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Path - Sarah > Immunology > Flashcards

Immunology Flashcards

1
Q

Which immune deficiency is this?CD4: lowCD8: lowB cells: normal or lowIgM: normal or lowIgG: lowIgA: low

A

SCID

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2
Q

Which immune deficiency is this?CD4: lowCD8: lowB cells: normalIgM: normalIgG: low

A

DiGeorge

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3
Q

Which immune deficiency is this?CD4: lowCD8: normalB cells: normalIgM: normalIgG: low

A

BLW

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4
Q

Which immune deficiency is this?CD4: normalCD8: normalB cells: lowIgM: lowIgG: lowIgA: low

A

Bruton’s

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5
Q

Which immune deficiency is this?CD4: normalCD8: normalB cells: normalIgM: highIgG: lowIgA: low

A

HyperIgM

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6
Q

Which immune deficiency is this?CD4: normalCD8: normalB cells: normalIgM: normalIgG: normalIgA: low

A

Selective IgA deficiency

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7
Q

Which immune deficiency is this?CD4: normalCD8: normalB cells: normalIgM: normal or lowIgG: lowIgA: low

A

CVID

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8
Q

Which immune deficiency is this?Neutrophil count - absentLeukocyte adhesion markers - normalNitroblue test of oxidative killing - absentPus - no

A

Kostmann syndrome (congenital neutropenia)

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9
Q

Which immune deficiency is this?Neutrophil count - normalLeukocyte adhesion markers - normalNitroblue test of oxidative killing - abnormalPus - yes

A

Chronic granulomatous disease

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10
Q

Which immune deficiency is this?Neutrophil count - increased during infectionLeukocyte adhesion markers - absentNitroblue test of oxidative killing - normalPus - no

A

Leukocyte adhesion deficiency

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11
Q

What are the consequences of T cell deficiencies?

A

Increased susceptibility to viral, fungal and some bacterial infections. Early malignancy.

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12
Q

What is the treatment for T Cell deficiencies?

A

Infection prophylaxisIg replacement if necessarySpecific other treatments

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13
Q

What is bare lymphocyte syndrome?

A

Defect of regulatory factor X or Blass II transactivator. Absent expression of HLA molecules within the thymus -> lymphocytes fail to develop.

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14
Q

What are the 2 types of Bare Lymphocyte Syndrome?

A

Type I - MCH I absent - low CD8 cellsType II - MCH II absent - low CD4 cells

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15
Q

Which type of bare lymphocyte syndrome is more common and what are its features?

A

Type 2 - profound deficiency of CD4 but normal CD8 and B cells. B cell class switch needs CD4 so less IgA and IgG are made.

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16
Q

What is bare lymphocyte syndrome associated with?

A

Sclerosing cholangitis

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17
Q

When do children with bare lymphocyte syndrome become unwell?

A

By 3 months of age

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18
Q

What is DiGeorge’s Syndrome?

A

Impaired development of the 3rd and 4th pharyngeal pouches (oesophagus, thymus, heart)

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19
Q

What ist he cause of DiGeorge’s Syndrome?

A

22q11.2 deletion - 75% are sporadic

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20
Q

What are the features of DiGeorge’s syndrome?

A

Low set ears, cleft lip and palateLow calciumSusceptible to viral infectionVery low numbers of mature T cells due to absent thymus

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21
Q

How is DiGeorge’s syndrome treated?

A

Thymus transplant

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22
Q

What are the consequences of B cell deficiencies?

A

Increased susceptibility to bacterial and some viral infections and toxins e.g. tetanus, diphtheria

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23
Q

What is the treatment for B cell deficiency?

A

Ig replacementBone marrow transplant in some situationsVaccination not effective except in IgA deficiency

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24
Q

What causes Bruton’s agammaglobulinaemia and what are its features?

A

X linked tyrosine kinase defectMutation in BTK geneFailed production of mature B cellsNo antibodies Symptoms after 3-6 months

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25
Q

What is the cause/defect in Hyper IgM syndrome?

A

X linked condition - Xq26CD40L, CD40, CICDA or CD154 defect

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26
Q

How does hyper IgM syndrome present?

A

Boys present in 1st year of life with recurrent bacterial infections especially pneumocystis carinii and failure to thrive

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27
Q

What is the pathophysiology of Hyper IgM Syndrome?

A

Activated T cells can’t interact with B cells to class switch, therefore B cells can’t make IgA and IgG but elevated IgM.Also less lymphoid tissue as no germinal centre development

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28
Q

What are the long term risks of Hyper IgM syndrome?

A

Autoimmunity and malignancy

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29
Q

What is the cause and defect of Common Variable Immune Deficiency?

A

Defect in B cell differentiation with many genetic causes.Low IgG, IgE and IgA

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30
Q

What are the features of common variable immune deficiency?

A

Failure to thriveRecurrent infectionsAutoimmunityGranulomatous diseases

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31
Q

What is the most common B cell deficiency?

A

Selective IgA deficiency

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32
Q

How common is selective IgA deficiency?

A

Affects 1 in 600 caucasians

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33
Q

What are the features of selective IgA deficiency?

A

70% asymptomatic but can cause recurrent gastrointestinal and respiratory infections

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34
Q

Where is IgA found?

A

Mucosal areas, saliva, tears, breast milk

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35
Q

Where is IgE found?

A

Allergy - histamine release from mast cells

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36
Q

What is special about IgG?

A

Can cross from the placenta to the foetus

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37
Q

Where is IgM found?

A

On the surface of B cells (immature B cells express only IgM)

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38
Q

What is the half life of human normal Ig?

A

18 days

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39
Q

What is the cause of severe combined immune deficiency?

A

Defects in lymphoid precursors e.g. adenosine deaminase gene, IL-2 receptor.45% are X linked

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40
Q

What are the features of SCID?

A

Low or normal B cell numbers, reduced T cells, low antibodiesRecurrent infections, failure to thrive, diarrhoea and early infant death

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41
Q

What is the treatment for SCID?

A

Bone marrow transplant the only established treatment

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42
Q

What is the consequence of phagocyte deficiency?

A

Increased susceptibility to bacterial and fungal infections often with deep abscesses

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43
Q

What is the main defect in Kostmann syndrome?

A

Severe congenital neutropenia

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44
Q

What is the cause of Kostmann syndrome?

A

Autosomal recessive - HAX1

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45
Q

How common is Kostmann syndrome?

A

1-2 cases per million

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46
Q

How does Kostmann syndrome present?

A

Recurrent infections shortly after birth

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47
Q

How is Kostmann syndrome diagnosed?

A

Chronically low neutrophils and bone marrow test showing an arrest of neutrophil precursor maturation

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48
Q

What is the treatment for Kostmann syndrome?

A

G-CSF, prophylactic antibiotics, BMT if G-CSF ineffective

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49
Q

What is the main defect in leukocyte adhesion deficiency?

A

Failure to express leukocyte adhesion markers

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50
Q

How does leukocyte adhesion deficiency present?

A

Neonatal bacterial infections, often life threateningHigh neutrophil count and delayed umbilical cord separation

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51
Q

What are the 2 types of leukocyte adhesion deficiency and what’s the difference between them?

A

LAD1 - deficiency of the beta2 intern subunit (CD18) of the leukocyte adhesion moleculeLAD2 - much rarer and has severe growth restriction and mental retardation

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52
Q

What is the treatment for leukocyte adhesion deficiency?

A

Bone marrow transplant

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53
Q

What is the main defect in chronic granulomatous disease?

