Immunology Flashcards

1
Q

What does IRAK 4 deficiency lead to?

A

Recurrent pyogenic infections due to down regulation of the NF Kappa B signalling from TLR.

Distal to Toll Like Receptor 4/7/8/9

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2
Q

Where are the different types of Pattern Recognition Receptors of the innate immune system found?

A
Toll Like Receptors (membrane bound and intracellular) 
Inflammasomes (cytosol) 
C-type lectin receptors 
Nod Like receptors (Intracellular)
Rig like receptors (Intracellular)
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3
Q

U1RNP association

A

Mixed connective tissue disorder

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4
Q

ANA negative Ribo P positive

A

Lupus

Often quoted as being associated with cerebral lupus/neuropsychiatric lupus

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5
Q

RNA polymerase 3 association

A

Impending scleroderma renal crisis

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6
Q

RNA polymerase antibodies detectable in Scleroderma

A
RNAP III (worse prognosis) 
or
CEN-P
or
or ScL-70

Mutually exclusive

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7
Q

IL-4

A

Atopy
Exczema
Asthma

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8
Q

IL-12

A

Inflammatory

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9
Q

IL-10

A

Anti-inflammatory

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10
Q

Interferon Gamma

A

Th1/CD4 mediated

- Interferon gamma (drives B cells)

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11
Q

Regulatory t cells (CD4+/CD25+)

A

Govern peripheral tolerance

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12
Q

SMA (Smooth muscle antibody) associated with

A

Autoimmune hepatitis

Weak SMA in other liver disorders ie

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13
Q

Biomarker for autoimmune hepatitis activity

A

IgG (often elevated)

Transaminases

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14
Q

Autoimmune hepatitis types

A

Type 1: SMA (adult, often do better)
Type 2: ALKM (children, often do worse)

Lymphoplasmacytic infiltrate around the triad (interface)

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15
Q

Marker of PBC

A

Anti-mitochondrial antibody

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16
Q

Treatment for AIH

A

Induction: Steroids
Continuation: Azathioprine if required

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17
Q

Treatment for PBC

A

Urso

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18
Q

Anti-phospholipid syndrome risk factors for developing new thrombotic events

A

Recent discontinuation of anti-coagulation (particularly if previous clot as vein damaged)

Prothrombotic rank of antibodies: Cardiolipin –> Beta2 –> Lupus anticoagulant
All three = greatest risk

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19
Q

Treatment for chronic idiopathic urticaria

A

Antihistamine
Methotrexate
Monteleukast
Chronic and does not respond to the above: Omalizumab

20
Q

Indications for Omalizumanb

A

1) Maintenance treatment of moderate-to-severe allergic asthma in patients treated with inhaled corticosteroids and with raised serum IgE levels
2) Chronic spontaneous urticaria inadequately controlled with antihistamines

21
Q

What does B7 on an APC bind to on a T-Cell

A

CD28

22
Q

Two main cytokines produced by TH2 ells

A

1) IL 4 –> B cell to produce IgE

2) IL 5 –> Eosinophils

23
Q

Genetic mutation associated with systemic mastocytosis

A

KIT (gain of function mutation)

24
Q

Genetic mutation associated with HAE

A

C1INH (SERPING 1)

25
Q

Basal tryptase levels in systemic mastocytosis

A

> 20ng/ml

26
Q

Main differentiating clinical feature between HAE and Systemic Mastocytosis?

A

Urticaria

27
Q

Treatment for systemic mastocytosis

A

Acute: IM Adrenaline 0.3mg of 1:1000 solution
Chronic: Midostaurin, Cladribine or antihistamines

28
Q

When does Tryptase peak post anaphylaxis?

A

1-2 hours (should do serial measurement at 2hr–>24hr –> discharge).

Follow initial tryptase with a convalescent sample of tryptase to rule out systemic mastocytosis

Nb most useful in insect venom and medication-related anaphylaxis

29
Q

Mechanism underpinning hereditary HAE

A

C1 esterase inhibitor deficiency/dysfunction

30
Q

Diagnostic test for HAE

A

If clinical suspicion low: C4 level (excluded in normal)

If clinical suspicion high: C1 esterase inhibitor level and C4 level

30
Q

Diagnostic test for HAE

A

If clinical suspicion low: C4 level (excluded in normal)

If clinical suspicion high: C1 esterase inhibitor level and C4 level

31
Q

Diagnosis of CVID

A

Hypogammaglobulinaemia with severely reduced IgG +/- IgA +/- IgM (but with normal B-Cell levels)
Recurrent infections
Absence of antibody response to vaccination (impaired functional antibody response)

32
Q

Treatment for CVID

A

1) Gammaglobulin
2) Avoid live vaccines
3) Early and prolonged treatment of infections

33
Q

Pathogenesis of X-linked agammaglobulinaemia (Bruton’s agamagloublinaemia)

A

Absence of Brutons tyrosine kinase which is a signal molecule necessary for B-cell development (i.e the B-cell development signal is not propagated)

34
Q

Diagnosis of X-linked agammaglobulinaemia

A

EPG –> Hypogammaglobulinaemia
IgG level –> Undetectable
B cell count –> 0
Tissue biopsy –> No plasma cells or germinal centres

35
Q

Cardinal manifestations of Autoimmune Polyendocrine Syndrome Type 1

A

Chronic Mucocutaneous Candidiasis
Autoimmune Hypoparathyroidism
Autoimmune Addisons Disease

36
Q

Likely defect in recurrent fungal and staphylococcal infections

A

Phagocytes defect

i.e. Chronic Granulomatous Disease that affects phagocytes ability to perform the oxidative burst
Dx: Abnormal neutrophil oxidative burst

37
Q

Typical features of complement pathway deficiencies

A

1) Encapsulated bacterial infection (esp Neisseria): terminal pathway/MAC complex deficiency
2) Bacterial infections when additional cause of immunocompromised: Mannose binding lectin pathway deficiency

38
Q

Management of patients with complement deficiency

A

Vaccinate against all encapsulated organisms

Lifelong penicillin prophylaxis to prevent meningococcal disease

39
Q

Most common infections in primary antibody deficiency

A

Streptococcus pneumonia and Haemophilus influenzae

40
Q

Infections commonly associated with primary t-lymphocyte deficiencies

A

Viral, protozoal and fungal infections

41
Q

Likely deficiency in recurrent oral candidiasis?

A

TH17 and or Il-17 deficiency

42
Q

Mechanism of Eculizumab

A

Prevents C5 from splitting into C5a and C5b

significant risk of Neisseria infection - need to vaccinate first

43
Q

Mechanism of Avacopan

A

C5a receptor blocker (theoretically avoids the Neisseria risk associated with Ecalizumab)

44
Q

Where do you find MHC II molecules?

A

Antigen presenting cells: Dendritic cells, macrophages and B cells

45
Q

Which cytokine do T reg cells produce

A

IL -10
TGF-Beta

“immunosuppressive cytokines”