Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Year 5 > Pathology - Immunology > Flashcards

Pathology - Immunology Flashcards

1
Q

What antibodies might you test for when suspecting SLE?

A

Anti nuclear antibodies - first and more vague test

Anti double strand antibodies - more specific for SLE

Antihistone antibodies - drug induced SLE (stop the drug)

Anti smith - most specific for SLE (Smith-Specific)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What antibodies would you test for in suspected systemic sclerosis (scleroderma) ?

A

Diffuse form = anti DNA topoisomerase (scl 70)

Limited form (CREST) = anti centromere

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is immuno pathology?
A. Reflects genetic abnormality affecting the innate immune system, often in a site-specific manner

B. May describe damage resulting from the immune response to ongoing infection

C. Reflects genetic abnormality affecting the adaptive immune system and is often associated with presence of auto-antibodies

A

B. May describe damage resulting from the immune response to ongoing infection

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is autoimmune disease?
A. Reflects genetic abnormality affecting the innate immune system, often in a site-specific manner

B. May describe damage resulting from the immune response to ongoing infection

C. Reflects genetic abnormality affecting the adaptive immune system and is often associated with presence of auto-antibodies

A

C. Reflects genetic abnormality affecting the adaptive immune system and is often associated with presence of auto-antibodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is autoinflammatory disease?
A. Reflects genetic abnormality affecting the innate immune system, often in a site-specific manner

B. May describe damage resulting from the immune response to ongoing infection

C. Reflects genetic abnormality affecting the adaptive immune system and is often associated with presence of auto-antibodies

A

A. Reflects genetic abnormality affecting the innate immune system, often in a site-specific manner

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Give an example of monogenic auto-inflammatory disease

A

Familial mediterranean fever

TRAPS

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Give 3 examples of polygenic autoinflammatory diseases

A 
Crohn's disease
Ulcerative colitis
OA
giant cell arteritis
Takayasu's arteritis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Give 2 examples of mixed pattern disease

A

Ankylosing spondylitis
Psoriatic arthritis
Bechet’s syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Give 5 examples of polygenic autoimmune disease

A 
RA
myasthenia gravis
pernicious anaemia
Graves disease
SLE
primary biliary cirrhosis
ANCA associated vasculitis
Goodpasture disease
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Give an example of rare monogenic autoimmune disease

A

APS-1 (autoimmune polyendocrine syndrome type 1), APECED (autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy syndrome)
ALPS (autoimmune lymphoproliferative syndrome)
IPEX (immune dysregulation, polyendocrinopathy, enteropathy X-linked syndrome)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Monogenic autoinflammatory disease occurs as a result of abnormalities in which pathways?

A

Pathways associated with innate immune cell function eg cytokine pathways involving TNF/IL-1 common

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Familial Mediterranean fever - what is the pathogenesis?

A

Mutation in MEFV gene, encoding pyrin-marenostrin (expressed mostly in neutrophils)
Failure to regulate cryopyrin driven activation of neutrophils
Autosomal recessive
(CF-THE INFLAMMASOME COMPLEX)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Familial Mediterranean fever - epidemiology?

A

sepharic>ashkenazi Jews

armenian, Turkish, Arabs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Familial Mediterranean fever - presentation?

A

periodic fevers lasting 48-96 hours with:

  • abdo pain due to peritonitis
  • chest pain due to pleurisy and pericarditis
  • arthritis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Familial Mediterranean fever - complications?

A

long term risk of amyloidosis
Nephrotic syndrome
Renal failure

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Familial Mediterranean fever - treatment?

A

COLCHICINE 500ug bd (binds to tubulin in neutrophils and disrupts neutrophil functions including migration and chemokine secretion)
ANAKINRA (IL1 R anatagonist)
ETANERCEPT (TNF alpha inhibitor)
TYPE 1 INTERFERON

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Monogenic autoimmune disease occurs as a result of abnormalities in which pathways?