A

Failure of oxidative killing - defect of NADPH oxidase leading to reduced reactive oxygen speciesydrogen peroxide

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54
Q

How can you test for chronic granulomatous disease?

A

Negative NBT test - NBT = a dye that changes from yellow to blue following interaction with hydrogen peroxideDihydrorhodamine flow cytometry test - DHR is oxidise to rhodamine which is strongly fluorescent after interaction with hydrogen peroxide

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55
Q

What are the features of chronic granulomatous disease?

A

Pneumonia, abscesses, suppurative arthritis and the diseases.Infections are with catalase positive organisms (can resist catalase negative organisms)

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56
Q

What are the catalase positive organisms?

A

PLACESS:PseudomonasListeriaAspergillusCandidaE coliStaph aureusSerratia

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57
Q

What is the cause/inheritance of chronic granulomatous disease?

A

Lots of mutations but mostly X linked

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58
Q

What is the treatment or chronic granulomatous disease?

A

Trimethoprim, itraconazole and interferon - can sometimes use a stem cell transplant

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59
Q

What is the cause of cyclic neutropenia?

A

Mutations in the ELA1 gene

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60
Q

What are the features of cyclic neutropenia?

A

Episodic neutropenia occurring every 3 weeks and lasting several days

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61
Q

What is the treatment for cyclic neutropenia?

A

G-CSF

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62
Q

Which cytokines are commonly deficient (in cytokine deficiencies)

A

IFN gamma, IFN gamma receptor, IL12, IL12 receptor

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63
Q

What do IFN gamma, IFN gamma receptor, IL12, IL12 receptor do?

A

Involve din signalling between T cells and macrophages to stimulate TNF and activate NADPH oxidase

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64
Q

What are the consequences of cytokine deficiencies?

A

Predispose to infections caused by salmonella, atypical mycobacteria, TB and BCGUnable to form granulomata

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65
Q

What is the most severe form of SCID?

A

Reticular dysgenesis

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66
Q

What is deficient in reticular dysgenesis?

A

Absolute deficiency in:NeutrophilsLymphocytesMonocytes/macrophagesPlatelets

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67
Q

What is the prognosis/treatment for reticular dysgenesis?

A

Fatal in very early life unless treated with bone marrow transplant

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68
Q

What is the consequence of complement deficiency?

A

Increased susceptibility to encapsulated bacterial infections

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69
Q

What are the 4 complement pathways where there can be deficiencies?

A

Classical, lectin, alternative, common/terminal

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70
Q

What is lacking in a classical pathway complement deficiency?

A

C1q/r/s, C2 (commonest, C4

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71
Q

What is classical pathway complement deficiency associated with?

A

SLE - as classical pathway is involved in removing immune complexes

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72
Q

Which test is abnormal in a classical pathway complement deficiency?

A

CH50 testy compleet deficiency?

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73
Q

What is the common deficiency in the lectin complement pathway?

A

MBL in 10%

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74
Q

How common is lectin pathway complement deficiency and is it significant?

A

Very common, not really clinically significant

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75
Q

Which factors are involved in alternative pathway complement deficiency?

A

Factors B/I/P

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76
Q

What is the alternative complement pathway involved in?

A

Killing bacteria

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77
Q

What are the features of alternative pathway complement deficiency?

A

Infections with encapsulated bacteria:Strep pneumoniaGroup B strepHaemophilus influenzaNeisseria meningitidis

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78
Q

Which test is abnormal in alternative pathway complement deficiency?

A

AP50 test

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79
Q

What is lacking in common/terminal pathway complement deficiency?

A

C3, 5, 6, 7, 8, 9 - so cannot form MAC to kill bacteria

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80
Q

What are the features of common and terminal pathway complement deficiency?

A

Susceptibility to bacterial infections - meningitis, pneumoniaMay be associated with membranoproliferative glomerulonephritis

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81
Q

Which test is abnormal in common and terminal pathway complement deficiency?

A

AP50 and CH50

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82
Q

What is the treatment for complement deficiency?

A

VaccinationProphylactic antibioticsHigh levels of suspicionScreen family members

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83
Q

Describe a type 1 hypersensitivity reaction

A

Immediate reaction provoked by re exposure to an allergen

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84
Q

Which Ig mediates a type 1 hypersensitivity reaction?

A

IgE

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85
Q

What is the pathophysiology of a type I hypersensitivity reaction?

A

Mast cells release mediators resulting in vasodilation, increased permeability, smooth muscle spasm

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86
Q

What are the typical symptoms of a type 1 hypersensitivity reaction?

A

Angioedema, urticaria, rhino conjunctivitis, wheeze, diarrhoea and vomiting, anaphylaxis

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87
Q

What is the prevalence of asthma in 13-14 year olds in the UK?

A

30%

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88
Q

What is the prevalence of allergic rhinitis in 13-14 year olds in the UK?

A

20%

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89
Q

What is the prevalence of atopic dermatitis in 13-14 year olds in the UK?

A

15%

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90
Q

What is the prevalence of food allergy in 13-14 year olds in the UK?

A

2.3%

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91
Q

What % of children with asthma also have food allergy?

A

4%

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92
Q

What are the components of the ‘atopic triad’?

A

Eczema, asthma, hay fever

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93
Q

What are some common type 1 hypersensitivity syndromes?

A

Atopic dermatitisFood allergyOral allergy syndromeLatex fruit/food syndromeAllergic rhinitisAcute urticaria

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94
Q

What is the allergen in atopic dermatitis?

A

Irritants, food and environmental

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95
Q

What is the pathology in atopic dermatitis?

A

Defects in B defensive predispose to staph aureus superinfection

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96
Q

How is atopic dermatitis diagnosed and when does it present?

A

Clinical - 80% present in the first year of life

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97
Q

How is atopic dermatitis treated?

A

Emollients, skin oils, topical steroids, antibiotics, PUNA phototherapy etc

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98
Q

What is the allergen in food allergy?

A

Milk, egg, peanut, tree nut, fish, shellfish

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99
Q

What is the pathology in food allergy?

A

IgE (anaphylaxis, OAS), cell mediated (coeliac) or both (atopic dermatitis)

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100
Q

How is food allergy diagnosed and when does it resolve?

A

Food diary, skin prick test, RAST challenge.Usually resolves by adulthood

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101
Q

How is food allergy treated?

A

Dietitian, food avoidance, epicene, control asthma if present

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102
Q

What is the allergen in oral allergy syndrome?

A

Birch pollen and rosacea fruit, ragweed and melons, mugwort and celery (cross reactivity)

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103
Q

What is the pathology in oral allergy syndrome?

A

Exposure to allergen induces allergy to food. Symptoms are limited to the mouth but 2% get anaphylaxis

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104
Q

How is oral allergy syndrome diagnosed?

A

Skin prick testing can be useful but clinical

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105
Q

How is oral allergy syndrome treated?

A

Avoid food. If ingested, wash mouth and use antihistamine

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106
Q

What is the allergen in latex food/fruit syndrome?

A

Chestnut, avocado, banana, potato, tomato, kiwi, papaya, eggplant, mango, wheat, melon

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107
Q

What is the pathology in latex food/fruit syndrome?

A

Some foods have latex like components so latex allergy sufferers are allergic to them

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108
Q

How is latex food/fruit syndrome diagnosed?

A

Skin prick test

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109
Q

How is latex food/fruit syndrome treated?

A

Strike avoidance of causative food

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110
Q

What is the allergen in allergic rhinitis?

A

Seasonal (tree and grass pollen, fungal spores) or perennial (pets, house dust mite) or occupational (latex, lab animals)

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111
Q

What are the features of allergic rhinitis?

