Hypersensitivity Disorders Flashcards

1
Q

immediate response (within 2 hours) provoked by re-exposure to an allergen. IgE mediated, resulting in mast cell degranulation which affects at least 2 organ systems.

A

Type I hypersensitivity

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2
Q

most prevalent allergic disorder in the UK

A

Asthma

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3
Q

Delayed, T-cell mediated reaction.

A

Type IV

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4
Q

defects in B-defensin and fillagrin. allergens can include irritants, foods and envrionmental factors. can predispose to S.Aureus superinfection. clinical diagnosis, usually <1yo.

A

Atopic dermatitis

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5
Q

gold standard test for IgE food allergy

A

Food challenge in hospital

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6
Q

exposure to allergens causes peri-oral tingling. Allergens usually include: birch pollen and rosacea fruit, mugwort and celery. 2% anaphylaxis

A

oral allergy syndrome. Diagnose by SPT

Rx: wash out mouth and oral antihistamine

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7
Q

Type 1 hypersens to chestnut, avocado, banana, kiwi, aubergine, melon, wheat.

A

Latex food syndrome

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8
Q

wheals which resolve within 6 weeks. 50% idiopathic. can be related to drugs, latex, viral infections.

A

Acute urticaria

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9
Q

Criteria for diagnosis of anaphylaxis (5)

A
  1. sudden onset and rapid progress,
  2. life-threatening compromise of A +/- B +/- C,
  3. skin changes - urticaria, angioedema,
  4. D, V, cramps, sense of doom
  5. Hx of exposure to allergen
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10
Q

IgE-mediated mast cell degranulation

A

peanut, penicillin, stings, latex

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11
Q

Non-IgE mediated mast cell degran

A

NSAIDS, IV contrast, opiods, exercise

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12
Q

Management of anaphylaxis in order (8)

A

1) Help 2) elevate legs 3) 100% O2 4) IM adrenaline 500mcg 5) inhaled bronchodilators 6) IV hydrocortisone 100mg 7) chloramphenamine 10mg IV 8) IV fluids

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13
Q

definition of positive SPT

A

wheal which is >= 2mm greater than the negative control

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14
Q

Gold standard to support diagnosis of allergy (not food)

A

SPT. remember to stop antihistamines 48hr before test

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15
Q

Indications for RAST

A

cant stop antihistamines, anaphylaxis hx, extensive eczema making test interpretation difficult

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16
Q

Component resolved diagnostics

A

measuring the IgE response to a SPECIFIC allergen protein

i.e: Ara-h-2 high risk allergen in peanuts, anaphylaxis

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17
Q

What to measure during an acute episode to determine if allergic/anaphylactic reaction:

A

Mast cell tryptase - evidence of mast cell degran

measure at 1hr, 3hr and 24hr - rise proportional to drop in BP

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18
Q

epipen dose for adults

A

300ug

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19
Q

epipen dose for children

A

150ug

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20
Q

presents in neonates with IgG-mediated reticulocytosis and anameia. Diagnosis made via positive DAT test

A

HDN. Rx with maternal plasma exchange, exchange transfusion in utero. prevent with anti-D. Type 2 HS

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21
Q

destruction of red blood cells by auto-antigens. Positive DAT.

A

AIHA. Type II

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22
Q

Evans syndrome

A

AIHA + ITP. Type II

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23
Q

anti-platelet antibodies against glp IIb/IIIa. presents with purpura, bruising, bleeding.

A

Autoimmunhe thrombocytopenic purpura

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24
Q

glomerulopnephritis, pulmonary haemorrhage. linear smooth immunoflurescence staining shows IgG deposits on the BM.

A

Goodpastures. anti-GBM

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25
Q

IgG/IgM which react with self antigens, resulting in tissue damage or receptor activation/blockade

A

Type II Hypersensitivity

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26
Q

antibodies against epidermal cadherin.

direct immunofluorescence shows IgG deposition.

