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My Year 5 Pathology > Immunology > Flashcards

Immunology Flashcards

1
Q

What are 3 examples of live attenuated vaccines?

A

MMR
BCG
typhoid

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2
Q

Why was the clinical trial for cancer vaccine halted?

A

Development of meningocephalitis

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3
Q

What can interferon alpha be used for?

A

Hep a
Hep b
Kaposi’s sarcoma

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4
Q

What receptor does ipilimumab act on?

A

CTLA4

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5
Q

What is the problem with plasmapheresis?

A

Rebound antibody production limits efficacy therefore usually given with anti proliferative agent

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6
Q

Give the 3 phases of transplant rejection

A

1- recognition of antigen
2-activation of antigen specific lymphocytes
3- effector phase

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7
Q

Give 3 risk factors for chronic rejection of a transplant

A

1- multiple acute rejections
2- HTN
3- hyperlipidaemia

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8
Q

Which types of graft rejection does not respond to immunosuppressants

A

Chronic

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9
Q

Which types of graft rejection causes fibrosis

A

Chronic

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10
Q

What does HIV virus bind to during attachment?

A

Gp120 and gp41 on CD4 T cells

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11
Q

How is HIV diagnosed?

A

Screening - detects anti-HIV ab via ELISA

Confirmation - detects ab via western blot 
Requires seroconversion (after 10 weeks)
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12
Q

How long is the incubation period of HIV before seroconversion

A

10 weeks

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13
Q

What is the most common anti Retrovir HIV drug in pregnancy?

A

Zidovudine - antelartum PO

Delivery IV Po to newborn for 6/52

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14
Q

What does Zidovudine treatment antenatally decease transmission by?

A

26%-8%

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15
Q

which immune cells are the basis of innate immunity?

A

neutrophils
macrophages
NK cells

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16
Q

how common is primary immunodeficiency?

A

very rare: 1/10,000 live births

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17
Q

give 3 drugs which cause secondary immunodeficiencies

A
  1. corticosteroids
  2. cytotoxic agents
  3. antiproliferative immunosuppressants
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18
Q

which 3 malignancies cause secondary immunosuppression

A
  1. myeloma
  2. leukaemia
  3. lymphoma
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19
Q

give 3 infections causeing secondary immunosuppression

A
  1. HIV
  2. Measles
  3. Mycobacterial infection
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20
Q

what are 6 clinical features that are suggestive of immunodeficiency?

A
  1. two major or one major and recurring minor infections in one year
  2. unusual organisms
  3. unusual sites
  4. unresponsive to treatment
  5. chronic infections
  6. early structural damage
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21
Q

which antibody is the major antibody produced in mucosal secretions?

A

IgA

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22
Q

what immunodeficiency is associated with redcurrant respiratory and GIT infections (in 30% )

clue: mucous membranes

A

IgA deficiency

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23
Q

How many caucasian people are affected by complete IgA deficiency?

A

1 in 600

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24
Q

What pattern recognition receptors on some immune cells recognise?

A

PAMPS - pattern associated molecular patterns

e.g. bacterial sugars, DNA, RNA

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25
Q

What are Fc receptors for?

A

To allow immune cells to detect immune complexes

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26
Q

What is the target for IgG antibodies wen protecting against influenza virus?

A

Hemagluttinin (HA)

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27
Q

What is the purpose of a depot adjuvant?

A

Main aim os to increase the immune response without altering its specificity - by promoting antigenic persistance by slowing the release of antigen to be a steady stream

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28
Q

what accumulates in Alzheimers disease?

A

amyloid and tau proteins (plaques and tangles)

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29
Q

define atopy

A

the production of specific IgE responses to common environmental antigens

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30
Q

what is the prevalence of allergic rhinitis in adults?

A

20%

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31
Q

what is the prevalence of atopic dermatitis in adults

A

5%

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32
Q

What are clinical features associated with IgE mediated allergy?

A

angioedema, prurirus, urticaria, rhinitis, conjunctivitis, D+V, anaphylaxis

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33
Q

what are 3 investigations you could do to test an allergy?

A
  • skin prick test
  • lab measurement of allergen specific IgE
  • patch test (48h delayed response)
  • challenge test (supervised test)
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34
Q

what is the gold standard investigation for allergy?

A

skin prick test

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35
Q

what are 3 disadvantages of skin prick tests

A
  • risk of anaphylaxis (1 in 3000)
  • limited value in patients with skin conditions such as eczema
  • requires expert to interpret
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36
Q

what sort of allergy testing would be indicated for a patient with extensive eczema

A

specific serum IgE testing

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37
Q

What type of allergy testing would you avoid, and which would you choose in a patient with a history of anaphylaxi?