A

Nasal itch and obstruction, sneezing, anosmia, eye symptoms

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112
Q

How is allergic rhinitis diagnosed?

A

Clinical: pale bluish swollen nasal mucosaSkin prick test and RAST

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113
Q

How is allergic rhinitis treated?

A

Allergen avoidanceAntihistamineSteroid nasal spraySodium cromoglycate eye dropsOral steroidsIpratropium nasal sprayGrass pollen desensitisation

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114
Q

What is the allergen in acute urticaria?

A

50% idiopathic50% caused by food, drugs, latex, viral infections, and febrile illnesses

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115
Q

What are the features of acute urticaria?

A

IgE mediated reaction - wheals which completely resolve within 6 weeks

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116
Q

How is acute urticaria diagnosed?

A

Mainly clinical - sometimes skin prick test

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117
Q

How is acute urticaria treated?

A

Allergen avoidance, antihistamines

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118
Q

What is anaphylaxis?

A

Severe systemic allergic reaction with respiratory difficulty and hypotension

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119
Q

Which allergens cause IgE mediated mast cell degranulation in anaphylaxis?

A

PeanutPenicillinStingsLatex

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120
Q

Which allergens cause non-IgE mediated mast cell degranulation in anaphylaxis?

A

NSAIDsIV contrastOpioidsExercise

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121
Q

What is the management for anaphylaxis?

A

Elevate legs100% oxygenIM adrenaline 500mcgInhaled bronchodilatorsHydrocortisone 100mg IVChlorphenamine 10mg IVIV fluidsSEEK HELP

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122
Q

How is a skin prick test done?

A

Positive control = histamineNegative control = diluentPositive test is a wheal ≥2mm greater than the negative controlDiscontinue antihistamines 48h before test (corticosteroids OK)

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123
Q

What are skin prick tests useful for?

A

Useful to confirm clinical history, and negative test excludes IgE mediated allergy

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124
Q

What does a RAST test measure

A

Levels of IgE in serum against a particular allergen

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125
Q

What is a RAST test useful for?

A

Confirms diagnosis of allergy and monitors response to anti-IgE treatment

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126
Q

How does a RAST test compare to skin prick testing?

A

Less sensitive/specific than a skin prick

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127
Q

What are the indications for a RAST test?

A

Can’t stop antihistamines, anaphylaxis history, extensive eczema

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128
Q

What does a ‘component resolved diagnosis’ measure?

A

The IgE response to a specific allergen protein, whilst conventional tests measure response to a range of allergen proteins

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129
Q

What is the gold standard test for food allergy?

A

Double blind oral food challenge - increasing volumes of offending food/drug ingested under close supervision

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130
Q

What’s the risk in a double blind food challenge test?

A

Severe reaction

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131
Q

What can you measure during an acute allergy episode?

A

Mast cell tryptatePeak at 1-2 hours and baseline by 6 hours

132
Q

What is the general pathophysiology in Type II hypersensitivity disorders?

A

IgG or IgM antibody reacts with cell or matrix associated self antigen resulting in tissue damage, receptor blockade/activation

133
Q

What are some examples of type II hypersensitivity disorders?

A

Haemolytic disease of the newbornAutoimmune haemolytic anaemia (+ ITP = Evans’ syndrome)Autoimmune thrombocytopenic purpuraGood pasture’s syndromePemphigus vulgarisGraves diseaseMyasthenia gravesAcute rheumatic feverPernicious anaemiaChurg-Strauss syndrome (eGPA)Wegener’s granulomatosisMicroscopic polyangiitisChronic urticaria

134
Q

Haemolytic disease of the newborn:Antigen = Pathology = Diagnosis is made by = Treatment is =

A

Haemolytic disease of the newborn:Antigen = on neonatal erythrocytesPathology = maternal IgG mediated reticulocytosis and anaemiaDiagnosis is made by = positive direct Coombs testTreatment is = maternal plasma exchange, exchange transfusion

135
Q

Autoimmune haemolytic anaemia:Antigen = Pathology = Diagnosis is made by = Treatment is =

A

Autoimmune haemolytic anaemia:Antigen = Numerous autoantigens e.g. Rh blood group AgPathology = Destruction of RBCs by autoantibody + complement + FcR + phagocytes, anaemiaDiagnosis is made by = Positive direct Coombs test, anti red cell AbTreatment is = steroids

136
Q

Autoimmune thrombocytopenic purpura:Antigen = Pathology = Diagnosis is made by = Treatment is =

A

Autoimmune thrombocytopenic purpura:Antigen = Glycoprotein IIb/IIIa on plateletsPathology = bruising/bleeding (purpura)Diagnosis is made by = anti-platelet antibodyTreatment is = steroids, IVIG, anti-D antibody, splenectomy

137
Q

Goodpasture’s syndrome:Antigen = Pathology = Diagnosis is made by = Treatment is =

A

Goodpasture’s syndrome:Antigen = non-collagenous domain of basement membrane collagen IVPathology = glomerulonephritis, pulmonary haemorrhageDiagnosis is made by = anti GBM Ab, linear smooth IF staining of IgG deposits on BMTreatment is = corticosteroids and immunosuppression

138
Q

Pemphigus vulgaris:Antigen = Pathology = Diagnosis is made by = Treatment is =

A

Pemphigus vulgaris:Antigen = epidermal cadherinPathology = Non-tense blistering of the skin and bullaeDiagnosis is made by = direct immunofluorescence showing IgGTreatment is = Corticosteroids and immunosuppression

139
Q

Graves disease:Antigen = Pathology = Diagnosis is made by = Treatment is =

A

Graves disease:Antigen = TSH receptorPathology = hyperthyroidismDiagnosis is made by = Anti-TSH-R AbTreatment is = carbimazole and propylthiouracil

140
Q

Myasthenia gravis:Antigen = Pathology = Diagnosis is made by = Treatment is =

A

Myasthenia gravis:Antigen = acetylcholine receptorPathology = Fatiguable muscle weakness, double visionDiagnosis is made by = Anti-Ach-R Ab, abnormal EMG, tension testTreatment is = neostigmine, pyridostigmine, if serious use IVIG and plasmaphoresis

141
Q

Acute rheumatic fever:Antigen = Pathology = Diagnosis is made by = Treatment is =

A

Acute rheumatic fever:Antigen = M proteins on group A strepPathology = myocarditis, arthritis, Sydenham’s choreaDiagnosis is made by = clinical, based on Jones criteriaTreatment is = aspirin, steroids and penicillin

142
Q

Pernicious anaemia:Antigen = Pathology = Diagnosis is made by = Treatment is =

A

Pernicious anaemia:Antigen = intrinsic factor and gastric parietal cellsPathology = low Hb, low B12 Diagnosis is made by = anti-gastric parietal cell Ab, anti-IF Ab, Schilling testTreatment is = dietary B12 or IM B12

143
Q

Churg-Strauss syndrome:Antigen = Pathology = Diagnosis is made by = Treatment is =

A

Churg-Strauss syndrome:Antigen = medium and small vessel vasculitisPathology = allergy -> asthma -> systemic disease (male predominancE)Diagnosis is made by = pANCA against myeloperoxidase, granolas, eosinophil granulocytesTreatment is = prednisolone, azathioprine, cyclophosphamide

144
Q

Wegener’s granulomatosis:Antigen = Pathology = Diagnosis is made by = Treatment is =

A

Wegener’s granulomatosis:Antigen = medium and small vessel vasculitisPathology = sinus problems, lung cavitation and haemorrhage, crescentic glomerulonephritisDiagnosis is made by = cANCA against proteinase-3, granulomasTreatment is = corticosteroids, cyclophosphamide, cotrimoxazole