A

Pemphigus Vulgaris. non-tense blistering of the skin and bullae

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27
Q

anti-TSHR abs

A

Grave’s disease

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28
Q

Treatment of Grave’s disease

A

Carbimazole and propylthiouracil

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29
Q

anti-AChR abs. fatiguable muscle weakness, double vision. Tensilon test shows improvement in fatiguability

A

MG

30
Q

Rx of MG

A

Neostigmine, pyridostigmone

31
Q

antibodies to the M proteins on GAS, can cross react with cardiac Myosin protein. Dx using Jones criteria

A

Acute rheumatic fever

32
Q

Major criteria of Jones criteria for RF

A
Sydenhams chorea
Transient arthritis
Rheumatoid nodules
Erythema marginatum
Pancarditis
33
Q

anti-intrinsic factor abs (50% specific), anti-gastric parietal cell ab (90% specific). causes megaloblastic anaemia

A

Pernicious anaemia

34
Q

medium and small vessel vasculitis with an allergic prodrome and eosinophilia. Granulomas.

A

eGPA (Churg strauss)

p-ANCA against MYELOPEROXIDASE

35
Q

medium and small vessel vasculitis that presents with sinusitis, pulmonary cavitations and haemorrhage, crescenteric glomerulonephritis.

A

GPA (Wegeners)

c-ANCA against PROTEINASE 3

36
Q

Rx for condition with p-ANCA against MYELOPEROXIDASE

A

P-ANCA Rx = PA ‘n’ C
Pred
Azathioprine
Cyclophosphamide

37
Q

Rx for condition with c-ANCA against PROTEINASE 3

A

c-ANCA Rx = CCC
Corticosteroids
cyclophosphamide
co-trimoxazole

38
Q

pauci-immune necrotising small vessel vasculitis, presents with livedo, purpura. many organs affected.

A

microscopic polyangiitis

p-ANCA +ve

39
Q

itchy wheals lasting >6 weeks. can be assoc with NSAIDS, cold, food, perssure, sun. associated with angioedema in 50% of cases.

A

Chronic urticaria

IgG against FcER1

40
Q

Tissue damage mediated by IgG or IgM immune complexes (abs against soluble antigens)

A

Type III

41
Q

Hypersens condition in which antibodies directed at intracellular proteins such as nuclear ag (anti dsDNA - 95% specific, anti-histones) and cytoplasmic ag (anti-Ro, La and Sm).
ab-ag complexes activate complement cascade via classical pathway and lead to C4 (+/- C3) depletion.

A

SLE

42
Q

anti-CCP antibodies (95% specific) form immune complexes with citrullinated proteins and deposit, trigger complement.

A

RA

43
Q

joint pain, splenomeg and skin/nerve involvement. associated with Hep C.

A

Mixed Essential Cryoglobulinaemia. IgM against IgG +/- hepatitis C ags
Cryo = C = Hep C = Chronic

44
Q

Exposure to anti-serum i.e penicllin, causes rash arthralgia itching LN fever malaise. occurs 7-12d post-exposure. Decreased C3 levels.

A

Serum sickness. Rx with steroids, antihistamines and stop the precipitant.

45
Q

fever, fatigue, weakness, arthralgia, MI, pericarditis, kidney involvement. associated with Hep B. Rosary sign.

A

Polyarteritis Nodosa. ab against hep B viral antigens

46
Q

anti-GAD abs. CD8+-mediated destruction

A

T1DM. also anti-ilset abs, anti-insulin abs

47
Q

abs against antigens in the synovial membrane. RF+ve, (85%) anti-CCP (95%), raised CRP and ESR

A

RA

48
Q

Itching, blisters and wheals, usually short lived. can be triggered by chemicals, poison ivy, nickel.

A

Contact demratitis - patch test to dx

49
Q

Type IV hypersensitivity, used in testing for mycobacterium

A

Mantoux

50
Q

Diarrhoea, bloating, blood in stool. Th1 mediated, associated with NOD2 gene mutation in 30%. Dx using biopsy.

A

Crohn’s. Chronic inflammation and skip lesions.