A

avoid skin prick test,

chose specific IgE serum testing

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38
Q

what test would you do if a patient suddenly becomes hypotensive and gets a severe rash during anaesthesia?

A

serum mast cell tryptase - it’s a product of degranulated mast cells and so helps diagnose anaphylaxis

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39
Q

what is the gold standard for diagnosing food allergy

A

challenge test

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40
Q

A 18 years old lady with a history extensive eczema who is unable to stop taking anti-histamine medication. What is the most appropriate test to look for IgE sensitisation?

A

IgE serum test (or RAST test)

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41
Q

A 15 year old with a history of asthma and hayfever who notices increasing cough and wheeze shortly after eating peanuts. What is the most appropriate initial diagnostic test ?

A

component resolve diagnostic (CRD) or IgE serum

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42
Q

what is the management of anaphylaxis

A
  • IM adrenaline 500ug
  • Oxygen 100%
  • inhaled bronchodilators
  • fluid replacement
  • hydrocortisone 100mg IV
  • chlorpheniramine 10mg IV (skin rash)
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43
Q

what is the most common food allergy in adults (in the USA)

A

shellfish (2%)

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44
Q

A 35 year old man with a history of spring time hayfever and immediate lip tingling and swelling immediately after eating and apples.

a) What is the most likely explanation for IgE hypersensitivity ?
b) How would you confirm the diagnosis?
c) What tests may be useful to advise on prognosis?

A

a) birch pollen oral allergy syndrome (OAS)
b) with a skin prick test or RAST
c) CRD (component resolve diagnostics) - can predict severity of reaction

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45
Q

what drugs are a common cause of angioedema, which may be misleading in a doagnosis of anaphylaxis?

A

ACE inhibitors

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46
Q

What type of disease is Familial mediterranean fever?

A

Monogenic auto-inflammatory disease

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47
Q

what is the inheritance pattern of familial mediterranean fever?

A

autosomal recessive

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48
Q

what disease is a result of a mutation in the MEFV gene which codes for pyrin-marenostrin?

A

familial mediterranean fever

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49
Q

What is the main difference in the mutations associated with monogenic autoimmune diseases, and monogenic autoinflammatory diseases?

A

Auto-Inflammatory = innate immune response

auto-immune = adaptive immune response

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50
Q

what monogenic auto-immune disease is characterised by a Single gene mutation involving FOXp3 resulting in abnormality of T reg cells

A

IPEX (immune dysregulation, polyendocrinopathy, enteropathy, X linked)

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51
Q

Name the polygenic auto-inflammatory disease. ~30% patients have a mutation of CARD15 which may affect response of myeloid cells to bacteria.

A

Chron’s disease

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52
Q

Name a mixed pattern auto-inflammatory / auto-immune disease with >90% heritability that results in inflammation typically involving the sacro-iliac joints and responds to TNF alpha antagonists

A

Ankylosing spondylitis

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53
Q

which 2 HLA genetic polymorphisms are responsible for a 25 fold increased risk of type 1 diabetes?

A

HLA-DR3/DR4

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54
Q

What type of hypersensitivity reaction is Goodpasture’s diisease?

A

Type II - antibody driven. The autoantigen is the Basement membrane collagen type 4 - leading to glomuronephritis and pulmonary haemorrhage

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55
Q

which hypersensitivity reaction type is rheumatoid arthritis?

A

type III - immune complex driven autoimmune disease, due to the Fc region of IgG causing arthritis

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56
Q

What type of hypersensitivity reaction is SLE?

A

Type III - immune complex formation

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57
Q

What type of hypersensitivity reaction is eczema

A

Type 1

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58
Q

which antibodies stimulate the TSH receptor in Graves disease?

A

IgG

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59
Q

What is the most common cause of hypothyroidism in iodine replete areas?

A

Hashimoto’s thyroiditis

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60
Q

Why is Hashimoto’s thyroiditis asociated with goitre?

A

because T and B cells infiltrate it and enlarge it

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61
Q

What antibodies is Hashimoto’s thyroiditis associated with?

A

anti thyroid peroxidase antibodies

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62
Q

describe the involvement of T cells in T1 diabetes?

A

CD8 cytotoxic T cells recognise auto-antigens presented by MHC class I molecules on the insulin producing beta cells

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63
Q

What type of hypersensitivity reaction is Grave’s?

A

Type II

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64
Q

what is the pathology of pernicious anaemia?

A

antibodies against gastric parietal cells or intrinsic factor, preventing B12 binding and absorption, leading to B12 deficiency, macrocytic anaemia, subacute degeneration of cord leading to peripheral and optic neuropathy

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65
Q

What antibodies are involved in myaesthenia gravis?

A

antibodies against Acetylcholine receptors

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66
Q

What is the likely diagnosis in a patient with muscle weakness which gets worse on repetetive movements, drooping eye and worse at the end of the day?