A

Adaptive immune cell function:

  • abnormality in tolerance
  • abnormality of regulatory T cells
  • abnormality of lymphocyte apoptosis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

APS1 and APECED:

  • 1) pathogenesis?
  • 2) presentation?
  • 3) full name?
A
  • 1) autosomal recessive disorder
  • defect in AutoImmune REulator (AIRE), TF involved in development of T-cell tolerance in thymus
    • ->upregulates expression of self-antigens by thymic cells
    • -> promotes T cell apoptosis
  • antibodies vs parathyroid and adrenal glands –> hypoPTH + Addison’s
  • 2) mild immune deficiency eg Candida infections
  • 3) autoimmune polyendocrine syndrome type 1;
  • autoimmune polyendocrine candidiasis ectodermal dystrophy syndrome
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

IPEX:

  • 1 pathogenesis?
  • 2 presentation?
  • 3 full name?
A
  • mutation in FOXP3 (required for development of Treg cells)
  • 2 endocrinopathy (DMT1, thyroid disease)
  • diarrhoea
  • eczematous dermatitis
  • 3 immune dysregulation, polyendocrine enteropathy X-linked syndrome
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

ALPS:

  • 1 pathogenesis?
  • 2 presentation?
  • 3 full name?
A
  • 1 mutations within FAS pathway (eg mutations in TNFRSF6 which encodes FAS)
  • disease is heterogeneous depending on the mutation
  • defect in apoptosis of lymphocytes, failure of tolerance, failure of lymphocyte homeostasis
  • 2 high lymphocyte numbers with large spleen and lymph nodes
  • associated with lymphoma
  • 3 autoimmune lymphoproliferative syndrome
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

IPEX: (+what does it stand for?)

A. Single gene mutation involving MEFV and affecting the inflammasome complex, resulting in recurrent episodes of serositis

B. Mutation within the Fas pathway associated with lymphocytosis, lymphomas and auto-immune cytopenias

C. Single gene mutation involving FOXp3 resulting in abnormality of T reg cells

A

C. Single gene mutation involving FOXp3 resulting in abnormality of T reg cells

(Immune dysregulation polyendocrinopathy enteropathy X linked)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Familial Mediterranean fever:

A. Single gene mutation involving MEFV and affecting the inflammasome complex, resulting in recurrent episodes of serositis

B. Mutation within the Fas pathway associated with lymphocytosis, lymphomas and auto-immune cytopenias

C. Single gene mutation involving FOXp3 resulting in abnormality of T reg cells

A

A. Single gene mutation involving MEFV and affecting the inflammasome complex, resulting in recurrent episodes of serositis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

ALPS: (+what does it satnd for)

A. Single gene mutation involving MEFV and affecting the inflammasome complex, resulting in recurrent episodes of serositis

B. Mutation within the Fas pathway associated with lymphocytosis, lymphomas and auto-immune cytopenias

C. Single gene mutation involving FOXp3 resulting in abnormality of T reg cells

A

B. Mutation within the Fas pathway associated with lymphocytosis, lymphomas and auto-immune cytopenias

(autoimmune lymphoproliferative syndrome)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Crohn’s disease:

  • what genes?
  • what is the role of these genes?
A

polygenic autoinflammatory disease - local factors at sites predisposed to disease lead to activation of innate immune cells

IBD1-8 = regions associated with susceptibility
IBD1 gene on choromosome 16 = NOD2 –> associated with increased risk

also found in patients with severe psoriasis and psoriatic arthritis

NOD2 expressed in cytoplasm of myeloid cells (macrophages, neutrophils, dendritic cells)

  • act as microbial sensor (to muramyl dipeptide)
  • activates NFkB–>induce pro-inflammatory cytokine genes
  • regulates innate immune response to bacterial products
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Crohn’s disease

  • 1) pathogenesis?
  • 2) clinical features?
  • 3) treatment?
A

1) expression of pro-inflammatory cytokines + leukocyte recruitment
–> focal inflammation in crypts
–> formation of granulomata
release of proteases, free radicals, platelet activating factor
–> tissue damage with mucosal ulceration
2) abdo pain and tenderness
-diarrhoea (blood, pus, mucus)
-fevers, malaise
-oral ulcers, pyoderma gangrenosum, arthritis
-raised inflammatory markers
3) corticosteroids, azathioprine and TNF alpha antagonists

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Giant cell arteritis:

  • 1) presentation?
  • 2) investigations?
  • 3) treatment
A

1) temporal headache, claudication pain on chewing, visual loss reflecting involvement of opthalmic artery
2) high CRP and ESR
- abnormal temporal artery biopsy with intimal proliferation, disrupted internal elastic lamina, mononuclear cells throughout vessel wall
3) immunosuppression with corticosteroids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

distribution of giant cell arteritis?