145
Q

Microscopic polyangiitis:Antigen = Pathology = Diagnosis is made by = Treatment is =

A

Microscopic polyangiitis:Antigen = pauci-immune necrotising small vessel vasculitisPathology = purpura, lived, many different organs affectedDiagnosis is made by = pANCA against myeloperoxidaseTreatment is = prednisolone, cyclophosphamide, or azathioprine, plasmaphoresis

146
Q

Chronic urticaria:Antigen = Pathology = Diagnosis is made by = Treatment is =

A

Chronic urticaria:Antigen = medications (NSAIDs), cold, food, pressure, sun, exercise, insect stings, bites, idiopathicPathology = persistent itchy wheals lasting >6 weeks associated with angioedema in 50% cases. IgG against FceRI or IgG against IgE. Exclude urticarial vasculitis in those who respond poorly to antihistamine. Diagnosis is made by = Challenge test, ESR (raised in urticarial vasculitis), skin prick testingTreatment is = avoid precipitants. Check for thyroid disease. Preventative antihistamine. IM adrenaline for pharyngeal angioedema. 1% menthol in aqueous cream for pruritus (also Doxepin and cyclosporin)

147
Q

What is the general pathology in type III hypersensitivity disorders?

A

IgG or IgM immune complex (Ab vs soluble Ag) mediated tissue damage

148
Q

What are some examples of type III hypersensitivity disorders?

A

Mixed essential cryoglobulinaemiaSerum sicknessPolyarteritis nodosaSystemic lupus erythematosus

149
Q

Mixed essential cryoglobulinaemia:Antigen = Pathology = Diagnosis is made by = Treatment is =

A

Mixed essential cryoglobulinaemia:Antigen = IgM against IgG +/- hep C antigensPathology = Joint pain, splenomegaly, skin, nerve and kidney involvement, associated with hep CDiagnosis is made by = Mixture of clinical biopsiesTreatment is = NSAIDs, corticosteroids and plasmaphoresis

150
Q

Serum sickness:Antigen = Pathology = Diagnosis is made by = Treatment is =

A

Serum sickness:Antigen = reaction to proteins in antiserum (penicillin)Pathology = rashes, itching, arthralgia, lemphadenoapthy, fever and malaise. Symptoms take 7-12 days to develop.Diagnosis is made by = low C3, blood shows immune completes or signs of blood vessel inflammationTreatment is = discontinuation of precipitant, steroids, antihistamines +/- analgesia

151
Q

Polyarteritis nodosa:Antigen = Pathology = Diagnosis is made by = Treatment is =

A

Polyarteritis nodosa:Antigen = hep B, hep C virus antigensPathology = fever, fatigue, weakness, arthralgia, skin, nerve and kidney involvement, pericarditis and MI, associated with Hep BDiagnosis is made by = clinical criteria and biopsy (raised ESR, raised WCC, raised CRP), ‘rosary sign’ (small bead like aneurysms)Treatment is = prednisolone and cyclophosphamide

152
Q

Systemic lupus erythematosus:Antigen = Pathology = Diagnosis is made by =

A

Systemic lupus erythematosus:Antigen = main intracellular components: DNA, histones, RNPPathology = M:F 1:9, 4 of these 11: serositis, seizures, aphthous ulcers, arthritis, photosensitivity, discoid rash, malar rash, haematology, kidney findings, antinuclear antibody (ANA positive), immunological finding (anti dsDNA, anti-sm)Diagnosis is made by = low C4 (low C3 only in severe disease), antibodies to dsDNA, histones (drug induced), Ro, La, Sm, U1RNP, high ESR, normal CRP

153
Q

Which drugs can cause drug induced SLE?

A

Hydrazine, procainamide, isoniazid

154
Q

What is the general pathology of type IV hypersensitivity disorders?

A

Delayed hypersensitivity, T cell mediated

155
Q

What are some examples of type IV hypersensitivity disorders?

A

T1DMMSRheumatoid arthritisContact dermatitisMantoux testCrohn’s

156
Q

Type 1 diabetes:Antigen = Pathology = Diagnosis is made by = Treatment is =

A

Type 1 diabetes:Antigen = pancreatic beta cell proteins (glutamate decarboxylase, GAD)Pathology = insulitis, beta cell destructionDiagnosis is made by = blood glucose, ketonuria, glutamate decarboxylase antibodies, islet cell antibodiesTreatment is = insulin via injections or continuous infusion

157
Q

MS:Antigen = Pathology = Diagnosis is made by = Treatment is =

A

MS:Antigen = oligodendrocyte proteins (myelin basic protein, proteolipid protein)Pathology = demyelinating disease, perivascular inflammation, paralysis, ocular lesionsDiagnosis is made by = CSF shows oligoclonal bands of IgG on electrophoresisTreatment is = corticosteroids, interferon beta

158
Q

Rheumatoid arthritis:Antigen = Pathology = Diagnosis is made by = Treatment is = Is also hypersensitivity type:

A

Rheumatoid arthritis:Antigen = antigen in synovial membranePathology = chronic arthritis, rheumatoid nodules, lung fibrosisDiagnosis is made by = X ray, rheumatoid factor (85% sensitive), anti CCP (95% specific), increased ESR, increased CRPTreatment is = analgesia, steroids, DMARDsIs also hypersensitivity type: III - IgM Ab vs Fc region of IgG

159
Q

Contact dermatitis:Antigen = Pathology = Diagnosis is made by = Treatment is =

A

Contact dermatitis:Antigen = environmental chemicals, poison ivy, nickelPathology = dermatitis with usually short lived itching, blisters and wheals Diagnosis is made by = clinical or use patch testTreatment is = if no resolution use corticosteroids or antihistamines

160
Q

Mantoux test:Antigen = Pathology =

A

Mantoux test:Antigen = tuberculinPathology = skin induration indicates TB exposure

161
Q

Crohn’s disease:Pathology = Diagnosis is made by = Treatment is =

A

Crohn’s disease:Pathology = Th1 mediated chronic inflammation in skip lesions in GIT. NOD2 gene mutation in 30%Diagnosis is made by = biopsy of lesion. Can affect any part of GIT from mouth to anusTreatment is = antibiotics, anti-inflammatory drugs, e.g. mesalazine, TNF alpha antagonists, e.g. infliximab, steroids

162
Q

HLA ASSOCIATIONS: ankylosing spondylitisSusceptibility allele = Relative risk (fold) =

A

HLA ASSOCIATIONS: ankylosing spondylitisSusceptibility allele = HLA B27Relative risk (fold) = 87

163
Q

HLA ASSOCIATIONS: Goodpasture’s syndromeSusceptibility allele = Relative risk (fold) =

A

HLA ASSOCIATIONS: Goodpasture’s syndromeSusceptibility allele = HLA DR15/DR2Relative risk (fold) = 10

164
Q

HLA ASSOCIATIONS: Grave’s diseaseSusceptibility allele = Relative risk (fold) =

A

HLA ASSOCIATIONS: Grave’s diseaseSusceptibility allele = HLA DR3Relative risk (fold) = 4

165
Q

HLA ASSOCIATIONS: SLESusceptibility allele = Relative risk (fold) =

A

HLA ASSOCIATIONS: SLESusceptibility allele = HLA DR3Relative risk (fold) = 6

166
Q

HLA ASSOCIATIONS: Type 1 diabetesSusceptibility allele = Relative risk (fold) =

A

HLA ASSOCIATIONS: Type 1 diabetesSusceptibility allele = HLA-DR3/DR4Relative risk (fold) = 25

167
Q

HLA ASSOCIATIONS: Rheumatoid arthritisSusceptibility allele = Relative risk (fold) =

A

HLA ASSOCIATIONS: Rheumatoid arthritisSusceptibility allele = HLA-DR4Relative risk (fold) = 4

168
Q

What is PTPN22 and what is this polymorphism associated with?