A

Myaesthenia gravis

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67
Q

what 2 investigations can confirm the diagnosis of Myaesthenia gravis?

A
  1. ant-Ach receptor antibodies
  2. EMG (elecromyogram)
  3. Tensilon test + (inject edrophonium (anti-cholinesterase_ which prolongs the life of Ach and allows it to access residual receptors
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68
Q

Give the autoantibody seen in:

a) Goodpasture disease
b) Myaesthenia gravis
c) Graves disease

D) Pernicious anaemia

E) Diabetes Mellitus

f) Hashimoto’s thyroiditis

A

a) anti-basement membrane antibody
b) anti acetylcholine receptor antibody
c) Anti TSH receptor antibody
d) anti-intrinsic factor antibody
e) Anti GAD/ anti-insulin / anti-islet cells / anti-IA-2
f) Anti-thyroglobulin, anti thyroid peroxidase antibodies

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69
Q

polymorphisms affecting which inflammatory cytokines increases the risk of RA?

A

TNF, IL1, IL6, IL10

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70
Q

what environmentla factors are associated with RA?

A
  1. smoking (due to increased citrullination)

2. GUM infection with Porphyromonas gingivalis

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71
Q

Why are polymorphisms in PAD (Peptidylarginine deiminases) type 2 and 4 associated with rheumatoid arthritis?

A

because polymorphisms are associated with increased citrullination, which in turn creates a high load of citrullinated proteins - Th cells then promote development of an antibody response

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72
Q

what antibodies are associated with rheumatoid arthritis

A

anti-CCP antibodies

rheumatoid factor

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73
Q

What is a rheumatoid factor?

A

An antibody directed against the common (Fc) region of human IgG

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74
Q

What is the region of the HLA DR beta chain that predisposes to development of rheumatoid arthritis

A

the shared epitope

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75
Q

What antibody Binds to Fc region of IgG

A

Rheumatoid fator

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76
Q

What antibody Binds to citrullinated proteins and has ~95% specificity for development of rheumatoid arthritis

A

Anti-CCP or ACPA

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77
Q

What bacteria is known to expresse PADI enzymes capable of deiminating arginine to form citrullinated proteins

A

P. gingivalis (gum infection associated with increased risk of RA)

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78
Q

What does staining Hep-2 cells used to detect in patient serum?

A

anti-nuclear antibodies (ANA)

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79
Q

What is the immunopathology of SLE?

A
  1. antibodies bind to antigens, forming immune complexes, 2. imune complees deposi in the tissue
  2. immune complxes activate complement (classical pathway) resulting in increased inflammatory response
  3. immune complexes stimulate cells expressing Fc and complement receptors
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80
Q

What are the main targets of ANA in SLE?

A

dsDNA

extractable nuclear antigens (ENAs) e.g. ribonucleoproteins such as Ro and La, enzymes

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81
Q

Which 2 tests are used to diagnose antiphospholipid syndrome?

A
  1. anti-cardiolipin antibody

2. lupus anti-coagulant

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82
Q

what are the features of CREST syndrome?

A 
Calcinosis 
Rayneuds 
Eosophageal dysmotility 
Sclerodactyly 
Telangectsia
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83
Q

How are diffuse and limited systemic sclerosis different in terms of anatomy?

A 
limited = not beyond forearms (can sometimes include oral mucosa) 
diffuse = beyond forearms
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84
Q

what antibodies are involved in a) limited and b) diffuse cutaneous systemic sclerosis?

A

a) limited = anti-centromere antibodies

b) diffuse = anti-topoisomerase antibodies (Scl-70)

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85
Q

What are donors of immunoglobulin screened for before donation?

A

Hep B, Hep C, HIV

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86
Q

what are pembrolizumab and nivolumab antibodies specific for?

A

PD-1

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87
Q

what do corticosteroids inhibit, reducing inflammation?

A

Phospholipase A2, which blocks formation of arachidonic acid and therefore stops prostaglandin formation

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88
Q

Give some side effects of corticosteroids?

A

cushings, hirsutism, central obestiy, diabetes, pancreatitis, transient neutrophilia, glaucoma, osteoporosis, acne, HTN, peptic ulceration, AVN

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89
Q

Name the 4 cytotoxic antiproliferative agents?

A 
CAMM
Cyclophosphamide
Azathioprine 
Mycophenolate Mofetil 
Methotrexate
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90
Q

which cytotoxic anti-proliferative drug is metabolised into 6-mercaptopurine by the liver?

A

Azathioprine

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91
Q

which cytotoxic drug cannot be metabolised by people with a TPMT mutation?

A

Azathioprine

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92
Q

What are general toxic/Side effects of cytotoxic anti-proliferative drugs?

