulation

143
Q

how do leukotriene receptor antagonists work in the treatment of allergies?

A

a leukotriene receptor antagonist which blocks the effects of leukotrienes (which are synthesised by mast cells after activation) to prevent the effects of mast cell activation and degranulation

144
Q

what type of drug is montelukast?

A

leukotriene receptor antagonist

145
Q

how do corticosteriods work in the treatment of allergies?

A

anti-inflammatory effects
inhibits formation of many different inflammatory mediators (such as platelet activating factor, prostaglandins and cytokines)

146
Q

what is the step up treatment protocol for asthma?

A
  1. occasional use of inhaled short-acting B2 adrenoceptor agonist (SABA)
  2. low dose inhaled corticosteriod
  3. low/moderate dose inhaled corticosteroid and long-acting B2 adrenoceptor agonist (LABA)
  4. high dose inhaled corticosteroids and regular bronchodilators and leukotriene receptor antagonist or theophylline
  5. regular oral corticosteroids
147
Q

what would you also give to a patient with atopic asthma if they can’t avoid trigger?

A

anti-histamine

148
Q

what is the treatment for anaphylaxis?

A

self-injectable adrenaline

149
Q

how does adrenaline work in the treatment of anaphylaxis?

A

acts on B2 adrenergic receptors to constrict arterial smooth muscle (increases blood pressure and limits vascular leakage)
and to dilate bronchial smooth muscle (decreases airflow obstruction)

150
Q

what is immunotherapy?

A

controlled exposure to increasing amounts of allergen

151
Q

how is the allergen administered in immunotherapy?

A

subcutaneous injections

152
Q

what is the main mechanism within direct cell killing? (type 2 hypersensitivity)

A

antibody binds to cell surface antigens

153
Q

what results from the binding of the antibody to the cell surface antigens in direct cell killing? (type 2 hypersensitivity)

A

activation of complement (cell lysis, opsonisation)

antibody-mediated phagocytosis

154
Q

what are the 3 ways of complement activation?

A

classical pathway
lectin pathway (MBL)
alternative pathway
(All result in C3 production which starts complement cascade)

155
Q

what are the 4 effects of complement activation?

A
  1. direct killing of encapsulated bacteria (with membrane attack complex)
  2. opsonisation to enhance phagocytosis
  3. solubilisation of immune complexes
  4. chemotaxis (stimulates migration of neutrophils and macrophages)
156
Q

what does the membrane attack complex (C5b, C6, C7, C8, C9) do?

A

punches holes in bacterial cell membranes

157
Q

how does complement achieve chemotaxis? (which stimulates migration of neutrophils and macrophages)

A

increases vascular permeability to increase cell trafficking to the site of inflammation

158
Q

specific fragments of complement proteins are released after activation and are the direct cause of the increased vascular permeability. what is the name of these fragments?

A

anaphylotoxins

159
Q

what type of feedback is the process of complement fragments dissolving immune complexes (which triggered them in the fist place)?

A

negative feedback

160
Q

by complement dissolving the immune-complexes, what occurs?

A

switching off of complement activation

161
Q

what are the 2 main immunoglobulins involved in direct cell killing? (type 2 hypsensitivity)

A

IgM and IgG

162
Q

what type of sensitivity is ABO blood transfusion reactions?

A

type 2 hypersensitivity

163
Q

what 4 signs/symptoms shows the overwhelming systemic inflammatory response during a ABO blood transfusion haemolytic transfusion?

A

pyrexia and rigors
tachycardia/tachypnoea
hypotension (and therefore dizziness, headaches)
chest or lumbar pain

164
Q

what type of immune hypersensitivity reaction are certain autoimmune diseases which causes cell lysis?

A

type 2: direct cell killing

165
Q

what are the 2 ways to manage a type 2 hypersensitivity?

A

plasmapheresis

immunosuprresion

166
Q

how does plasmapheresis work in the management of type 2 hypersensitivity? (direct cell killing)

A

removes the pathogenic antibody

167
Q

how does immunosuppresion work in the management of type 2 hypersensitivity reactions? (direct cell killing)

A

switches off B cell production of antibody

168
Q

which is more effective for the treatment of type 2 hypersensitivity reactions (autoimmune- direct cell killing), plasmapharesis or immunosuppresion?

A

immunosuppresion as there is usually a rebound antibody production which limits the efficacy of plasmapheresis

169
Q

what is the main feature of a type 3 hypersensitivity reaction? (immune-complex mediated)

A

the accumulation of immune complexes (in response to an antigen) in small vessels which causes complement activation and an infiltration of macrophages and neutrophils

170
Q

what type of hypersensitivity is acute hypersensitivity pneumonitis? (farmers lung and bird fanciers lung etc)

A

type 3 hypersensitivity- immune complex mediated

171
Q

what is the agent that causes acute bird fanciers lung? (type 3 hypersensitivity- immune complex mediated)

A

avian serum proteins

172
Q

what are the organisms that causes acute farmers lung? (type 3 hypersensitivity-immune complex mediated)

A

aspergillus fumigatus
micropolyspora faeni
(2 types of fungi)

173
Q

in a normal patient what can aspergillus cause?

