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Y1 Respiratory > Immunology > Flashcards

Immunology Flashcards

1
Q

What are the hallmarks of immune deficiency?

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

What is a primary immunodeficiency disorder?

A

A disorder characterised by the absence of one or more parts of the immune system.

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3
Q

Which primary immunodeficiencies are most associated with sinusitis and otitis media?

A

Primary antibody deficiency

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4
Q

Give examples of primary antibody deficiency.

A

Selective IgA deficiency
Common variable immunodeficiency
Specific antibody deficiency
X-linked agammaglobulinemia

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5
Q

What type of conditions should be considered in patients with laryngeal angioedema?

A

Complement system disorders

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6
Q

Give an example of a complement system disorder

A

Hereditary angioedema

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7
Q

What is the most common pulmonary symptom of many primary immune deficiencies?

A

Pneumonia

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8
Q

True or false? All people suffering from a primary immune deficiency disorder are registered.

A

True

The united kingdom primary immune deficiency registry (UKPID) serves to track these conditions

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9
Q

What types of pathogens are people suffering from congenital neutropenia more susceptible too?

A

Bacteria

Fungi

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10
Q

What is Kostmann syndrome?

A

A severe congenital neutropenia

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11
Q

What are the clinical manifestations of Kostmann syndrome?

A

Severe chronic neutropenia from birth
Accumulation of precursor cells in the bone marrow
Recurrent bacterial and fungal infections with no pus

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12
Q

What is the treatment for severe congenital neutropenia?

A

Recombinant G-CSF

A haemopoietic growth factor

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13
Q

What is a recurrent infection with no pus suggestive of?

A

Severe neutropenia (most likely congenital)

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14
Q

What is Leukocyte adhesion deficiency?

A

An autosomal recessive immunodeficiency

Causes failure of neutrophil adhesion and migration

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15
Q

What is the clinical picture of leukocyte adhesion deficiency?

A

Marked leukocytosis and localized bacterial infections

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16
Q

What is Chronic granulomatous disease?

A

A condition causing the absence of the respiratory burst in phagocytes
Commonly by effecting the NADPH oxidase complex

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17
Q

What are the clinical features of chronic granulomatous disease?

A

Recurrent deep bacterial infections and recurrent fungal infections
Failure to thrive
Lymphadenopathy and hepatosplenomegaly
Granuloma formation

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18
Q

Briefly explain the IL-12: IFN-gamma network

A

A mechanism to improve action against intracellular pathogens
Infected macrophages produce IL-12
IL-12 stimulates NK cells and Th1 cells to secrete IFN-gamma
IFN-gamma stimulates macrophages and neutrophils to produce TNF-alpha which stimulates activation of the NADPH oxidase complex

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19
Q

What are the treatments for phagocyte deficiencies?

A

Immunoglobulin replacement therapy (IVIg)
Aggressive management of infection (PO/IV antibiotics or antifungals, surgical abscess drainage)
Definitive therapy is by a haematopoietic stem cell transplant

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20
Q

Where do mature B cells come from?

A

The bone marrow (B for Bone)

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21
Q

Where do mature T cells come from?

A

The thymus (T for Thymus)

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22
Q

What is the normal function of an antibody?

A

To aid the identification of pathogens by opsonisation
To recruit/activate other aspects of the immune response (e.g complement, phagocytes, NK cells)
Neutralisation of toxins

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23
Q

What is the main group of pathogens antibodies act against?

A

Bacterias

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24
Q

How are macrophages stimulated?

A

TH1 cells costimulation with pathogen-derived peptide

IFN-gamma

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25
Q

How are TH0 cells stimulated?

A

By IL-2 released from TH0 cells

Stimulation of CD4+ T cells through MHC-II presented antigen to T-cell receptors cause differentiation of TH0 cells

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26
Q

How are CD8+ T cells stimulated to cytotoxic T lymphocytes?

A

MHC-I costimulation by antigen presenting cells leads to differentiation
IL-2 from TH0 cells contributes to this

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27
Q

How are B cells stimulated?

A

Cytokines + Costimulation from TFH cells along with stimulation of B-cell receptors and opsonin receptors by opsinosided pathogens

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28
Q

What is reticular dysgenesis?

A

Failure of production of all lymphocytes

Fatal without a haematopoietic stem cell transplant

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29
Q

What is severe combined immunodeficiency?

A

The failure in the production of lymphocytes

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30
Q

Give the clinical phenotype of combined immunodeficiency.

A 
Unwell by 3 months of age
Persistent diarrhoea
Failure to thrive
All types of infections
Unusual skin disease (Graft versus host disease)
Family history of early infant death
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31
Q

What is the most common genetic defect in severe combined immunodeficiency?