A

infection, teratogenic, BM suppression, malignancy, hepatotoxicity

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93
Q

what is the mechanism of action of cyclophosphamide (cytotoxic agent)

A

alkylation of guanine base of DNA, damaging it and preventing cell replication

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94
Q

which cytotoxic drug is indicated for Chrons or UC?

A

azathioprine

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95
Q

which inhibitor of cell signalling is known for the unwanted side effect of gingival gum hypertrophy?

A

Cyclosporin

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96
Q

which 2 immunosuppressive drugs work by inhibiting calcineurin?

A

cyclosporin + tacrolimus

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97
Q

What are the 5 immunosuppressive drugs which work by inhibiting cell signalling?

A 
TACTS - tactics for stopping cell signalling 
Tacrolimus 
Apremilast 
Cyclosporin
Tofactinib 
Sirolimus
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98
Q

What does tofactinib inhibit, and what condition is it used to treat??

A

JAK
Rheumatoid Arthritis

(Jack SB has RA and is eating tofu with a pen nib)

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99
Q

What does Apremilast inhibit and what can it be used to treat?

A

PDE4 inhibitor
Works for psoriasis and psoriatic arthritis
Apres ski me last time because I did a bit too much PDeffection4 and I had scaley psoriasis

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100
Q

which cytotoxic drug is associated with infection, in particular PML (progressive multifocal leukoencephalopathy?)

A

mycophenolate mofetil

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101
Q

which cytotoxic drug is associated with infertility?

A

cyclophosphamide

102
Q

what is basiluximab targeted against?

A

anti-CD25

103
Q

What is rituximab targeted against?

A

anti-CD20

104
Q

What is infliximab antibody against?

A

TNF-alpha

105
Q

give some indications for use of anti TNF antibodies

A

RA, psoriasis, ankylosing spondylitis, IBD,

106
Q

What is denosumab antibody directed agaist?

A

RANK ligand

107
Q

what is an indication for Denosumab?

A

Osteoporosis - as it inhibits RANK mdiated osteoclast differentition and function

108
Q

what deficiencies are seen in coeliac disease?

A

iron, B12, calcium, folate, fat

109
Q

what 2 antibodies can you test for coeliac?

A

IgA anti-transglutaminase antibody,

IgA anti - endomysial antibody

110
Q

What features on a histological report would you expect from a duodenal biopsy which confirms your suspicion of coeliac disease?

A
  • vilous atrophy (reduced villous ratio)
  • increased intraepithelial lymphocytes
  • crypt hyperplasia
  • increased inflammatory cells in the lamina propria
  • no evience of giardia
111
Q

Which infectious disease can often give a similar picture to coeliac disease?

A

Girdiasis

112
Q

what are the complications of coeliacs?

A
  • malabsorption
  • osteoporosis / osteomalacia (so need regular bone scans)
  • neurological disease e.g. epilepsy, cerebral calcification, lymphoma, hyposplenism
113
Q

what is the genetic component for coeliacs?

A

HLA-DQ2

114
Q

What support should you offer patients diagnosed with coeliac diease?-

A
  • ptient support groups eg. coeliac.co.uk
  • dieticians
  • medical staff
  • family
  • reassure and recognise that adjusting to a chronic meical condition can be a
115
Q

In a follow up visit with a patient diagnosed with Coeliacs, you discover the anti-TTG antibody is still positive. What 2 things could cause this?

A
  1. non compliance with non gluten diet

2. lymphoma

116
Q

what conditions is coeliac disease associated with?

A 
- dermatitis herpetiformis 
(100%prevalence) 
- T1DM 
- AI thyroid disease 
- Downs syndrome
117
Q

what are the steps in the life cycle of HIV infection?

A
  1. Attachment/entry
  2. Reverse transcription of Viral RNA into DNA
  3. integration into host DNA
  4. Transcription of viral proteins
  5. viral protein synthesis
  6. virus assembly & release
  7. maturation
118
Q

What types of drug is HAART composed of?

A 
2X NRTI
1X PI (or NNRTI)
119
Q

What is the median time from infection with HIV to the development of AIDS?

A

8-10 years

120
Q

What % of HIV patientd are LTNP (long term non progressors)

A

<5%

121
Q

What is the screening test for HIV?

A

ELISA test for anti-HIV antibodies

122
Q

What is the confirmatory test for HIV?

A

Western blot anti-HIV antibody

123
Q

What is the ‘window period’ in HIV patients?

A

The period where a person is HIV infected, but tests negative on the ELISA test due to low levels of anti-HIV antobodies. Most people are detectable in 2-6 weeks.

124
Q

what are the 2 ways for testing HIV-1 resistance?