A

temproal artery and opthalmic artery

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

giant cell arteritis:

-pathogenesis?

A
  1. dendritic cells in adventitia activate T cells
  2. T cells activate macrophages
  3. macrophages produce cytokines, tissue destructive enzymes (eg MMPs), and reparative factors leading to proliferation of intima (eg PDGF)
  4. proliferation if intima leads to disrupted internal elastic lamina leading to formation of giant cell
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

genetic polymorphisms in Giant cell arteritis?

A

TLR 4 - dendritic cell activation
IL6, IL8 IL10 gene promotor - cytokine expression
ICAMS1 - cell migration
MMP9 - tissue destruction
nitric oxide synthase - tissue destruction
MHC polymorphism - T cell activation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

ankylosing spondylitis

  • 1) 5 presentation?
  • 2) treatment?
A

1) low back pain and stiffness, symptoms worse after rest, pain and swelling usually affecting hips and kness, enthesitis, uveitis
2) NSAIDs, immunosuppression (TNF alpha antagonists)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

5 genetic polymorphisms in ankylosing spondylitis?

A

1) IL23R - IL23 receptor promotes differentiation of Th17 cells
2) ERAP1 (Type 1 TNF receptor shedding aminopeptidase regulator) - cleaves surface cytokine receptors and trims peptides for presentation by HLA1
3) ANTXR2 (anthrax toxin receptor 2) - involved in forming capillaries and maintaining BM structure
4) ILR2 - inhibits IL1 activity
5) HLA B27 - present antigen to CD8 T cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

anklosing spondylitis
A. Polygenic auto-inflammatory disease. ~30% patients have a mutation of CARD15 which may affect response of myeloid cells to bacteria.

B. Mixed pattern auto-inflammatory / auto-immune disease with >90% heritability that results in inflammation typically involving the sacro-iliac joints and responds to TNF alpha antagonists

C. Polygenic auto-inflammatory disease resulting in a large vessel vasculitis and requiring immediate treatment with high dose corticosteroids

A

B. Mixed pattern auto-inflammatory / auto-immune disease with >90% heritability that results in inflammation typically involving the sacro-iliac joints and responds to TNF alpha antagonists

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Crohn’s disease
A. Polygenic auto-inflammatory disease. ~30% patients have a mutation of CARD15 which may affect response of myeloid cells to bacteria.

B. Mixed pattern auto-inflammatory / auto-immune disease with >90% heritability that results in inflammation typically involving the sacro-iliac joints and responds to TNF alpha antagonists

C. Polygenic auto-inflammatory disease resulting in a large vessel vasculitis and requiring immediate treatment with high dose corticosteroids

A

A. Polygenic auto-inflammatory disease. ~30% patients have a mutation of CARD15 which may affect response of myeloid cells to bacteria.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

giant cell arteritis
A. Polygenic auto-inflammatory disease. ~30% patients have a mutation of CARD15 which may affect response of myeloid cells to bacteria.

B. Mixed pattern auto-inflammatory / auto-immune disease with >90% heritability that results in inflammation typically involving the sacro-iliac joints and responds to TNF alpha antagonists

C. Polygenic auto-inflammatory disease resulting in a large vessel vasculitis and requiring immediate treatment with high dose corticosteroids

A

C. Polygenic auto-inflammatory disease resulting in a large vessel vasculitis and requiring immediate treatment with high dose corticosteroids

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

Give 10 examples of polygenic autoimmune disease

A 
Graves disease
Hashimoto's thyroditis
DM
pernicious anaemia
AIHA
myasthenia gravis
Goodpasture disease
RhA
SLE
Sjogren's syndrome
systemic sclerosis
dermatomyositis
ANCA associated vasulitis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

3 considerations for polygenic autoimmune disease?

A

genetic factors
environmental factors
loss of tolerance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

HLA presentation of antigen is required for development of T cell and T cell-dependent B cell responses.
What susceptibility allele is Goodpasture disease associated with?

A

HLA-DR15

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

HLA presentation of antigen is required for development of T cell and T cell-dependent B cell responses.
What susceptibility allele is Graves disease associated with?

A

HLA-DR3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

HLA presentation of antigen is required for development of T cell and T cell-dependent B cell responses.
What susceptibility allele is SLE associated with?

A

HLA-DR3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

HLA presentation of antigen is required for development of T cell and T cell-dependent B cell responses.
What susceptibility allele is DMT1 associated with?