A

aspergiloma

a fungal ball in a pre-existing cavity- ie from prev TB or existing CF etc

174
Q

in an immunosuppressed patient what can aspergillus cause?

A

invasive aspergillosis

175
Q

what is the main feature of acute hypersensitive pneumonitis?
(type 3 hypersensitivity- immune cell mediated)

A

immune complexes deposited in the walls of alveoli and bronchioles

176
Q

what symptoms does acute hypersensitivity pneumonitis cause?

A

wheezing and malaise 4-8 hours after exposure to antigen

may be associated with dry cough, pyrexia, SOB

177
Q

why does a wheeze occur with acute hypersensitivity pneumonitis?

A

inflammation of terminal bronchioles and alveoli caused by activated phagocytes and complement

178
Q

why does breathlessness occur with acute hypersensitivity pneumonitis?

A

alveolitis- caused by activvated phagocyes and complement- results in decreased efficiency of gas transfer

179
Q

why does malaise and pyrexia occur with acute hypersensitivity pneumonitis?

A

systemic manifestation of inflammatory response

180
Q

what type of hypersensitivity reaction is SLE?

A

type 3- immune complex mediated

181
Q

what type of autoantibodies are produced in SLE?

A

antibodies against contents of cell nuclei

182
Q

what are the 3 ways to manage type 3 hypersensitivity reactions?
(immune complex mediated)

A

avoidance (not always an option)
corticosteroids (decrease inflammation)
immunosuppression (decrease antibody production)

183
Q

are corticosteroids based on glucocorticoids or mineralocorticoids?

A

glucocorticoids

184
Q

what is the key feature of type 4 hypersensitivity? (delayed type hypersensitivity)

A

T cell mediated hypersensitivity in response to an antigen

infiltrate of activated T cells

185
Q

what is the pathophysiology of type 4 hypersensitivity reactions? delayed type hypersensitivity?

A

initial sensitisation to antigen generated primed T cells
subsequent exposure activated previously primed T cells, recruitment of macrophages, other lymphocytes, neutrophils and release of proteolytic enzymes
results in persistent inflammation

186
Q

what forms because of the persistent inflammation and collection of activated macrophages and lymphocytes in a delayed type hypersensitivity reaction?

A

granuloma

187
Q

what autoimmune conditions are associated with delayed type hypersensitivity reactions?

A

diabetes type 1
psoriasis
rheumatoid arthritis

188
Q

what non-autoimune conditions are associated with delayed type hypersensitivity reactions?

A 
nickely hypersensitivity
TB
leprosy
sarcoidosis
cellular rejection of solid organ transplant
189
Q

what is a granuloma?

A

an organised collection of persistently activated macrophages and lymphocytes

190
Q

what can trigger a granuloma?

A

diverse antigenic agents or inert foreign materials with failure to remove stimuli

191
Q

what can granulomas n the lungs lead to?

A

tissue damage and fibrosis

192
Q

what are the management options of sarcoidosis?

A

nothing (spontaneous remission)
NSAIDs (for acute onset of diease)
systemic corticosteroids (to block T cell and macrophage activation)

193
Q

what type of hypersensitivity are dust diseases such as berylliosis and silicosis?

A

type 4- delayed type hypersensitivity

194
Q

what type of hypersensitivity is the chronic stage of hypersensitivity pneumonitis?

A

type 4-delayed hypersensitivity

195
Q

what has been the most effective public health intervention in the world?

A

clean water

196
Q

what has been the 2nd most effectiv public health intervention in the worl?

A

vaccination

197
Q

if a child who has had a Haemophilus Influenza B vaccine gets HIB what question should you ask?

A

is patient immunosuppressed?

198
Q

compare first and second exposure to an antigen in terms of B cell maturation?

A

1st time: B cells need helper t cells to stimulate maturation
2nd time: only memory B cells needed

199
Q

compare first and second exposures to an antigen in terms of IgG productions?

A

1st time: IgG made over the course of the week

2nd time: due to memory B cells IgG can be made immediately

200
Q

what does a secondary antibody response usually result in?

A

the ability to clear infection during incubation period, before the onset of clinical features

201
Q

what cells does memory form in?

A

B and T cells

202
Q

what is the meaning of immunisation?

A

the process through which an individual develops immunity/memory to a disease (includes natrual infection)

203
Q

what is the meaning of vaccination?