A
  1. phenotypic (viral replication measured in cell cultures with increasing concs of antiretrovirals, compared to WT)
  2. genotypic - mutations determined by sequencing of amplified HIV genome
125
Q

give an example of an NNRTI

A

efavirenz

126
Q

What are examples of nucleotide RTIs?

A

truvada

tenofovir

127
Q

what type of drug is zidovudine?

A

nucleoside RTI

128
Q

What type of hypersensitivity response is anaphylaxis?

A

Type I

129
Q

What are the clinical features of anaphylaxis? (should be able to say 10)

A

angioedema of lips/mucous membranes/eyes, laryngeal obstruction/stridor, bronchoconstriction/wheeze, flushing, urticaria, pruritus, hypotension, dizziness, syncope,cardiac arrythmias, MI, D+V, abdo pain.

130
Q

what are the 2 most common clinical manifestations of anaphylaxis?

A

urticaria and angioedema

131
Q

What is the immediate treatment of anaphylaxis?

A

ABCD - A = airway may be closed - bronchoconstriction so may need to intubate, or if laryngeal/tongue swelling may need to do tracheostomy

132
Q

What is the purpose of IM adrenaline in anaphylaxis?

A

acts on B2 adrenergic receptors to constrict arterial smooth muscle - increases BP, limits vascular leakage and is a bronchodilator

133
Q

what dose of IM adrenaline should you give adults

A

0.5mg

134
Q

what is it important togive to prevent rebound anaphylaxis?

A

IV corticosteroids (hydrocortisone 200mgs)

135
Q

What might an allergy to bananas indicate?

A

An allergy to chitinase containing foods and potentially cross-reactivity with latex

136
Q

What is the purpose of testing CH50?

A

testing the integrity of the classical complement cascade

137
Q

name the components in the classical complement pathway

A

C1 C4 C2

138
Q

what should you always test in patients with spordic and recurrant meningococcal disease?

A

complement deficiency - so test AP50 and CH50

AP and C Hall do not complement eachother when they’re 50

139
Q

what is the gel and coombs classification of hypersensitivity? examples of conditions for each

A 
I = allergy: anaphylaxis, atopy, asthma - IgE mediated
II = cytotoxic antibodies,  Graves, Myaesthenia gravis, goodpastures 
III = immune complex mediated: RA, SLE, post streptococcal glomerulonephritis - IgG 
IV = delayed antibody independent - e.g Hashimotos, contact dermatitis, coeliac disease, memory T cell response
140
Q

which 4 infections can you give a specific immunoglobulin as PEP?

A

rabies, VZV, tetanus, Hep B

141
Q

what is basiliximab targeted against, and what does it treat?

A

CD-25, inhibits T cell proliferation, treats allograft rejection
(basil helps stop graft rejections when youre 25)

142
Q

what is rituximab targeted against, and what isit used to treat?

A

CD-20 - lymphoma, RA, SLE

Wear a tux when you’re 20

143
Q

what is abatacept targeted against and what is it used to treat?

A

anti-CTLA4-Ig reduces T cell activation, used in RA

Bat Cat Rat

144
Q

What is Natalizumab targeted against and what is it used to treat?

A

anti-alpha4 integrin (inhibits T cell migration) - treats Cron’s and relapsing remitting MS

(natalie drives on the A4 with a grin because it’s cured her MS and Chrons)

145
Q

what conditions do anti-TNF alpha monoclonal antibodies treat?

A

e.g. infliximab - (remember netflix TNF)

treats - psoriasis, psoriatic arthritis, inflammatory bowel disease, RA, ankylosing spondylitis

146
Q

which monoclonal antibody targets TNFalpha/TNFbeta receptor p75-IgG fusion protein

A

Etanercept (tanning requires A + B up to 75)

147
Q

what monoclonal antibody can be used to treat osteoporosis and s targeted against RANK ligand antibody?

A

Denosumab (a den made of rank bones, drug to keep it standing)

148
Q

what polymorphism renders people unable to metabolise azathioprine?

A

TPMT

149
Q

what would be your top differentials if the lab reports ENA+ Ro, La, Sm, RNP

A

SLE or Sjogrens

150
Q

what would be your top differential is the lab reported SCL70, RNA Pol, Fibrillarin

A

Diffuse cutaneous scleroderma

151
Q

what are anti-centromere antibodies suggestive of?

A

limited cutaneous scleroderma

152
Q

what must you check before prescribing azathioprine?

A

check TPMT status

153
Q

what does allopurinol inhibt?

A

xanthine oxidase

154
Q

what are the 4 TNF alpha inhibitors?

A

infliximab, adalimumab, certolizumab, golimumab (adam and gollum watching netflix for certain )

155
Q

Why should you not use anti-TNF alpha drugs to treat SLE?

A

It can precipitate cutaneous lupus

156
Q

describe the pathology of serum sickness?