A

HLA-DR3/DR4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

HLA presentation of antigen is required for development of T cell and T cell-dependent B cell responses.
What susceptibility allele is RhA associated with?

A

HLA-DR4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

What is PTPN 22?

What diseases does the 1858T allele increase susceptibility of?

A

protein tyrosine phopshate non-receptor 22 –> lympocyte specific tyrosine phosphate which suppresses T cell activation
RhA, SLE, T1DM

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

What is CTLA4?

What diseases are allelic variants found in?

A

expreseed by T cells and transmits inhibitory signal to control T cell activation
SLE, T1DM, auto-immune thyroid disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

HLA DR4
A. Tyrosine phosphatase expressed in lymphocytes associated with development of auto-immune disease including rheumatoid arthritis

B. MHC class II molecule that is associated with development of auto-immune disease including rheumatoid arthritis

C. Receptor for CD80/CD86, expressed on T cells, that influences T cell activation and is associated with auto-immune disease including diabetes and thyroid disease

A

B. MHC class II molecule that is associated with development of auto-immune disease including rheumatoid arthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

PTPN22
A. Tyrosine phosphatase expressed in lymphocytes associated with development of auto-immune disease including rheumatoid arthritis

B. MHC class II molecule that is associated with development of auto-immune disease including rheumatoid arthritis

C. Receptor for CD80/CD86, expressed on T cells, that influences T cell activation and is associated with auto-immune disease including diabetes and thyroid disease

A

A. Tyrosine phosphatase expressed in lymphocytes associated with development of auto-immune disease including rheumatoid arthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

CTLA4
A. Tyrosine phosphatase expressed in lymphocytes associated with development of auto-immune disease including rheumatoid arthritis

B. MHC class II molecule that is associated with development of auto-immune disease including rheumatoid arthritis

C. Receptor for CD80/CD86, expressed on T cells, that influences T cell activation and is associated with auto-immune disease including diabetes and thyroid disease

A

C. Receptor for CD80/CD86, expressed on T cells, that influences T cell activation and is associated with auto-immune disease including diabetes and thyroid disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

Mechanisms of peripheral tolerance?

A

Anergy - by cells lacking co-stimulatory molecules (thus do not respond to subsequent challenge)
Regulation - by regulatory cell populations (eg T regs, Tr1 cells, CD8 reg T cells)
Immune privilege - lymphocytes denied entry (eg eye, testes, CNS)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

How can
-a) central tolerance?
-b) peripheral tolerance?
failure lead to autoimmune disease?

A

a) inappropriate survival of autoreactive B and T cells
b) aberrant expression of co-stimulatory molecules; decrease number/function of regulatory t cells; damage at immunologically privileged sites

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

central tolerance of T cells
A. T cells that express FoxP3 and CD25 and secrete cytokines IL-10, TGF beta to suppress activation of other T cells

B. Within the thymus cells that bind with low affinity to HLA molecules die by neglect and those that bind with high affinity to HLA molecules are deleted

C. T cells that recognise HLA/peptide complexes on cells that do not express co-stimulatory molecules subsequently fail to respond to stimulation with antigen

A

B. Within the thymus cells that bind with low affinity to HLA molecules die by neglect and those that bind with high affinity to HLA molecules are deleted

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

T reg cells

A. T cells that express FoxP3 and CD25 and secrete cytokines IL-10, TGF beta to suppress activation of other T cells

B. Within the thymus cells that bind with low affinity to HLA molecules die by neglect and those that bind with high affinity to HLA molecules are deleted

C. T cells that recognise HLA/peptide complexes on cells that do not express co-stimulatory molecules subsequently fail to respond to stimulation with antigen

A

A. T cells that express FoxP3 and CD25 and secrete cytokines IL-10, TGF beta to suppress activation of other T cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

T cell anergy:

A. T cells that express FoxP3 and CD25 and secrete cytokines IL-10, TGF beta to suppress activation of other T cells

B. Within the thymus cells that bind with low affinity to HLA molecules die by neglect and those that bind with high affinity to HLA molecules are deleted

C. T cells that recognise HLA/peptide complexes on cells that do not express co-stimulatory molecules subsequently fail to respond to stimulation with antigen

A

C. T cells that recognise HLA/peptide complexes on cells that do not express co-stimulatory molecules subsequently fail to respond to stimulation with antigen

