Immunology

Question 1

What IL does anakinra block?

Answer 1

IL-1

Question 2

What IL does Sekukinumab block?

Answer 2

IL17

Question 3

How do toll like receptors induce an immune response?

Answer 3

Act via NF-kappa-B which causes release of cytokines/chemokines and expression of costimulatory molecules

Question 4

What are intracellular TLR?

Answer 4

TLR3,7-9 (recognise DNA/RNA of engulfed pathogens)

Question 5

What is TLR4s role in sepsis?

Answer 5

in Gram - bacteraemia there is widespread lipopolysaccaride that activates TLR4 to stimulate cytokine release throughout the body leading to shock.
TLRs are meant to only be a localised reaction but lead to shock in disseminated infeciton

Question 6

What are the common pro-inflammatory cytokines?

Answer 6

IL-1, IL-6, TNF

Question 7

What does Tocilizumab block?

Answer 7

IL-6

Question 8

What is one of the main role sof PAMPs and PRR?

Answer 8

Upregulation of costimulation receptors (B7-CD28)

Question 9

Deficiency of MAC part of complement makes pts more susceptible to what?

Answer 9

Neisseria infections

Question 10

What do the C1-3 complement component deficiencies lead to?

Answer 10

Immune complex disease - SLE, GN, Vasculitis

Question 11

What is deficient in angioedema?

Answer 11

C1esterase inhibitor

Question 12

What is the major mediator in C1esterase inhibitor deficiency?

Answer 12

Bradykinin (hereditary angioedema)

Question 13

What is the pattern of inheritance of hereditary angioedema?

Answer 13

AD

Question 14

What test is used to differentiate heredutary from acquired C1 inhibitor deficiency?

Answer 14

C1q levels - depleted in acquired (where complement is consumed by another deisorder e.g MGUS or lymphoma)

Question 15

What is icatibant?

Answer 15

Bradykinin-2 receptor antagonist - used in hereditary angioedema

Question 16

What is chronic granulomatous disease?

Answer 16

Deficiency of subunit of NADPH oxidase - leading to recurrent infections. - Test with NBT test ( to check for oxygen metabolism)

Question 17

What is seen on mature B-cell surface compared to immature?

Answer 17

IgM AND IgD (only IgM on immature)

Question 18

Recurrent sinupulmonary and gut infections (sinusitis, bronchtis, tonsilitis, otitis media) are signs of what?

Answer 18

Lack of antibody response - as in CVID

Question 19

What are needed for diagnosis of CVID

Answer 19

Marked decreased IgG +/- low IgA +/- Low IgM
- on 2 occasions
AND one of
- Poor antibody response to vaccination
IgG <2 and delay to proviginf Ig replacement
Low switched memory B cells
Absent isohaemagglutinins AND be >4y/o

Question 20

What are non-infection features of CVID?

Answer 20

Autoimmunity (ITP, AIHA), vitiligo, Cancers - gastric, lympohoma

Question 21

How do you treat CVID?

Answer 21

IgG regularly, prompt treatment of infection, no live vaccines

Question 22

X-linked Agammaglubulinaemi is secondary to what?

Answer 22

BTK mutation - Brutons TYR kinase

Question 23

What are clinical features of X-linked agammablobulinaemia

Answer 23

Recurrent infections from age ~6 months

Question 24

B cell levels are ~0 in what ?

Answer 24

X-linked agammaglobulinaemia

Question 25

What is eculizumab?

Answer 25

Anticomplement MAB - used in PNH

Question 26

What E.coli subtype is implicated in HUS?

Answer 26

E.coli 0157:H7 + shiga toxin producing E.coli

Question 27

How doe Shiga toxin cause HUS?

Answer 27

Binds to glucophingolipid receptor activating endothlin 1 -> vasoconstriction -> acure renal failure Also causes neutrophil activation which damaged glomululaer cells and form thrombus

Question 28

What is

Question 29

What is anatercept?

Answer 29

Anti B-7

Question 30

What is polyglandular syndrome 1?

Answer 30

AIRE gene - Autoimmune Regulator gene. AIRE mutated in this leading to recurrent candida and hypoparathyroid and adrenal insufficiency

Question 31

What is polyglandular syndrome 2?

Answer 31

Common phenotype of autoimmune thyroid involvement, addisons disease as well as T1DM and coeliac disease in 20% of cases. Polygenic association with HLADR3 and 4

Question 32

Ustekinumab?

Answer 32

IL12, IL23 inhibitor

Question 33

What protein is overactive in familial mediterranean fever?

Answer 33

Pyrin

Question 34

What are high risk HLA for T1DM?

Answer 34

HLA DR4-DQ8 and/or DR3-DQ2

Question 35

What is the mechanism of GVHD?

Answer 35

Graft T cell sensitisation against host MHC antigens

Question 36

What goes ani-GBM antibody attack?

Answer 36

alpha-3 subunit of type IV collagen in the GBM

Question 37

What meds cause Jak-kinase inhibiotion?

Answer 37

Tofacitinib, baricitinib

Question 38

How does natalizumab work??

Answer 38

Anti-alpha 4 interrin

Question 39

What infections are more common with proteasone inhibitor use? (bortezumib)

Answer 39

HSV, resp tract, influ complications

Question 40

What is the MoA of Abatercept?

Answer 40

Costimulation blockade of CD28

Question 41

What cell has CD14?

Answer 41

Macrophage-monocyte lineage

Question 42

What cells have MHC I?

Answer 42

ALL cells (except RBC and some neurons)

Question 43

What are the features of bechets¿

Answer 43

Turkish people Autoimflammatory vasculitis Apthous ulcers recurrent, Occular inflammation, genital ulcers, erythema nodosum, pustules VTE Tx with colcicine + steroids

Question 44

What are the features of PAN?

Answer 44

Arteritis Ishchaemia with infarct No GN, lung haemorrhage, No ANCA Ass Hep B Steroids Subacute over weeks-months Tx with steroids Doens't relapse/uncommonly

Question 45

What results in HyperIgM?

Answer 45

Mutation in CD40-CD40L costimulatory pathway - reults in no T cell activation og B cells => they can only make IgM

Question 46

What is caused by mutation in britons tyrosine kinase?

Answer 46

X-linked Agammaglobulinaemia

Question 47

What are the features of Haemophagocytic lymphohistiocytosis:

Answer 47

Defectve NK cells and CD8 T cells. Can be triggered by viruses eg EBV, CMV, malignancy, Fevers, hepatosplenomegaly, high ferritin, cytopenia, high LDL, TGL, Bili Hamophagocytosis (phagocytosis of BM)