A

penicillin can bind to cell surface proteins, acting as a neo-antigen- stimulating a strong IgG antibody response. This person then becomes ‘sensitised’ to penicillin. Next exposure, stimulates formatio of immune complexes of penicillin and IgG which accumulate in microvasculature of kidneys, joints and skin - resulting in the sequalea of AKI, purpuric vasculitis and arthralgia

157
Q

why does renal function deterorate in serum sickness?

A

Immune complexes deposit in glomeruli, activating complement and causing infiltration of macrophages and neutrophils. This results in inflammation of the glomeruli, causing raised creatinine, proteinuria ad haematuria

158
Q

Why might a person become disorientated in serum sickness?

A

small vessel vasculitis affecting cerebral vessels may compromise oxygen delivery to the brain

159
Q

why can purpura develop in serum sickness

A

immune complex deposition in small vessels causes inflammation, whch can cause leakage and local haemorrhage, also become plugges with clots which further compromises oxygen delivery

160
Q

What is the management of a patient with serum sickness?

A

stop penicillin immediately
decrease systemic inflammation (corticosteroids)
ensure appropriate fluid balance

161
Q

what is the treatment for X linked agammaglobulinaemia

A

IV immunoglobulin replacement therapy (IvIg) every 3-4 weeks for life.

162
Q

what is the most likely diagnosis in a young patient suffering from recurring respiratory infections, and a quantitive test reveals absence of B cells?

A

Bruton’s X linked agammaglobulinaemia

163
Q

why does multiple myeloma commonly present in recurrent infections

A

because it is characterised by neoplastic proliferation of plasma cells, resulting in massive expansion of a single type of immunoglobulin with a single specificity. This suppresses the production of normal immunoglobulins, resulting in functional antibody deficiency aka immune paresis

164
Q

what are the 2 main reaosns for anaemia in multiple myeloma?

A

1) space limitation - the malignant plasma cell expansion crowds out normal precursors of RBCs
2) inhibitors - tumour may produce cytokines which inhibit normal BM function

165
Q

why does rheumatoid arthritis commonly present post-partum?

A

might be due to the swich in T cell profiles - during pregnancy Th2 cells dominate, whereas switch to Th1 after delivery

166
Q

which specific subtypes of HLA-DR4 are specifically associated with RA?

A

Dw4 Dw14 Dw15

167
Q

What is first line treatment for RA?

A

methotrexate if not tolerates, sulphasalazine, hydrochloroquine, leflunomide,

168
Q

Kostmann’s syndrome is a congenital deficiency of what component of the immune system?

A

Neutrophils

169
Q

Produced by the liver, when triggered, enzymatically activate other proteins in a biological cascade and are important in innate and antibody mediated immune response?

A

complement

170
Q

Which component of the innate immune system is usually one of the first to respond to infection through a cut?

A

neutrophils

171
Q

Which infection is most common as a consequence of B cell deficiency?

A

Bacterial

172
Q

Meningococcal infections are quite common as a result of which deficiency of the component of the immune system?

A

complement

173
Q

94% of angioedema cases presenting to A&E are drug induced, what s the most common drug class?

A

ACE inhibitors

174
Q

What is the treatment for allergic rhinitis?

A

oral antihistamines and intranasal corticosteroids

175
Q

which MHC class is associated with Th1 cells?

A

MHC II

176
Q

What acts on hepatocytes to induce synthesis of acute phase proteins in response to bacterial infection

A

IL 6

177
Q

which MHC is associated with cytotoxic T cells

A

MHC I

178
Q

which MHC is associated with cytotoxic T cells

A

MHC I

179
Q

The most abundant (in terms of g/L) immunoglobulin in normal plasma is…

A

IgG

180
Q

A complete deficiency in this molecule is associated with recurrent respiratory and gastrointestinal infections.

A

IgA

181
Q

Leukocyte Adhesion Deficiency is characterised by a very high count in which immune cell ?

A

neutrophils

182
Q

Which crucial enzyme is vital for the oxidative killing of intracellular micro-organisms?

A

NAPDH oxidoase

183
Q

Which complement factor is an important chemotaxic agent?

A

C3a

184
Q

What is the functional complement test used to investigate the classical pathway?

A

CH50

185
Q

What type of HS reaction is ABO hemolytic transfusion reaction

A

Type II - antibody mediated

186
Q

What is the single most useful antibody test to diagnose SLE?

A

anti-dsDNA antibody

187
Q

What is the single most useful antibody test to diagnose Wegener’s granlomatosis?

A

c-ANCA

188
Q

What is the single most useful antibody test to diagnose Primary biliary cirrhosis

A

anti-mitochondrial antibody

189
Q

What does p-ANCA diagnose?