A

Tyrosine phosphatase expressin lymphocytes - associated with development of RA, SLE, T1DM

169
Q

What is CTLA4 and what is this polymorphism associated with?

A

Receptor for CD80/CD86 expressed by T cells, transmits inhibitory signal to control T cell activation; associated with SLE, T1DM, autoimmune thyroid disease

170
Q

What are the features of limited cutaneous scleroderma (CREST syndrome)?

A

CalcinosisRaynaud’sOesophageal dysmotilitySclerodactylyTelangiectasiaand primary pulmonary hypertensionSkin involvement is up to forearms only, and perioral

171
Q

How do you diagnose CREST syndrome?

A

Anti centromere antibodies

172
Q

What are the risks in CREST syndrome?

A

High risk of lung fibrosis and renal crisis

173
Q

What are the features of diffuse cutaneous scleroderma?

A

CREST +GITInterstitial pulmonary diseaseRenal problems

174
Q

What antibodies do you see in diffuse cutaneous scleroderma?

A

Anti-topoisomerase/Scl70RNA Pol I, II, IIIFibrillarin

175
Q

Is diffuse cutaneous scleroderma more common in women or in men?

A

Female:male 4:1

176
Q

In whom is Sjogren’s syndrome most common?

A

M:F= 1:9Onset in late 40s

177
Q

What are the features of Sjogren’s syndrome?

A

Xerostomia (dry mouth)Keratoconjunctivitis siccaNose and skin involvementMay affect kidneys, blood vessels, lungs, liver, pancreas, PNSMay get parotid or salivary gland enlargement

178
Q

What antibodies are there in Sjogren’s syndrome?

A

Anti-Ro and anti-La

179
Q

What test can you do in Sjogren’s syndrome other than antibodies?

A

Schirmer test to measure tear production

180
Q

What does IPEX syndrome stand for?

A

Immune dysregulationPolyendocrinopathyEnteropathyX-linked inheritance syndrome+ autoimmune diseases

181
Q

What are the features of IPEX syndrome?

A

Eczematous dermatitisNail dystrophyAutoimmune skin conditions e.g. alopecia universalis, bullous pemphigoid

182
Q

What is the prognosis in IPEX syndrome?

A

Most affected children die within the first 2 years of life

183
Q

How is IPEX syndrome inherited?

A

X-linked recessive - exclusive expression in males

184
Q

How can you treat IPEX syndrome?

A

Bone marrow transplant is the only cure, but you can use immunomodulators to help

185
Q

What is the basic pathophysiology in coeliac disease?

A

Failure of tolerance to gluten -> villous atrophy and enteropathy

186
Q

What are the features of coeliac disease?

A

GIT discomfort, constipation, diarrhoea, bloating, fatigueIron, B12, folate, fat, vitamins A, D, E + K + calcium deficiencies

187
Q

What antibodies are associated with coeliac disease?

A

IgA EMA (anti-endomysial) - disappears with exclusion diet, 95% specific, 85% sensitive IgA TGT (anti transglutaminase) - 95% specific, 90-94% sensitive IgG anti-gliadin - most persistent, 30-50% specific, 57-80% sensitive

188
Q

What’s associated with coeliac disease?

A
  • Dermatitis herpetiformis
  • Down’s
  • North Africa 20/1000
  • 95% have DQ2 or DQ8 (two eight or not to eat)
189
Q

What’s the gold standard test for coeliac disease?

A

Duodenal biopsy - but not 1st line

190
Q

What are the 4 extractable nuclear antibodies?

A

RoLaSmU1RNP

191
Q

What autoantibody is associated with antiphospholipid syndrome?

A

Against:

  • Cardiolipin
  • Beta2 glycoprotein
  • Lupus anticoagulant
192
Q

What autoantibody is associated with autoimmune hepatitis?

A
  • Anti-smooth muscle
  • Anti liver kidney microsomal 1 (anti-LKM-1)
  • Anti soluble liver antigen (anti-SLA)
193
Q

What autoantibody is associated with autoimmune haemolytic anaemia?

A

Anti-Rh blood group antigen

194
Q

What autoantibody is associated with autoimmune thrombocytopenic purpura?

A

Anti glycoprotein IIb-IIIa or Ib-IX antibody

195
Q

What autoantibody is associated with Churg-Strauss syndrome (eGPA)?

A

Peri-nuclear/protoplasmic staining anti neutrophil cytoplasmic antibodies (pANCA)

196
Q

What autoantibody is associated with coeliac disease?

A
  • Anti tissue transglutaminase (IgA)

* Anti endomysial (IgA)

197
Q

What autoantibody is associated with congenital heart block in infants of mothers with SLE?

A

Anti-Ro antibody

198
Q

What autoantibody is associated with dermatitis herpetiformis?

A

Anti-endomysial antibody (IgA)

199
Q

What autoantibody is associated with dermatomyositis?

A

Anti-Jo-1 (tRNA synthetase)

200
Q

What autoantibody is associated with diffuse cutaneous scleroderma?

A

Antibodies to topoisomerase/Scl70, Rna Pol I, II, III, fibrillarin (nucleolar pattern)

201
Q

What autoantibody is associated with Goodpasture’s syndrome?

A

Anti-GBM antibody

202
Q

What autoantibody is associated with Grave’s disease?

A

Anti-TSH receptor antibody

203
Q

What autoantibody is associated with Hashimoto’s thyroiditis?

A

Antibodies to thyroglobulin and thyroperoxidase

204
Q

What autoantibody is associated with Limited cutaneous scleroderma (CREST)?

A

Anti centromere antibody

205
Q

What autoantibody is associated with microscopic polyangiitis (MPA)?

A

Perinuclear/protoplasmic-staining anti neutrophil cytoplasmic antibodies (pANCA)

206
Q

What autoantibody is associated with mixed connective tissue disease?

A

Anti-U1RNP antibody (speckled pattern)

207
Q

What autoantibody is associated with myasthenia gravis?

A

Anti-Ach receptor antibody

208
Q

What autoantibody is associated with pernicious anaemima?

A

Antibody to gastric parietal cells (90%) and intrinsic factor (10%)

209
Q

What autoantibody is associated with polymyositis?

A

Anti-Jo-1 (tRNA synthetase)

210
Q

What autoantibody is associated with primary biliary cirrhosis?

A

Anti-mitochondrial antibody

211
Q

What autoantibody is associated with rheumatoid arthritis?

A

Anti-CCP antibodiesRheumatoid factor (less specific)

212
Q

What autoantibody is associated with Sjogren’s syndrome?

A

Anti-RoAnti-La (speckled pattern)60-70% Rheumatoid factor positive

213
Q

What autoantibody is associated with SLE?

A
  • dsDNA
  • Histones (homogenous)
  • Ro
  • La
  • Sm
  • U1RNP (speckled)
214
Q

What autoantibody is associated with type 1 diabetes mellitus?

A

Antibodies to glutamate decarboxylase and beta cells

215
Q

What autoantibody is associated with Wegener’s granulomatosis (GPA)?

A

Cytoplasmic antineutrophil cytoplasmic antibodies (cANCA)

216
Q

What is the target of anti T cell monoclonal antibodies?