A

the deliberate administration of antigenic material to produce immunity in to a disease

204
Q

compare active immunity to passive immunity

A

active immunity: protection produced by the persons own immune system (vaccine or natrual infection)- usually permanent
passive immunity: protection transferred from another person or animal- temporary and wanes over time

205
Q

how does an active vaccine work?

A

stimulates immune response and generation of immunological memory to antigen through same pathways as natural infection

206
Q

are you increase the similarity between the vaccine to the disease what effect does it have on the 2nd exposure immune response to the disease?

A

improves the immune response to the disease

207
Q

what is variolation?

A

a type of immunisation where the same organisms is being administered as the disease causing organism, but the route of administration is different

208
Q

what are the 5 key features of an inactivated vaccine?

A 
cannot replicate
generally not as effective as live vaccines
immune response primarily antibody based
antibody titer may diminish with time
require multiple doses
209
Q

how do you make an inactivated vaccine?

A

expose pathogen to:
chemical fixatives
heat denaturation
irradiation

210
Q

what are the problems you face with making an inactivated vaccine?

A

under inactivation (viable pathogens within organism) or over inactivation (loss of conformational antibody binding sites)

211
Q

what was the problem with the Salk Polio vaccine?

A

under inactivation

212
Q

what are the 4 advantages of an inactivated vaccine?

A
  1. can be made quickly (to prevent epidemics)
  2. may elicit good antibody responses
  3. easy to store- no refrigeration required
  4. usually safe (can be given to immunocompromised individuals)
213
Q

what are the 3 disadvantages of an inactivated vaccine?

A
  1. due to lack of replication may be hard to stimulate an immune response
  2. poor at eliciting T cell responses
  3. variable memory (boosters needed)
214
Q

what are the 3 disadvantages of an inactivated vaccine?

A
  1. due to lack of replication may be hard to stimulate an immune response
  2. poor at eliciting T cell responses
  3. variable memory (boosters needed)
215
Q

what can be used to improve the immunogenicity of an inactivated vaccine?

A

adjuvant

216
Q

what is an adjuvant?

A

a substance added to a vaccine which increases stimulation of immune response by creating an inflammatory environment

217
Q

what are the 2 problems of adjuvants?

A

toxic

alter the immune response (immunity response is generated to vaccine:protein conjugate rather than the vaccine itself)

218
Q

what 2 types of inactivated vaccines can you get?

A

whole cell vaccines

fractional vaccines

219
Q

what is a whole cell vaccine?

A

whole organism used in the vaccine

220
Q

what is a fractional vaccine?

A

only part of the organism used in the vaccine (subunit or toxoid or only polysaccharide)

221
Q

what is the major positive of fractional vaccines compared to whole cell vaccine?

A

no risk of infection

222
Q

what is a polysaccharide fractional inactivated vaccine?

A

only polysaccharides of outer capsule of bacteria used

223
Q

what is the problem about a polysaccharide fractional inactivated vaccine?

A

polysaccharides are not good at stimulating response- antibodies are generated with less functional activity

224
Q

what is a live attenuated vaccine?

A

exposure to a less virulent version (weakened) of the same pathoden

225
Q

how do you weaken a pathogen to use for live attenuated vaccine?

A

passaging- growing attenuated strains through repeated subculturing (in other cells or animals)

226
Q

what are the 3 advantages of a live attenuated vaccine?

A
  1. v similar to natural infection, so relevant effector mechanisms elicited (antibody, activated T cells)
  2. localised, strong response
  3. memory good, therefore boosting not usually required
227
Q

what are the 3 disadvantages of a live attenuated vaccines?

A
  1. immune response can be interfered with by circulating antibody
  2. safety issues
  3. fragile- need to be stored and handled carefully
228
Q

what are the safety issues that come with live attentuated vaccines?

A

may acquire new mutations and revert to virulence

may cause infection in immunocomprimosed host

229
Q

what form of virus is the Sabin polio vaccine?

A

live attenuated virus

subcultured in monkey kidney cells

230
Q

what are the naturally acquired sources of passive immunity?

A

transplacental transfer of antibody

231
Q

what are the 4 therapeutic sources of passive immunity?

A
  1. pooled normal human immunoglobulin
  2. hyperimmune globulin
  3. heterologous hyperimmune serum
  4. monoclonal antibody against specific pathogen
232
Q

what is palivizumab?

A

a monoclonal antibody produced against a single determinant of respiratroy syncitial virus (RSV)

233
Q

what is palivizumab used in?

A

prevention of sever lower respiratory tract infections in high-risk infants

234
Q

how often does palivizumab need to be administered?

A

monthly intramuscular injections during RSV season

235
Q

what is an addiction vaccine?

A

aim to reduce drug levels in the brain by stimulating an antibody wihich binds to the drug before it enters the brain

236
Q

what are the 3 reasons for improved outcome of transplant?