A

polyarteritis nodosa

190
Q

What do p-ANCA and c-ANCA each bind to?

A

p-ANCA = myeloeroxidase

c-ANCA = PR3 (proteinase 3)

191
Q

in SLE what is the classic pattern of ESR and CRP

A

raised ESR and normal CRP

192
Q

What is the specific auto-antigen that is the target of the immune system in Goodpasture’s?

A

Type IV collagen (in the Basement membrane)

193
Q

The pattern of the antibody deposition in the glomerular basement membrane is typically described as what?

A

smooth linear pattern

194
Q

The pattern of the antibody deposition in the glomerular basement membrane is typically described as what?

A

smooth linear pattern

195
Q

What is the definition of a secondary lympoid organ?

A

anatomical sites of interaction between microorganisma and naive lymphocytes

196
Q

what are examples of secondary lymphid organs

A

spleen, mucosal associated lymphoid tissue (MALT), lymph nodes

197
Q

which type of T cell are particularly important in defence against viral infections and tumours

A

CD8+ cytotoxic T cells

198
Q

How do cytotoxic T cells kill cells directly?

A

perforin and granzymes, and Fas ligand expression induces apoptosis

199
Q

in B cell memory, which immunoglobulin dominated the secondary response to an antigen?

A

IgG

200
Q

in SCID, what protects the neonate from infection in the 1st 3 months of life?

A

Circulating maternal IgG from the maternal placenta/milk/colostrum

201
Q

what is the syndrome involving 22q11.2 deletion?

A

DiGeorge’s syndrome

202
Q

what are the features associated with DiGeorge’s syndrome

A

high forehead, low set abnormally folded ears, cleft palate, small mouth and jaw, congenital heart disease, oesophageal atresia, hypocalcaemia, T cll lymphopenia

203
Q

Which cells are absent in DiGeorge’s syndrome?

A

T cells - as the thymus is absent

204
Q

what is the definitive treatment for DiGeorges syndrome?

A

thymus transplant

205
Q

what immune deficiency is caused by a deficiency in regulatory factor X or class II transactivator?

A

Bare lymphocyte syndrome

206
Q

what type of cells are deficient in Bare Lymphocyte syndrome type I (BLS I)

A

affects MHC I so CD8+ cells

207
Q

What type of cells are deficient in bare lymphocyte syndrome type II?

A

Affects MHC II so low CD4+ cells

208
Q

What type of cells are deficient in bare lymphocyte syndrome type II?

A

Affects MHC II so low CD4+ cells

209
Q

Which type of BLS is more common (I or II) and so which cells are low?

A

BLS II is more common, so low CD4+ cells

210
Q

which immunoglobulins are extremely deficient in BLS and why?

A

IgG and IgA - because CD4+ is low and you need it for the B cell class switch

211
Q

what does IL-12 stimulate T cells to do

A

release IFN-gamma

212
Q

List the immunodeficiencies involving T cells

A
  • SCID
  • DiGeorge’s
  • Bare Lymphocyte Syndrome
  • IL-12 (r) deficiency
  • IFN-gamma (r) deficiency
213
Q

Severe recurrent infections from 3 months,CD4 and CD8 T cells absent, B cell present but immature phenotype, some IgM present, IgA and IgG absent. Normal facial features and cardiac echocardiogram

A

X linked SCID

214
Q

Recurrent infections in childhood, abnormal facial features, congenital heart disease, normal B cells, low T cells, normal IgM, borderline low IgA and IgG

A

DiGeorge Syndrome

215
Q

6 month baby with two recent serious bacterial infections. T cells present – but only CD8+ population. B cells present. IgM present but IgG absent

A

Bare lymphocyte syndrome type II

216
Q

what is the mutation in Bruton’s x-linked agammaglobulinaemai?

A

X linked tyrosine kinase defect

217
Q

What immunoglobulins do naive B cells produce before antibody class switching occurs?

A

IgM and IgD

218
Q

what immunodeficiency syndrome is caused by a mutation in the CD40 ligand?

A

Hyper IgM syndrome

219
Q

why is there such high levels of IgM in hyper IgM syndrome

A

The mutation in CD40 ligand prevents T-B cell crosstalk and therefore antibody class switching, therefore IgMs which are prodced cannot change into IgA, IgE or IgG so they are deficient and IgM becomes really high.

220
Q

what deficiency is asymptomatic in 70% of people, but can present with recurrnt respiratory and gastric infections?

A

IgA deficiency

221
Q

Name all of the B cell immunodeficiencies

A
  • Bruton’s agammaglobulinaemia
  • Hyper IgM syndrome
  • CVID (common variable immune deficiency)
  • Selective IgA deficiency
222
Q

Recurrent respiratory tract infections, absent IgA, normal IgM and IgG

A

selective IgA deficiency

223
Q

Recurrent bacterial infections in a child, episode of pneumocystis pneumonia, T and B cells present, high IgM, absent IgA and IgG - what is the diagnosis and where is the mutation?