A

Common gamma chain (X-linked)

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32
Q

What is the commonest form of severe combined immunodeficiency?

A

X-linked SCID

45% of all SCIDs

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33
Q

Explain X-linked SCID

A

Mutation of the IL-2Rgammac gene
(A common element of many cytokine receptors)
Results in the inability to respond to cytokines

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34
Q

Give the clinical phenotype of X-linked SCID

A

Very low or absent T cells
Normal or increased B cells
Poorly developed lymphoid tissue and thymus

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35
Q

Give the treatment of severe combined immunodeficiency

A

Prophylactic: Avoid infections (prophylactic antibiotics/antifungals) aggressive treatment of existing infections, antibody replacement therapy
Definitive treatment: Haemopoietic stem cell transplant

36
Q

What is DiGeorge syndrome?

A

A failure in production of CD4+ and CD8+ T cells.

37
Q

Give the phenotype of DiGeorge syndrome.

A

Development defect of the 3rd/4th pharyngeal pouch
Low set abnormally folded ears, high forehead, cleft palate, small mouth and jaw
Hypocalcaemia
Oesophageal atresia (Incomplete oesophagus)
T cell lymphopenia
Complex congenital heart disease

38
Q

What type of infection is mostly associated with DiGeorge syndrome?

A

Viral due to lack of CD4+ and CD8+ T cells

All due to lack of CD4+ T cells

39
Q

Give the management of DiGeorge syndrome.

A

Correct metabolic/anatomic abnormalities
Prophylactic antibiotics
Early and aggressive treatment of infections
The possible need for IV immunoglobulin replacement

40
Q

In DiGeorge syndrome does T cell function improve or worsen with age?

A

Improve

Suggestive of extra-thymic maturation site of T-cells

41
Q

What is X-linked agammaglobulinaemia?

A

An absense of B cells due to a defect in Bruton tyrosine kinase

42
Q

What is the typical presentation of X-linked agammaglobulinaemia?

A

Lower and upper respiratory infections, diarrhoea, cellulitis, meningitis and sepsis
Usually due to Gram-positive highly pathogenic encapsulated organism
(Strep.pneumonia, H.influenzae)

43
Q

Which 2 pathogens are particaully associated with X-linked agammaglobulinaemia?

A

Streptococci pneumonia

Haemophilus infleunzae

44
Q

What is Hyper IgM syndrome?

A

A defect in class-switch recombination resulting in only/effectively only IgM being produced
A severe reduction in serum IgG and IgA
Normal/elevated IgM
Normal B cell numbers

45
Q

What is meant by class switch recombination?

A

The change of B-cell differentiation to plasma IgM to another type of Ig

46
Q

What is Selective IgA deficiency?

A

A defect in class-switch recombination resulting in a lack of IgA plasma cells (and thus a lack of IgA)

47
Q

What type of infections are seen in B cell deficiencies?

A

Recurrent bacterial infection often by very common organisms

48
Q

What is a hypersensitivity reaction?

A

The immune response that results in bystander damage to the self
Usually an exaggeration of normal immune mechanisms
(Includes allergies and autoimmunity)

49
Q

Explain the Gel and Coomb’s classification.

A

Type I: Immediate hypersensitivity (IgE)
Type II: Direct cell effect
Type III: Immune complex-mediated
Type IV: Delayed-type

50
Q

What is a Type I hypersensitivity?

A

An IgE mediated antibody response to an external antigen (AKA allergen)

51
Q

Give the theory behind the increase in allergic-type hypersensitivity reactions.

A

The hygiene hypothesis
The hygiene hypothesis poses that an overclean childhood promotes differentiation to Th2 cells leading to the allergic response

52
Q

What causes stimulation of B-cells to class switch to IgE plasma cells.

A

IL-4, IL-5, IL-13 from TH2 cells

53
Q

Explain the mechanism of an allergic reaction.

A

Sensitisation stage: Mast cells and eosinophils express Fc receptors for IgE antibodies, on the first allergen exposure IgE binds to these Fc receptors and the allergen is cleared.
Allergic stage: When an allergen is re-encountered the allergen binds to the IgE coats causing the release of pre-formed pro-inflammatory mediators

54
Q

Explain the MoA of a mast cell.

A

Resident in tissues (especially at the external environment interface)
Produce vasoactive inflammatory substance (Histamine, tryptase, heparin. leukotrienes, prostaglandins and pro-inflammatory cytokines)

55
Q

What type of pathogen are mast cells particularly useful in?

A

Mast cells

56
Q

What are the clinical features of a type I hypersensitivity reaction?

A

Muscle spasm (e.g bronchoconstriction)
Mucosal inflammation
Inflammatory cell infiltrate
Oedema (Anaphylaxis)

57
Q

What is a type II hypersensitivity?