A

Resting T cells

217
Q

Which immune therapies target T cell proliferation (involving IL-2)?

A

Antiproliferative agentsInhibitors of cell signallingCorticosteroids

218
Q

Which immune therapies target the effector functions of T cells e.g. production of TNFalpha +antibodies from B cells?

A
  • Anti TNFalpha monoclonal antibodies
  • Anti-IL-12/23 monoclonal antibodies
  • Plasmaphoresis
  • Corticosteroids
219
Q

How would you choose a regime to suppress T Cells?

A

One from each group:

  • Inhibitors of cell signalling (tacrolimus, ciclosporin)
  • Antiproliferative agents (azathioprine, mycophenolate mofetil, methotrexate, cyclophosphamide)
  • Blocker of cytokine production (prednisolone)
220
Q

What are some examples of anti-T cell monoclonal antibodies?

A

Muromonab CO3BasiliximabTocilizumabAbatacept

221
Q

What are some examples of anti-proliferative agents?

A

CyclophosphamideMycophenolate mofetilAzathioprine

222
Q

What are some examples of inhibitors of cell signalling?

A

TacrolimusCiclosporinSirolimus

223
Q

What is infliximab?

A

An anti-TNFalpha monoclonal antibody

224
Q

What is uskekinumab?

A

An anti-IL-12/23 monoclonal antibody

225
Q

Muromonab CD3:

  • Method of action
  • Indications
  • Side effects
A

Muromonab CD3:

  • Method of action - blocks CD3 on T cells
  • Indications - for active rejection
  • Side effects - fever, leucopenia
226
Q

Basiliximab:

  • Method of action
  • Indications
  • Side effects
A

Basiliximab:

  • Method of action - blocks CD25 (alpha chain of IL-2 receptor)
  • Indications - prevents rejection in transplantation
  • Side effects - GI disturbance
227
Q

Tocilizumab:

  • Method of action
  • Indications
  • Side effects
A

Tocilizumab:

  • Method of action - blocks IL-6 receptor
  • Indications - Rheumatoid arthritis if anti TNF drugs have failed
  • Side effects - infections
228
Q

Abatacept:

  • Method of action
  • Indications
  • Side effects
A

Abatacept:

  • Method of action - anti CTLA4 Ig, blocks co stimulation of T cells
  • Indications - rheumatoid arthritis if anti TNF drugs have failed
  • Side effects - infections and cough
229
Q

Cyclophosphamide:

  • Method of action
  • Indications
  • Side effects
A

Cyclophosphamide:

  • Method of action - akylates guanine base of DNA, affects B > T cells
  • Indications - multi system connective tissue disease e.g. SLE and cancer
  • Side effects - hair loss, bone marrow suppression, sterility, haemorrhagic cystitis
230
Q

Mycophenolate mofetil:

  • Method of action
  • Indications
  • Side effects
A

Mycophenolate mofetil:

  • Method of action - blocks de novo nucleotide synthesis, affects T>B cells
  • Indications - autoimmune disease, vasculitis, transplantation
  • Side effects - bone marrow suppression, herpes
231
Q

Azathioprine:

  • Method of action
  • Indications
  • Side effects
A

Azathioprine:

  • Method of action - metabolised to 6-mercaptopurine in the liver, blocks de novo purine synthesis
  • Indications - inflammatory and autoimmune diseases, transplantation
  • Side effects - bone marrow suppression (measure TPMT), hepatotoxicity
232
Q

Tacrolimus:

  • Method of action
  • Indications
  • Side effects
A

Tacrolimus:

  • Method of action - inhibits calcineurin which norally activates the transcription of IL-2, therefore decreases IL-2
  • Indications - mainly rejection prophylaxis in transplantation
  • Side effects - diabetes
233
Q

Ciclosporin:

  • Method of action
  • Indications
  • Side effects
A

Ciclosporin:

  • Method of action - inhibits calcineurin which norally activates the transcription of IL-2, therefore decreases IL-2
  • Indications - mainly rejection prophylaxis in transplantation
  • Side effects - gingival hypertrophy
234
Q

Sirolimus:

  • Method of action
  • Indications
A

Sirolimus:

  • Method of action - blocks clonal proliferation
  • Indications - mainly rejection prophylaxis in transplantation
235
Q

Infliximab:

  • Method of action
  • Indications
  • Side effects
A

Infliximab:

  • Method of action - binds to TNF alpha
  • Indications - psoriasis, Crohn’s, rheumatoid arthritis, others
  • Side effects - TB, lymphoma, autoimmune phenomena
236
Q

Uskekinumab:

  • Method of action
  • Indications
  • Side effects
A

Uskekinumab:

  • Method of action - binds to p40 subunit of IL-12 and IL-23
  • Indications - psoriasis
  • Side effects - infections and cough
237
Q

What is the method of action of prednisolone?

A

Inhibits phospholipase A2:
* Decreases platelet activating factor
* Decreases arachidonic acid
* Decreases trafficking of phagocytes (hence transient increase in phagocyte count)
Lymphopenia, apoptosis of T + B cells, decreased antibodies

238
Q

What are the indications for prednisolone?

A

Used as anti inflammatory and in autoimmune disease

239
Q

What are the side effects of prednisolone?

A
  • Diabetes
  • Central obesity
  • Adrenal suppression
  • Cataracts
  • Glaucoma
  • Pancreatitis
  • Osteoporosis
  • Moon face
  • Acne
  • Hirsutism
  • Neutrophilia
240
Q

What happens in plasmaphoresis?

A

Each time, 50% of patient’s plasma is replaced with donor’s

241
Q

What are the indications for plasmapharesis?

A

Goodpasture’sMyastheniaAntibody mediated rejection

242
Q

What are the side effects of plasmapharesis?

A

Reboud antibody production limits its efficacy

243
Q

What does rituximab do and what is it used for?

A

Anti CD20 - so decreases B cells (not plasma cells). Used for lymphoma and autoimmune disease

244
Q

What does methotrexatedo and what is it used for?

A

Inhibits dihydrofolate reductase (DHFR) and therefore decreases DNA synthesis. Used in autoimmune disease e.g. RA, psoriasis, Crohn’s etc. Also used in chemotherapy and as an abortifacent

245
Q

What are the main side effects of methotrexate?

A

Teratogenicity and hepatotoxicity

246
Q

What does alemtuzumab (Campath) do and what is it used for?

A

Monoclona antibody that binds to CD52 found on lymphocytes, resulting in depletion. OFten used in CLL and a new variant is being used for multiple sclerosis.

247
Q

What is a side effect of alemtuzumab?

A

Increased susceptibility to CMV infection

248
Q

What does natalizumab do and what is it used for?

A

Anti alpha-4 integrin; used in MS and Crohn’s

249
Q

Which polymorphism leaves someone unable to metabolise azathioprine?

A

TPMT polymorphism

250
Q

What are the 4 main ways to boost the immune system?

A
  • Vaccination
  • Human normal immunoglobulin
  • Specific immunoglobulin (passive vaccination)
  • Recombinant cytokines
251
Q

What is human normal immunoglobulin and how is it given?

A

From >1000 donors who have been screened for HIV, Hep B and hep C, it contains preformed IgG against a full range of organisms.Given every 3-4 weeks, the half life is 18 days.

252
Q

What is human normal immunoglobulin used for?

A

Primary antibody deficiencies (CVID, Brutons etc), secondary deficiencies (CLL, multiple myeloma, BMT), and passive vaccination

253
Q

What are some of the diseases you can get specific immunoglobulins for?