A

X linked hyper IgM syndrome - mutation in T cell expression of CD40 ligand

224
Q

1 year old boy. Recurrent bacterial infections. CD4 and CD8 T cells present. B cells absent, IgG, IgA, IgM absent

A

Bruton’s X linked agammaglobulinaemia

225
Q

What type of B cell immunodeficiency is this: Adult with bronchiectasis, recurrent sinusitis and development of atypical SLE

A

commmon variable immuodeficiency

226
Q

Which type B immunodeficiency is associated with Pneumocystis carnii infection?

A

Hyper IgM syndrome

227
Q

Which type B immunodeficiency is associated with Pneumocystis carnii infection?

A

Hyper IgM syndrome

228
Q

which receptor is mutated in Severe combined immunodeficiency?

A

IL-2 receptor, causing early arrest of T cell and NK cell development.

229
Q

A 25 year old woman comes to her GP about family planning. She is worried because she had an older brother who died before she was born and her grandmother lost two children which she things were both boys. Her GO thinks there may be a genetic disorder in her family affecting the IL-2 receptor. If correct she has a 50% of inheriting the trait from her mother and being a carrier herself. And there would be a 50% chance of passing it to her children. If inherited, her daughters would be carriers and her sons would require treatment which is usually a bone marrow transplant but gene therapy is sometimes used

A

Severe combined immunodeficiency (SCID)

230
Q

A jaundiced 8 month old child presents with failure to thrive, and a history of recurrent infections (viral, bacterial and fungal). On examination there is hepatomegally and blood tests show a raised alk phos and low CD4 count. A defect is found in the proteins that regulate MHC Class II transcription.

A

Bare lymphocyte syndrome (II)

231
Q

In acute rejection, release of this substance from CD8+ lymphocytes helps kill target cells

A

granzyme B

232
Q

In acute rejection, these are produced as a result of the activation of neutrophils and macrophages

A

TNF alpha, free radicals, NAPDH oxidase

233
Q

What are The 3 most important HLA types to screen for in renal transplantation when matching donor and recipient, in order of importance

A

HLA DR > B > A

234
Q

Treatment of a) acute antibody mediated rejection and b) cell mediated rejection

A

a) plasmapheresis and IV immunoglobulin therapy

b) high dose corticosteroids

235
Q

lymphocyte that responds to foreign HLA DR types =

A

CD4+ T cells

236
Q

lymphocyte that responds to foreign HLA A types =

A

CD8+ T ells

237
Q

which immmunosuppressant can cause gingival hypertrophy as a side effect?

A

ciclosporin

238
Q

with the drugs mycophenolate mofetil and cyclophosphamide, which cells are they each more specific to?

A

Mycophenolate mofetil = T cells

cyclophosphamide = B cells (although at high doses will affect all cells with a high turnover)

239
Q

What is The 3 most important HLA types to screen for in renal transplantation when matching donor and recipient, in order of importance

A

bee/wasp venom allergy

240
Q

What are important side effects of ciclosporin?

A

gingival hypertrophy, hypertension, reduced GFR

241
Q

What are important side effects of azathioprine?

A

bone marrow suppression (anaemia), N+V

242
Q

What are important side effects of methotrexate?

A

cirrhosis, pulmonary fibrosis, pneumonitis

243
Q

Which live attenuated organism do BCG vaccines contain?

A

mycobacterium bovis

244
Q

Which vaccination contains extracts of or detoxified exotoxin product by a microrganism

A

tetanus vaccine

245
Q

give a common vaccine that is made by recombinant DNA technology

A

Hepatitis B vaccine

246
Q

An antigen assembled in a multimeric form and saponin that provokes a strong serum antibody response.

A

immunostimulatory complexes (ISCOMS)

247
Q

The general name for a compound which increases the immune response without altering its specificity.

A

adjuvant

248
Q

Name 4 live attenuated vaccines

A

remember MMR BOY

MMR
BCG
Oral Polio
Yellow Fever

249
Q

Which conjugate vaccine is normally given to infants under the age of 13 months in the form of three doses at monthly intervals to protect against an infection that has symptoms similar to meningitis and predominantly occurs in children < 5 years?

A

Hib

250
Q

Agent used in humans that promotes a predominantly antibody response through the release of Il-4 that primes naïve B-cells.

A

Alum

251
Q

Which vaccine is given to the mother to prevent congenital cardiac defects, eye lesions (particularly cataracts), microcephaly, mental handicap and deafness of her newborn baby?

A

rubella

My Year 5 Pathology (5 decks)

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