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The respiratory system > immunology > Flashcards

immunology Flashcards

1
Q

What is the hallmark of immune deficiency?

A

Recurrent infections

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2
Q

What is SPUR and what does it indicate?

A
  • indicates immune deficiency
  • Serious infections
  • Persistant infections
  • Unusual Infections
  • Recurrent infections
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3
Q

What are some signs and symptoms suggestive of primary immunodeficiency?

A
  • weight loss or failure to thrive
  • severe skin rash
  • chronic diarrhoea
  • mouth ulceration
  • unusual autoimmune disease
  • lymphoproliferative disorders
  • cancer
  • family history
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4
Q

What are some of the conditions associated with secondary immune deficiency?

A
  • physiological immune deficiency: ageing, prematurity
  • Infection: HIV, measles
  • treatment interventions: immunosuppressive therapy
  • malignancy: cancer of the immune system
  • biochemical and nutritional disorders: malnutrition
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5
Q

What are some of the upper respiratory complications of PIDs

A
  • sinusitis
  • otitis media
  • laryngeal angiodema
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6
Q

What are some of the lower respiratory complications associated with PIDs?

A
  • malignancies
  • interstitial lung disease
  • pneumonia
  • bronchitis, bronchiectasis
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7
Q

What is the most frequent PID associated with sinusitis and otitis media?

A
  • primary antibody deficiency:
  • selective IgA deficiency
  • common variable immunodeficiency
  • specific antibody deficiency
  • X lined agammaglobulinemia
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8
Q

what PID is associated with laryngeal angioedema?

A
  • complement system disorders: hereditary angioedema
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9
Q

Give examples of PIDs associated with pneumonia?

A
  • primary antibody deficiency
  • complement system disorders
  • congenital phagocytosis deficiency
  • combined immunodeficiency
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10
Q

What is severe congenital neutropenia?

A
  • failure to produce neutrophils

- G- CSF is not produced so allow differentiation past progenitor stage

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11
Q

What is Kostmann syndrome?

A
  • severe congenital neutropenia

- rare genetic disorder: mutation in ELANE gene

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12
Q

What are the symptoms of Kostmann syndrome?

A
  • low neutrophil count
  • accumulation of precursor cells in bone marrow
  • recurrent bacterial/fungal infections with no pus
  • can be treated with recombinant G - CSF
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13
Q

What happens in defects in neutrophil trans - endothelial migration?

A
  • phagocytes are unable to bind to endothelial adhesion molecules
  • failure to recognise activation markers expressed on endothelial cells
  • neutrophils are mobilised but cannot exit the blood stream
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14
Q

What are the symptoms of defects in neutrophils trans endothelial migration?

A
  • recurrent bacterial and fungal infections
  • very high blood neutrophil count
  • infection in deep tissues but no pus
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15
Q

What is leukocyte adhesion deficiency?

A
  • rare autosomal recessive PID
  • caused by genetic defect in CD18 intern gene
  • failure of neutrophil adhesion and migration
  • characterised by marked leukocytosis and localised bacterial infections
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16
Q

What is chronic granulomatous disease?

A
  • deficiency of killing mechanism of phagocytes
  • deficiency of p47phox gene which is a component of the NADPH oxidase complex
  • inability to generate ROS
  • impaired killing of microorganism
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17
Q

What are the symptoms of chronic granulomatous disease?

A
  • recurrent deep bacterial infections
  • recurrent fungal infections
  • failure to thrive
  • lymphadenopathy and hepatosplenomegaly
  • granuloma formation
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18
Q

how are phagocyte deficiencies treated?

A
  • immunoglobulin replacement therapy
  • aggressive management of infection
  • definitive therapy: stem cell transplant, gene therapy
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19
Q

What is SCID?

A
  • failure of the production of T and B lymphocytes
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20
Q

What are the symptoms of SCID?

A
  • unwell by 3 months of age
  • persistant diarrhoea
  • failure to thrive
  • many types of infection
  • unusual skin disease
  • family history of early infant death
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21
Q

What type of infections would a patient with SCID get?

A
  • recurrent viral infection
  • recurrent bacterial infections
  • frequent fungal infections
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22
Q

what is the cause of SCID?