A

A direct cell effect reaction involving IgM or IgG antibodies to the cell surface antigens.

58
Q

What is the mechanism of a type II hypersensitivity reaction?

A

Self-reactive antibodies cause:
Activation of the complement
Opsonisation and phagocytosis
Antibody-dependent cellular cytotoxicity via NK cells
Anti-receptor antibodies disturb the normal function of cell surface receptor

59
Q

Explain Goodpasture’s syndrome

A

Autoreactive antibodies against the basement membrane [anti-GBM] (TypeII hypersensitivity)
Effects the alveoli and glomeruli
Due to exposure to environmental insult

60
Q

What is the treatment of Goodpasture’s syndrome

A

Corticosteroids
Plasmapheresis

Smoking cessation

61
Q

What is a type III hypersensitivity?

A

Immune complex-mediated
The presence of immune complexes activated complement and attracts inflammatory cells such as neutrophils
Enzymes from inflammatory cells cause local damage (often at the basement membrane)

62
Q

Explain the immunology of acute hypersensitivity pneumonitis.

A

A type III hypersensitivity reaction

Leukocyte accumulation and inflammation within the alveoli

63
Q

What is the clinical picture of acute hypersensitivity pneumonitis?

A

Wheeze and malaise 4-8hours post antigen exposure
Possible associated dry cough, pyrexia and breathlessness
O/E often normal

64
Q

What is the management of type III hypersensitivity reactions?

A

Avoidance of the causative antigen
Corticosteroids, decrease the inflammation
Immunosuppression, decrease the production of antibodies

65
Q

Explain type IV hypersensitivity reactions.

A

Activation of CD4+ T cells and Th1 cells along with macrophages cause damage to tissues.
This is due to antigen exposure directly to cells.

66
Q

What type of hypersensitivity is sarcoidosis?

A

Type IV?

67
Q

Explain the pathogenesis of sarcoidosis.

A

Inhalation of an antigen stimulates alveolar macrophages along with T cells, this fails to clear leading to persistent immune activation damage and fibrosis.

68
Q

What is the management of sarcoidosis?

A

Watching and waiting often goes into spontaneous remission
NSAIDs for acute onset
Systemic corticosteroids to block T cell and macrophage activation

69
Q

What is autoimmunity?

A

The presence of immune responses against self-tissue/cells

This can be harmful if the titres of autoreactive antibodies or T cells is high, can also be harmless

70
Q

What 3 states can T and B cells be when generated in primary lymphoid tissue

A

Not expressing an antigen-specific receptor
Expressing a non-self antigen-specific receptor (These are good B&T cells)
Expressing a self antigen-specific receptor (These can cause a problem)

71
Q

Explain central tolerance.

A

The deletion of self-reactive lymphocytes in primary lymphoid tissues.

72
Q

Explain peripheral tolerance.

A

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

73
Q

What are the major cells of peripheral tolerance

A

Regulatory T cells (Treg cells)

74
Q

What are the 2 major cytokines produced by Treg cells?

A

TGF-beta, IL-10

75
Q

Explain briefly the pathogenesis of autoimmune disease.

A

Genetic susceptibility is present
Initiating event leads to break down of immune tolerance to self-antigen
This leads to an autoimmune phenomenon or an autoimmune disease

76
Q

What is IPEX syndrome?

A

An X-linked genetic autoimmune disease
Presents in early childhood
Characterised by overwhelming systemic autoimmunity

77
Q

What are the symptoms of IPEX syndrome?

A 
Severe infections
Intractable diarrhoea
Eczema
Very early onset diabetes mellitus
Autoimmune manifestations
78
Q

What is the treatment of IPEX syndrome?

A

Hematopoietic stem cell transplantation

Supportive care: immunosuppressive drugs + parenteral nutrition

79
Q

What are MHC/HLA molecules?

A

Proteins which express peptides
T cells can only be stimulated by peptides expressed on these molecules
Can be class I or class II

80
Q

Why are MHC molecules polymorphic?

A

Allows maintenance of diversity in antigen responsiveness at the population level and at the level of the individual.

81
Q

Are autoimmune conditions more common in men or women?

A

Mostly in women

Diabetes mellitus is more common in men

82
Q

What is a superantigen?

A

A molecule that can cause MHC receptor stimulation without the presence of an antigen

83
Q

What is Grave’s disease?

A

A odd type II hypersensitivity disease
A leading cause of hyperthyroidism
Auto-antibodies are generated that bind to the thyroid stimulating hormone receptor

84
Q

What type of hypersensitivity is SLE?

A

type III

85
Q

What type of hypersensitivity is rheumatoid arthritis?

A

type IV

Y1 Respiratory (5 decks)

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