51
Q

HLA association for Ank Spond

A

HLA-B27 - By the time youre 27 you’ll know you have Ank Spond

52
Q

HLA-DR3

A

T1DM, Graves, SLE

53
Q

T1DM

A

HLADR3/DR4 - present when youre 3-4

54
Q

Goodpastures

A

HLADR-15/DR2 - kids get it - CPR = 15:2

55
Q

HLA-DR1/DR4

A

RA

56
Q

SLE

A

HLA-DR3 (SLE is 3 letters)

57
Q

PTPN22 genetic polymorphism associated with:

A

RA, SLE, T1DM (P(Q)RST)

58
Q

CTLA4 genetic polymorphism

A

receptor for CD80-86. assoc w AI thyroid disease, SLE,, T1DM

59
Q

Calcinosis, Raynauds, Esophageal dysmotility, sclerodactyly, telangiectasia. skin involvement on the forearms and perioral ONLY. associated with anti-centromere abs.

A

Limited cutaneous systemic sclerosis

60
Q

anti-Scl70, anti-RNA Pol, anti-fibrillarin. extensive skin disease, GI disease, interstitial pulmonary disease and sclerdermic kidney crises.

A

Diffuse cutaneous SS

61
Q

Low back pain, enthesitis, large joint arthritis, uveitis, stiffness. young males.

A

Ank Spnod HLA B27

62
Q

immune complex mediated vasculits. Perivascular CD4 cells. proximal muscle weakness and Gottron’s papules. ANA +ve, anti-Mi2, Jo-1 abs.

A

Dermatomyositis - muscles weakeneD

63
Q

Type IV hypersens, CD8 cells surround and destroy HLA I muscle fibres via perforin. Loss of muscle mass, interstitial lung disease, cardiac disease. anti-SRP, anti-Mi2.

A

Polymyositis

64
Q

periodic fevers lastimh 48-90 hours, chest pain (pleursy/pericarditis), abdo pain (peritonitis), arthritis and skin involvement. Can develop amyloidosis in long term.
MEFV gene defect
sephardic jews, arabs, turks

A

familial mediterranean fever

65
Q

Rx for FMF

A

1) colchicine - binds to tubulin of neutrophils and disrupts chemokine action and migration.
2) TNF-a/IL-1 inhibitors - etanercept, anakira

66
Q

CARD15 gene on chromosome 6

A

Crohns. also known as NOD2 gene

67
Q

mutation within Fas pathway (Fas-ligand) leading to defects in cell apoptosis, lymphocytosis, LN and HSM.

A

Autoimmune lymphoproliferative syndrome

68
Q

defect in AIRE gene, AR. leads to failure of central tolerance - auto-abs form against endocrine glands. hypoparathyroidism, addisons….
also have ab against IL-17 and IL-22 leading to candidiasis.

A
APECED
Autosomal recessive
Polyendocrinopathy
Candidiasis
Ectodermal Dystrophy
Also called APS1
69
Q

X-linked mutations in the FOXP3 genes needed to make CD4+ Treg cells. male infants, eczema, nail dystrophy, AI skin disease, multiple endocrinopathies, immune dysfunction and diarrhoea. usually die within first 2 years of life if no BMT.

A
IPEX
Immune dysfunction
Polyendcrinopathy
Enteropathy
X-linked recessive
70
Q

Dry mouth, dry eyes, dry nose, dry skin. F, late 40s. Can also affect kidneys, blood vessels, lungs, liver, pancreas, peripheral nervous system. Anti-Ro and La positive.
Schirmers test
Parotid or salivary gland enlargement.

A

Sjogrens

71
Q

associated with polymorphisms in PAD 2 and 4 enzymes, and infection with P.Gingivalis

A

RA

72
Q

CD25+, Foxp3+, CD4+ T reg cells secrete:

A

TGF-B, IL-10. Deficiency of these T reg cells due to lack of any of the above genes (i.e FOXP3) leads to dysfunction in peripheral tolerance = IPEX.