A
  • more than 20 possible pathways identified

- presence of different lymphocyte subsets depend on exact mutation

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23
Q

What is X-lined SCID?

A
  • caused by a mutation of a component of the IL-2 receptor
  • failure of T cell and NK cell development
  • production of immature B cells
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24
Q

describe the clinical phenotype of x-lined SCID?

A
  • very low or absent T cells
  • because IL2 is so important for T cell development
  • normal or increased B cells
  • poorly developed lymphoid tissue and thymus
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25
Q

How can SCID be treated?

A
  • prophylactic treatment: avoid infections, aggressive treatment of existing infection, antibody replacement
  • definitive treatment: stem cell transplant
  • gene therapy
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26
Q

What is Brutons x-lined hypogammaglobulinaemia?

A
  • no circulating B cells
  • No plasma cells
  • no circulating antibody after the first 6 months
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27
Q

What are hypersensitivity reactions?

A
  • immune response that results in bystander damage to self
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28
Q

What are type 1 hypersensitivity reactions?

A

IgE mediated antibody response to external antigen

29
Q

Give examples of type 1 hypersensitivity reactions?

A
  • asthma
  • hay fever
  • urticaria
  • angiodema
  • atopic eczema
  • allergies
30
Q

Why is the prevalence of allergy increasing?

A
  • the ‘hygiene hypothesis’

- changes in microbial stimuli influences the maturation of the immune response

31
Q

According to the hygiene hypothesis what may influence increased production of Th2 in a hypersensitivity reaction?

A
  • being an only child
  • widespread use of ABX
  • urban environment with allergen sensitisation
32
Q

Describe the sensitisation stage of an allergic reaction?

A
  • on first encounter with allergen B cells produce antigen specific IgE
  • allergen is cleared
  • residual IgE antibodies bind to circling mast cells via Ice receptors
  • no great consequence
33
Q

describe the allergic stage of an allergic reaction?

A
  • re encounter the allergen
  • allergen binds to IgE coated mast cells and disrupts cell membrane
  • immediate release go vasoactive mediators (histamine, tryptase)
  • also increased expression of pro-inflammatory cytokines and leukotrienes
34
Q

What are the clinical features of allergic disease?

A
  • muscle spasms
  • mucosal inflammation
  • inflammatory cell infiltrate
35
Q

Describe the management of IgE mediated allergic disorders?

A
  • avoidance of allergen
  • block mast cell activation
  • prevent effects of mast cell activation
  • anti-inflammatory agents
  • management of anaphylaxis
  • immunotherapy
36
Q

What are type II hypersensitivity reactions?

A

antibody mediated process in which IgG and IgM are directed against antigens on cells leading to cell lysis

37
Q

What are some of the mechanisms that type II hypersensitivity reactions can occur through?

A
  1. Immunological mechanisms
    - complement system activation
    - opsonisation and phagocytosis
    - ADVV via NK cells or eosinophils
  2. other mechanisms
    - Anti-receptor antibodies disturb the normal function of cell surface receptor
38
Q

give examples of type II hypersensitivity?

A
  • Goodpastures syndrome

- graves disease

39
Q

What is good pastures syndrome?

A
  • autoimmune disease that affects the lungs and kidneys
  • defined by presence of auto reactive antibodies to the a3 chain of type IV collagen
  • thought to result from an environmental insult in a person with genetic susceptibility
40
Q

What is the treatment for good pastures syndrome?

A
  • corticosteroids, cyclophosphamide
  • plasmapheresis
  • stop smoking
41
Q

What is plasmapheresis?

A
  • the removal of plasma from the blood
  • rapidly removes circulating antibodies
  • stops further production of antibodies
  • removes offending agents that may have initiated antibody production
42
Q

What are type III hypersensitivity reactions?

A
  • an accumulation of antibodies (blood vessels, joints and glomeruli) results in:
  • activation of complement
  • opsonisation
  • infiltration and activation of neutrophils and macrophages
43
Q

Give an example of type III hypersensitivity

A
  • Acute hypersensitivity pneumonitis (farmers lung ect)
44
Q

How are type III hypersensitivity disorders managed?

A
  • avoidance
  • decrease inflammation with corticosteroids
  • decrease production of antibody (immunosuppression)
45
Q

What is type IV hypersensitivity?