A

Ravies, varicella zoster, hep B, tetanus

254
Q

What is the use of recombinant cytokines?

A

Boost immune response to cancer and some pathogens

255
Q

What are some examples of recombinant cytokines?

A
  • Interferon alpha
  • Interferon beta
  • Interferon gamma
256
Q

What is interferon alpha given for?

A
  • Hepatitis C, hepatitis B
  • Kaposi sarcoma
  • Hairy cell leukaemia, chronic myelogenous leukaemia, malignant myeloma
257
Q

What is interferon beta given for?

A
  • Relapsing MS

* Mechanism of action is not known

258
Q

What is interferon gamma given for?

A

Chronic granulomatous disease

259
Q

How does allergen desensitisation work?

A
  • Supervised administration of an allergen, starting with a tiny dose and escalating every week until maximal dose reached
  • Maintenance dose given monthly for 3-5 years
  • Reduces clinical symptoms of monoallergic disorders
260
Q

What allergies does allergen desensitisationwork for? Which is it not very good for?

A

Bee and wasp venom, grass pollen, house dust mite. Not good for food, latex

261
Q

What are the advantages and disadvantages of allergen desensitisation?

A

Good: only treatment that alters the natural course of disease and reduces clinical symptomsBad: costly, laborious and risk of severe adverse reaction

262
Q

What is HIV?

A

An RNA virus that targets CD4+ T helper cells as hosts (also CD4+ monocytes and dendritic cells)

263
Q

What does the HIV virus bind to?

A

gp120 (initial binding)gp41 (conformational change)Most strains use CCR5 and CXCR4 chemokine co receptors

264
Q

What comprises the innate response to HIV?

A
  • Non specific activation of macrophages, NK cells and complement
  • Stimulation of dendritic cells via TLR
  • Release of cytokines and chemokines
265
Q

What comprises the adaptive response to HIV?

A
  • Neutralising antibodies: anti-gp120 and anti-gp41
  • Non-neutralising antibodies: anti-p24 gag IgG
  • CD8+ T cells can prevent HIV entry by producing chemokines MIP-Ia, and RANTES which block co receptors
266
Q

How does HIV damage the immune response?

A
  • Remains infectious even when antibody coated
  • Atibated infected CD4+ helper T cells are killed by CD8+ T cells
  • CD4 T cell memory is lost and there is failure to activate memory CTL
  • Monocytes and dendritic cells are therefore not activated by the CD4+ T Cells and cannot prime naïve CD8+ CTL (due to impaired antigen presenting functions)
  • Infected monocytes and dendritic cells are killed by virus or CTL
  • Quasispecies are produced by error prone reverse transcriptatse ->these escape from the immune response
267
Q

What are the 7 stages in the life cycle of HIV?

A
  • Attachment/entry
  • Reverse transcription and DNA synthesis
  • Integration
  • Viral transcription
  • Viral protein synthesis
  • Assembly of virus and release of virus
  • Maturation
268
Q

What is the median time from infecrtion to development of AIDS?

A

8-10 years

269
Q

How many HIV patients are ‘rapid progressors’ and how long do they take to develop AIDS?

A

10% - takes 2-3 years

270
Q

How many HIV patients are ‘long term non-progressors’ and what does this mean?

A
271
Q

What measure predicts disease progression in HIV?

A

The initial viral burden (set point)

272
Q

How is HIV diagnosed?

A

Screening test - detects anti-HIV Ab via ELISAConfirmation test detects Ab via Western Blot, but requires the patient to have seroconverted (started to produce Ab), which happens after a ~10 week incubation period

273
Q

What tests should you do in HIV after diagnosis?

A
  • Viral load - with PCR to detect viral RNA
  • CD4 count via FACS (flow cytometry), used to assess course of disease, and onset of AIDS correlates with diminuation of number of CD4+ T Cells
  • Resistance testing - to antiretrovirals, done with phenotypic and genotypic assays
274
Q

What CD4 count defines AIDS?

A
275
Q

When should you start treatment for HIV?

A
  • CD4
  • Patient is symptomatic
  • Start thinking about it when CD4
276
Q

What is HAART?

A

2 NRTIs and one PI (or NNRTI) e.g. emtricitabine + tenofovir + efavirenz

277
Q

What ARV should you give in pregnancy?

A

Zidovudine - antepartum PO, IV for delivery.PO to newborn for 6/52, reduces transmission from 26% to 8%

278
Q

What are the limitations of HAART?

A
  • Doesn’t eradicate latent HIV-1
  • Fails to restore HIV specific T cell responses
  • Toxicities
  • High pill burden
  • Adherence
  • Threat of drug resistance
  • QoL
  • Cost
279
Q

What are two classes of HIV drugs that target the docking process?

A

Fusion inhibitorsAttachment inhibitors

280
Q

Give an example of an HIV drug which is a fusion inhibitor, as well as its side effects

A

Enfuvirtide - local reactions to infections, hypersensitivity (0.1-1%)

281
Q

What is an example of an HIV drug which is an attachment inhibitor?

A

Maravirox

282
Q

What are some examples of NRTIs?

A

ZidovudineDidanosineStavudineLamivudineZalcitabineAbacavirEmtricitabineEpzicomCombivirTrizivir

283
Q

What are the side effects of NRTIs?

A
  • Generally rare
  • Fever
  • Headache
  • GI disturbance
  • BMS (zidovudine)
  • peripheral neuropathy (zalcitabine, stavudine)
  • Mitchondrial toxicity (stavudine)
  • Hypersensitivity (abacavir)
284
Q

What kind of drug is tenofovir?

A

Nucleotide RTI

285
Q

What are the side effects of tenofovir?

A

bone and renal toxicity

286
Q

What are some examples of non-NRTIs and their side effects?

A
  • Nevirapine - hepatitis and rash
  • Delavirdine - rash
  • Efavirenz - CNS effects
287
Q

What are some examples of integration inhibitor HIV drugs?

A

RaltegravirElvitegravir

288
Q

What are some examples of HIV drugs which are protease inhibitors?

A
  • Indinavir
  • Nelfinavir
  • Ritonavir
  • Amprenavir
  • Fosamprenavir
  • Lopinavir
  • Atazanavir
  • Saquinavir
289
Q

What are the side effects of protease inhibitors?

A
  • Hyperlipidaemias
  • Fat redistribution
  • Type 2 diabetes
290
Q

What are the 3 stages of transplant refection?

A
  • Recognition
  • Activation
  • Effector function
291
Q

Which antigens are recognised in transplant rejection?

A
  • HLA - A, B, DR
  • Minor HLA - other polymorphic self peptides
  • ABO blood antigens
292
Q

What are the two types of rejection?

A
  • Direct: donor APC presenting antigen and/or MHC to recipient T Cells; acute rejection mainly invovles direct presentation
  • Indirect: recipient APC presenting donor antigen to recipient T cells i.e. the immune system working normally, as it would for an infection
293
Q

Which type of rejection (presentation)does chronic rejection usually involve?