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

Gel and Coombs classification: Type I?

a) delayed type hypersensitivity T-cell mediated response
b) antibody reacts with cellular antigens
c) immediate hypersensitivity which is IgE mediated
d) antibody reacts with soluble antigen to form an immune complex

A

c) immediate hypersensitivity which is IgE mediated

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

Gel and Coombs classification: Type II?

a) delayed type hypersensitivity T-cell mediated response
b) antibody reacts with cellular antigens
c) immediate hypersensitivity which is IgE mediated
d) antibody reacts with soluble antigen to form an immune complex

A

b) antibody reacts with cellular antigens

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

Gel and Coombs classification: Type III?

a) delayed type hypersensitivity T-cell mediated response
b) antibody reacts with cellular antigens
c) immediate hypersensitivity which is IgE mediated
d) antibody reacts with soluble antigen to form an immune complex

A

d) antibody reacts with soluble antigen to form an immune complex

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

Gel and Coombs classification: Type IV?

a) delayed type hypersensitivity T-cell mediated response
b) antibody reacts with cellular antigens
c) immediate hypersensitivity which is IgE mediated
d) antibody reacts with soluble antigen to form an immune complex

A

a) delayed type hypersensitivity T-cell mediated response

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

Type I hypersensitivity:

  • antibody type?
  • what occurs?
A 
IgE
Fc receptors on mast cells bind to IgE 
--> mast cell degranulation
--> release of inflammatory cytokines
results:
-increased vascular permeability
-leukocyte chemotaxis
-smooth muscle contraction
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

Mechanisms of type II sensitivity?

A

1) antibody dependent destruction (NK cells –> release of cytolytic granules and membrane attack, phagocytes –> phagocytosis, complement–> cell lysis)
2) receptor activation or blockade

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

mechanism of type III hypersensitivity?

A
  • immmune complex formation and depsoition in blood vessels
  • complement activation or infiltration of macrophages and neutrophils
  • cytokine/chemokine expression
  • granule release from neutrophils
  • increased vascular permeability
  • inflammation and damage to vessels (–> cutaneous vasculitis, glomerulonephritis, arthritis)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
59
Q

type IV hypersensitivity mechanism?

A

T cell mediated delayed hypersensitivity

  • CD8 cells can recognise self peptide and lyse MHC1 cells
  • CD4 cells can recognise self peptide from MHC2 cells and release IFNg to stimulate macrophages, which mediate tissue damage
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
60
Q

Type III hypersensitivity?
Goodpasture disease

Eczema

SLE

Multiple sclerosis

A

SLE

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
61
Q

Type I hypersensitivity
Goodpasture disease

Eczema

SLE

Multiple sclerosis

A

eczema

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
62
Q

Type IV hypersensitivity
Goodpasture disease

Eczema

SLE

Multiple sclerosis

A

Multiple sclerosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
63
Q

Type II hypersensitivity
Goodpasture disease

Eczema

SLE

Multiple sclerosis

A

Goodpasture disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
64
Q

Graves disease:

  • pathogenesis?
  • what type of hypersensitivity?
A
  • Type II hypersensitivity
  • IgG antibodies stimulate TSH receptor, acting as TSH agonists
  • ->induce uncontrolled overporduction of thyroid hormones
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
65
Q

Hashimoto’s thyroiditis:

  • hypersensitivity type?
  • pathogenesis?
A
  • Type II and type IV
  • enlarged goitre infiltrated by T and B cells
  • associated with anti-thyroid peroxidase antibodies (presence correlates with thyroid damage and lymphocyte infiltration)
  • associated with anti-thyroglobulin antibodies
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
66
Q

What autoantibodies exist in DMT1?

A

anti-islet cell antibodies
anti-insulin antibodies
anti-GAD (glutamic acid dehydrogenase) antibodies
anti-IA-2 (islet antigen 2) antibodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
67
Q

DMT1:

-hypersensitivity type?