A
  • delayed immune response that occurs when CD4+Th1 cells recognise foreign antigen in a complex with MHC class II molecules
46
Q

Describe the mechanism of type IV hypersensitivity?

A
  • foreign antigen encountered by CD4+Th1 and MHC class II
  • CD4+T cells secrete IL2 and IFNgamma which mediates the immune response
  • Activated CD8 cells destroy the antigen on contact
47
Q

What are the autoimmune diseases associated with type IV hypersensitivity?

A
  • type 1 diabetes
  • psoriasis
  • rheumatoid arthritis
48
Q

What are the non autoimmune diseases associated with type IV hypersensitivity?

A
  • contact dermatitis
  • TB
  • leprosy
  • sarcoidosis
  • cellular rejection of organ transplant
49
Q

What is autoimmunity?

A
  • the presence of immune responses against self tissues and cells
50
Q

How does the immune system deal with the presence of autoreactive T and B cells?

A

specific tolerance mechanisms are required

  1. deletion of self reactive lymphocytes in primary lymphoid tissues
  2. inactivation of self reactive lymphocytes in peripheral tissues that escape central tolerance
51
Q

What is the role of Treg cells?

A
  • suppress hyper-reactive or auto-reactive T cells via the production of anti-inflammatory cytokines
52
Q

describe the pathogenesis of autoimmune disease?

A
  • genetic susceptibility
  • initiating event
  • breakdown of immune tolerance to self antigens
  • autoimmune phenomena or autoimmune disease
53
Q

what is IPEX syndrome?

A
  • rare genetic disorder of immune dysregulation
  • presents early
  • characterised by overwhelming systemic autoimmunity
54
Q

What is the treatment for IPEX syndrome?

A
  • hematopoetic stem cell transplant (cure)

- supportive care - immunosuppressive drugs ect

55
Q

Describe the genetic features of IPEX syndrome?

A
  • X linked (only males affected)
  • mutation in FOXP3 gene
  • failure of peripheral tolerance mechanisms due to defective/absent T cells (Tregs)
56
Q

which type of cells express class I MHC molecules?

A
  • all nucleated cells
57
Q

Which type of cells express class II MHC molecules?

A
  • specialised antigen presenting cells
58
Q

Why are all HLA (MHC) molecules highly polymorphic?

A
  • maintenance of diversity in antigen responsiveness at the population level and at the level of the individual
  • proteins are processed into many different component peptides
  • different HLA molecules bind to different subsets of peptides
  • to maximise the net availability to bind to all antigenic peptides
59
Q

How does sex influence autoimmune disease?

A
  • sex bias in predisposing genetic factors
  • different hormonal influences on lymphocyte function
  • alteration of the course of some autoimmune disease during pregnancy
60
Q

What are the factors that contribute to autoimmune disease?

A
  • genes
  • immune regulation
  • environment
61
Q

How are genetics associated with autoimmune disease?

A
  • via HLA genes

- autoimmune diseases share genetic associations within other gene loci

62
Q

How is the environment associated with autoimmune disease?

A
  • infection: cross reactivity between antigens expressed by pathogen and self
  • self antigens altered in some way
  • super antigens
  • tissue damage
63
Q

How is immune regulation associated with autoimmune disease?

A
  • importance of immune regulation in maintaining self tolerance
64
Q

How can autoimmune diseases be classified?

A
  1. clinical classification
    - organ specific disease
    - non organ specific or multi-system disease
  2. pathological classification
    - gel and coombs classification
65
Q

What is graves disease?

A
  • leading cause of hyperthyroidism
  • auto-antibodies are generated that bind to the thyroid stimulating hormone receptor
  • unusual type II hypersensitivity disease
66
Q

What is systemic lupus erythematous?

A
  • prototypic multi-system autoimmune disease
  • rare type III hypersensitivity disease
  • increased risk of cardiovascular disease
  • high female preponderance
    strong genetic predisposition
67
Q

describe the pathogenesis of systemic lupus erythematous?

A
  • increased apoptosis; defective clearance of apoptotic material and dysregulation of neutrophil NETS
68
Q

What is the function of Th17 cells?

A
  • associated with defence against extracellular bacteria and fungi as well as the development of autoimmune diseases
  • they are highly pro-inflammatory

The respiratory system (13 decks)

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