A

better understanding of immunology of rejection
better immunosuppressive agents
improved post-transplant monitoring

237
Q

what is the human form of MHC?

A

HLA

238
Q

what HLA class do all nucleated clles express?

A 
HLA class I
(HLA-A, HLA-B, HLA-C)
239
Q

what HLA class do antigen presenting cells also express?

A 
HLA class II
(HLA-DR, HLA-DQ, HLA-DP)
240
Q

how many variants of each HLA molecule do individuals express?

A

2

241
Q

what is the purpose of HLA molecule polymorphism?

A

maintenance of diversity

242
Q

in HLA matching in transplantation which class is more important to be matched?

A

HLA class II

243
Q

which HLA is more important to be matched in transplantation? (HLA-A or HLA-B)

A

HLA-B > HLA-A

244
Q

why dont lung and heart transplants use HLA matching to allocate donor?

A

because of limited donor pool and prolongation of cold ischaemic time

245
Q

which organ is HLA macthing used in transplantation? (kidney or liver)

A

kidney

246
Q

what cytokine do activated T cells make that causes T cel proliferation and maturation?

A

IL 2

247
Q

what type of hypersensitivity response is acute cellular rejection?

A

type 4 hypersensitivity

248
Q

how can you detect deteriorating graft function of a kidney transplant?

A

rise in creatinine, fluid retension, hypertension

249
Q

how can you detect deteriorating graft function of a lung transplant?

A

breathlessness

pulmonary infiltrate

250
Q

how can you detect deteriorating graft function of a liver transplant?

A

rise in LFTs

coagulopathy

251
Q

what are the general symptoms of deterioration of graft function of an organ transplant?

A

pain and tenderness over graft

fever

252
Q

what are the 4 types of transplant rejection?

A

hyperacute rejection
acute cellular rejection
acute vascular rejection
chronic allograft failure

253
Q

when does hyperacute transplant rejection occur?

A

minutes to hours after transplant

254
Q

what is the pathology of hyperacute transplant rejection?

A

thrombosis and necrosis

255
Q

what is the mechanism behind hyperacute transplant rejection?

A

preformed antibody and complement fixation

256
Q

when does acute cellular rejection occur?

A

5-30 days after transplant

usually T cell response is 5-7 days, 5-30 because of the immunosuppressive therapy post op

257
Q

what is the pathology behind acute cellular rejection?

A 
cellular infiltrate
(type 4 hypersensitivity)
258
Q

what is the mechanism behind acute cellular rejection?

A

CD4 and CD8 T cells

259
Q

what is the treatment for acute cellular rejection

A

immunosuprresion

260
Q

when does acute vascular rejection occur?

A

5-30 days after transplant

261
Q

what is the pathology behind acute vascular rejection?

A

vasculitis

262
Q

what is the mechanism behind acute vascular rejection?

A

T lymphocytes and antibody

263
Q

what is the treatment for acute vascular rejection?

A

immunosuppression

264
Q

when does chronic allograft failure occur?

A

.30 days after organ transplant

265
Q

what is the patholgy behind chronic allograft failure?

A

fibrosis, scarring

266
Q

what types of mechanisms are behind chronic allograft failure?

A

immune and non-immune mechanisms

267
Q

what is the treatment for chronic allograft failure?

A

minimse drug toxicity, hypertension, hyperlipidaemia

268
Q

in hyper acute transplantation rejection, why would an individual have preformed antibodies against donor cells?

A

if patient has been exposed to someone elses white cells or HLA
(ie during pregnancy)

269
Q

when are antibodies against blood group antigens formed?

A

naturally occuring pre-formed antibodies

270
Q

when are antibodies against HLA antigens formed?

A

arrise through previous exposure

271
Q

when are antibodies against HLA antigens formed?

A

arise through previous exposure

272
Q

what will transplantation of any donor tissue across incompatible blood groups result in?

A

hyperacute rejection

273
Q

what is the result of hyperacute rejection?

A

irretrievable graft loss

274
Q

how do you prevent hyperacute rejection?

A

ABO matching

identify if recipient has any anti-HLA antibodies

275
Q

what is crossmatching?

A

directly tests if serum from recipient is able to bind and/or kill donor lymphocytes

276
Q

what are the immune mechanisms behind chronic allograft failure?

A

HLA mismatch

277
Q

what are the non-immune mechanisms behind chronic allograft failure?

A 
non-compliance with medication
hypertension
hyperlipiademia
older donor age
calcineurin inhibitors
278
Q

what are the 3 major complications of long-term immune suppression

A

infection
malignancy
atheroclerosis

279
Q

what immunosuppressant drug used for the prevention of transplant rejection is associated with gingival hypertrophy?

A

ciclosporin

280
Q

what is the indication for a lung transplant?

A

advanced respiratory failure with life expectancy less than 2/3 years

Respiratory (10 decks)

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