Immunology Flashcards
What IL does anakinra block?
IL-1
What IL does Sekukinumab block?
IL17
How do toll like receptors induce an immune response?
Act via NF-kappa-B which causes release of cytokines/chemokines and expression of costimulatory molecules
What are intracellular TLR?
TLR3,7-9 (recognise DNA/RNA of engulfed pathogens)
What is TLR4s role in sepsis?
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
What are the common pro-inflammatory cytokines?
IL-1, IL-6, TNF
What does Tocilizumab block?
IL-6
What is one of the main role sof PAMPs and PRR?
Upregulation of costimulation receptors (B7-CD28)
Deficiency of MAC part of complement makes pts more susceptible to what?
Neisseria infections
What do the C1-3 complement component deficiencies lead to?
Immune complex disease - SLE, GN, Vasculitis
What is deficient in angioedema?
C1esterase inhibitor
What is the major mediator in C1esterase inhibitor deficiency?
Bradykinin (hereditary angioedema)
What is the pattern of inheritance of hereditary angioedema?
AD
What test is used to differentiate heredutary from acquired C1 inhibitor deficiency?
C1q levels - depleted in acquired (where complement is consumed by another deisorder e.g MGUS or lymphoma)
What is icatibant?
Bradykinin-2 receptor antagonist - used in hereditary angioedema
What is chronic granulomatous disease?
Deficiency of subunit of NADPH oxidase - leading to recurrent infections. - Test with NBT test ( to check for oxygen metabolism)
What is seen on mature B-cell surface compared to immature?
IgM AND IgD (only IgM on immature)
Recurrent sinupulmonary and gut infections (sinusitis, bronchtis, tonsilitis, otitis media) are signs of what?
Lack of antibody response - as in CVID
What are needed for diagnosis of CVID
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
What are non-infection features of CVID?
Autoimmunity (ITP, AIHA), vitiligo, Cancers - gastric, lympohoma
How do you treat CVID?
IgG regularly, prompt treatment of infection, no live vaccines
X-linked Agammaglubulinaemi is secondary to what?
BTK mutation - Brutons TYR kinase
What are clinical features of X-linked agammablobulinaemia
Recurrent infections from age ~6 months
B cell levels are ~0 in what ?
X-linked agammaglobulinaemia
What is eculizumab?
Anticomplement MAB - used in PNH
What E.coli subtype is implicated in HUS?
E.coli 0157:H7 + shiga toxin producing E.coli
How doe Shiga toxin cause HUS?
Binds to glucophingolipid receptor activating endothlin 1 -> vasoconstriction -> acure renal failure
Also causes neutrophil activation which damaged glomululaer cells and form thrombus
What is
What is anatercept?
Anti B-7
What is polyglandular syndrome 1?
AIRE gene - Autoimmune Regulator gene.
AIRE mutated in this leading to recurrent candida and hypoparathyroid and adrenal insufficiency
What is polyglandular syndrome 2?
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
Ustekinumab?
IL12, IL23 inhibitor
What protein is overactive in familial mediterranean fever?
Pyrin
What are high risk HLA for T1DM?
HLA DR4-DQ8 and/or DR3-DQ2
What is the mechanism of GVHD?
Graft T cell sensitisation against host MHC antigens
What goes ani-GBM antibody attack?
alpha-3 subunit of type IV collagen in the GBM
What meds cause Jak-kinase inhibiotion?
Tofacitinib, baricitinib
How does natalizumab work??
Anti-alpha 4 interrin
What infections are more common with proteasone inhibitor use? (bortezumib)
HSV, resp tract, influ complications
What is the MoA of Abatercept?
Costimulation blockade of CD28
What cell has CD14?
Macrophage-monocyte lineage
What cells have MHC I?
ALL cells (except RBC and some neurons)
What are the features of bechets¿
Turkish people
Autoimflammatory vasculitis
Apthous ulcers recurrent,
Occular inflammation, genital ulcers, erythema nodosum, pustules
VTE
Tx with colcicine + steroids
What are the features of PAN?
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
What results in HyperIgM?
Mutation in CD40-CD40L costimulatory pathway
- reults in no T cell activation og B cells => they can only make IgM
What is caused by mutation in britons tyrosine kinase?
X-linked Agammaglobulinaemia
What are the features of Haemophagocytic lymphohistiocytosis:
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)