A

Indirect

294
Q

Hyperacute transplant rejection:

  • Time -
  • Mechanism -
  • Pathology -
  • Treatment -
A

Hyperacute transplant rejection:

  • Time - mins-hours
  • Mechanism - preformed Ab which activates complement
  • Pathology - thrombosis and necrosis
  • Treatment - prevention: cross match
295
Q

Acute cellular transplant rejection:

  • Time -
  • Mechanism -
  • Pathology -
  • Treatment -
A

Acute cellular transplant rejection:

  • Time - weeks-months
  • Mechanism - CD4 activating a type IV reaction
  • Pathology - cellular infiltrate
  • Treatment - T cell immunosuppression
296
Q

Acute antibody mediated transplant rejection:

  • Time -
  • Mechanism -
  • Pathology -
  • Treatment -
A

Acute antibody mediated transplant rejection:

  • Time - weeks-months
  • Mechanism - B cell activation; antibody attacks vessels
  • Pathology - vasuclitis, C4d
  • Treatment - Ab removal and B cell immunosuppression
297
Q

Chronic transplant rejection:

  • Time -
  • Mechanism -
  • Pathology -
  • Treatment -
A

Chronic transplant rejection:

  • Time - months-years
  • Mechanism - immune and non immune mechanism
  • Pathology - fibrosis
  • Treatment - minimise organ damage
298
Q

Graft versus host disease:

  • Time -
  • Mechanism -
  • Pathology -
  • Treatment -
A

Graft versus host disease:

  • Time - days-weeks
  • Mechanism - donor cells attacking host
  • Pathology - skin (rash), gut (D+V, bloody stool), liver (jaundice)
  • Treatment - prevention/immunosuppression, corticosteroids
299
Q

What needs to be done to match a transplant?

A
  • Determine donor and recipient blood group and HLA type - using PCR, and maximise similarity
  • Check recipient’s pre formed Ab against ABO and HLA via CDC (complement dependent cytotoxicity) and FACS (flow cytometry), and luminex (like solid phase FACS to pick up Abs to individual HLAs)
  • Cross match via CDC and FACS; tests if serum from recipient is able to bind/kill donor lymphocytes - positive crossmatch is a contraindication for transplantation
  • After transplant check again for new antibodies against the graft
300
Q

Other than matching the transplant, what can you do to prevent rejection?

A
  • Pre transplant induction agent to suppress T cell responses e.g. anti CD52 (alemtuzumab) or anti CD25 (basiliximab)
  • Use immunosuppressants post transplant to reduce rejection e.g. CNI and MMF Treat episodes of acute rejection:
  • Cellular: steroids, IVIG
  • Antibody mediated: IVIG, plasma exchange, anti-C5
301
Q

What are the post-transplant complications (other than rejection)?

A

Infection:

  • Increased risk of conventional infections
  • Opportunistic infections: CMV, BK virus, pneumocystis carinii Malignancy
  • Viral associated (x100): Kaposi’s sarcoma (HHV8), lymphoproliferative disease (EBV)
  • Skin cancer x20
  • Other cancers e.g. lung, colon x2-3 Atherosclerosis
  • Hypertension, hyperlipidaemia
  • x20 increased risk of death from MI compared to age-matched general population
302
Q

What is in the UK vaccination programme?

A

2 months: DTaP/IPV/Hib/PCV/MenB 3 months: DTaP/IPV/Hib/Men C/Men B/rotavirus 4 months: DtaP/IPV/Hib/PCV/MMR 12-13 months:Hib/MenC/PCV/MMR/MenB 3 years 4 months: DTaP/IPV/MMR 13-18 years:DT/IPV Girls aged 12-13 years: HPV

303
Q

What vaccinations are given to at risk groups in the UK?

A
  • Anthrax
  • Hep A
  • Hep B
  • Men ACWY
  • Rabies
  • Varicella if not immune
  • BCG (babies in high prevalence areas, HCWs)
304
Q

What vaccinations should travellers from the UK consider?

A
  • Cholera
  • Hep A
  • Hep B
  • Japanese encephalitis
  • Tick born encephalitis
  • Typhoid
  • Yellow fever
305
Q

Where are central memory T cells found and what do they do?

A
  • Lymph nodes and tonsils - roll along and extravasate in High Endothelial Venules (HEVs)
  • CCR7+ and CD62L+ allow entry via HEVs to lymph nodes
  • Produce IL-2 to support other cells
  • More central memory in CD4 population
306
Q

Where are effector memory T cells found and what do they do?

A
  • Liver, lungs and gut
  • CCR7 -ve and CD62 low, therefore not found in lymph nodes
  • Effector - produce perforin and IFN gamma
  • More effector memory in CD8 population
307
Q

What is the function of CCR7?

A

Binds CCL19 and CCL21 present on the luminal surface of endothelial cells in lympho nodes, which causes firm arrest and the initiation of extravasation

308
Q

What is the function of CD62L?

A

Interacts with a molecule in HEV, which mediates attachment and rolling

309
Q

What do B memory cells do?

A

Can differentiate into long lived plasma cells, which produce:

  • Quicker response
  • More antibodies
  • Higher affinity antibodies
  • More IgG
  • Generally better antibodies
310
Q

What response do T helper cells produce?

A
  • Th1: cell mediated - involves cytokines IL-2, IFN gamma, TNF
  • Th2: humoral response, involves cytokines IL-4, IL-5, IL-6
311
Q

What are the advantages of live vaccines?

A
  • Lifelong immunity with no booster required

* Immune response to several antigens and protection against cross reactive strains

312
Q

What are the disadvantages of live vaccines?

A
  • Careful in immunodeficient pateitns
  • Reversion to virulence
  • Harder to store
313
Q

What are some examples of live vaccines?

A
  • Sabin polio (oral - no longer used)
  • MMR
  • Varicella
  • Yellow fever
  • BCG
  • Typhoid
314
Q

What are the advantages of inactivated vaccines?

A
  • Easy to store
  • Cheaper
  • Safe in ID patients
  • No mutation/reversion
  • Can eliminate wild-type virus from the community
315
Q

What are the disadvantages of inactivated vaccines?

A
  • Poorer and hosrter immunity

* Repeated boosters, adjuvants to combat this

316
Q

What are the four different kinds of inactivated vaccines and examples of them?

A
  • Inactivated - salk (polio), anthrax, cholera, bubonic plague, Hep A, rabies, pertussis
  • Component - Hep B (HbS antigen), HPV (capsid), influenza (haemagglutinin, neuraminidase)
  • Conjugate - tetanus (exotoxin), Hib
  • Toxoid - diphtheria, tetanus
317
Q

What is the point of adjuvants in vaccines?

A

They increase the immune response without altering the specificity of it

318
Q

How does alum work as an adjuvant and why is it a good adjuvant?

A
  • Provides slow release antigen to help prime the immune response
  • Activates Gr1+ cells to produce IL-4 -> helps prime naive B cells
  • Generally safe, and mild, so is commonly used
319
Q

What is CpG and how does it work as an adjuvant?

A
  • Unmethylated motif with 2 purines at the 5’ end and 2 pyrimidines at the 3’ end
  • Acts as an immunostimulatory adjuvant
  • Activates TLRs on APCs stimulating expression of costimulatory molecules
320
Q

What is Complete Freund’s adjuvant and what is it used for?

A

Water-in-oil emulsion containing mycobacterial cell wall components - mainly for animals, painful in humans

321
Q

What is ISCOMS (immune staining complex)?

A

Experimental multimeric antigen with adjuvant built in

322
Q

What is HBIG?

A

Hep B immunoglobulin

323
Q

What is HRIG?

A

Human rabies immunoglobulin

324
Q

What is HNIG?

A

Human normal immunoglobulin - Hep A and measles

325
Q

What is daviluzimab?

A

Monoclonal antibody for RSV

326
Q

How is the mantoux test done?

A
  • Inject 0.1ml of 5 tuberculin units intradermally, examine arm 48-72 hours later
  • Positive result is indicated by redness and an induration (swelling that can be felt) of at least 10mm in diameter
  • Implies previous exposure to tuberculin protein - could represent previous BCG exposure

Path - Sarah (2 decks)

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