A

type IV CD8 T-cell mediated (bind to MHC1 on beta cells of pancreatic islets cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
68
Q

pernicious anaemia:

  • type hypersensitivity?
  • pathogenesis?
  • leads to deficiency of what?
  • clinical features?
A
  • type II
  • antibodies to gastric parietal cells or intrinsic factor
  • failure of B12 absorption
  • neurological features with subacute combined degeneration of cord (posterior and lateral columns), peripheral neuropathy, optic neuropathy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
69
Q

myasthenia gravis:

  • hypersensitivity type?
  • pathogenesis?
  • symptoms?
A
  • type II
  • antibodies to ACh receptor
  • fluctuating weakness, extraocular weakness or ptosis
  • tensilon test positive
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
70
Q

Goodpasture’s disease:

  • type hypersensitivity?
  • pathogenesis?
  • symptoms?
  • investigations?
A
  • type II
  • smooth linear deposition of antibody along glomerular and alveolar BM (collagen type IV)
  • haemoptysis, reduced urine output, legs swelling, raised creatinine, microscopic haematuria and proteinuria
  • anti-neutrophil cytoplasmic antibody (-ve); anti-basement membrane antibody (+ve); cresenteric nephritis on biopsy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
71
Q

Goodpasture’s disease
A. Anti-GAD antibody

B. Anti-thyroglobulin antibody

C. Anti-basement membrane antibody

D. Anti-intrinsic factor antibody

E. Anti-acetylcholine receptor antibody

F. Anti-TSH receptor antibody

A

C. anti-basement membrane antibody

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
72
Q

myasthenia gravis
A. Anti-GAD antibody

B. Anti-thyroglobulin antibody

C. Anti-basement membrane antibody

D. Anti-intrinsic factor antibody

E. Anti-acetylcholine receptor antibody

F. Anti-TSH receptor antibody

A

E. Anti-acetylcholine receptor antibody

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
73
Q

Graves disease
A. Anti-GAD antibody

B. Anti-thyroglobulin antibody

C. Anti-basement membrane antibody

D. Anti-intrinsic factor antibody

E. Anti-acetylcholine receptor antibody

F. Anti-TSH receptor antibody

A

F. Anti-TSH receptor antibody

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
74
Q

Pernicious anaemia
A. Anti-GAD antibody

B. Anti-thyroglobulin antibody

C. Anti-basement membrane antibody

D. Anti-intrinsic factor antibody

E. Anti-acetylcholine receptor antibody

F. Anti-TSH receptor antibody

A

D. Anti-intrinsic factor antibody

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
75
Q

Diabetes mellitus
A. Anti-GAD antibody

B. Anti-thyroglobulin antibody

C. Anti-basement membrane antibody

D. Anti-intrinsic factor antibody

E. Anti-acetylcholine receptor antibody

F. Anti-TSH receptor antibody

A

A. Anti-GAD antibody

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
76
Q

Hashimoto’s thyroditis
A. Anti-GAD antibody

B. Anti-thyroglobulin antibody

C. Anti-basement membrane antibody

D. Anti-intrinsic factor antibody

E. Anti-acetylcholine receptor antibody

F. Anti-TSH receptor antibody

A

B. Anti-thyroglobulin antibody

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
77
Q

RhA:

PAD2 and PAD4 polymorphism - what does this result in?

A

high load of citrullinated proteins
(peptidylarginase deiminase=enzymes in deimination of arginine to create citrulline. polymorphisms are associated with increased citrullination)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
78
Q

risk factors for RhA?

why does these develop RhA?

A

Smoking and gum infection with Porphyromonas gingivalis

Both lead to increased citrullination

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
79
Q

what is the antibody test for RhA? What is it’s sensitivity and specificity?

A

anti-cyclic cirtullinated peptide (anti-CCP) antibody

  • specificity=95%
  • sensitivity=60-70%
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
80
Q

What is a rheumatoid factor?

specificity and sensitivity for diagnosis of RhA?

A

an antibody directed against the common (Fc) region of human IgG
-60-70% specificity and sensitivity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
81
Q

Joint changes in RhA?

A
  • increased synovial fluid volume
  • inflamed synovial tissue forms a pannus
  • overlaying and invading articular cartilage and adjacent bone tissues
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
82
Q

What hypersensitivity is involved in RhA pathology? Explain this.

A

Type II repsonse:
-antibody binding to citrullinated proteins may lead to: 1) activation of complement; 2) activation of macrophages; 3) NK cell activation (ie antibody dependent)
Type III repsonse:
-immune complex formation (RF and anti-CCP) and depsoition with complement activation
Type IV response:
-T cell activation, activates macrophages and fibroblasts, leading to TNF alpha, MMPs and IL1 prouction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
83
Q

RhA - shared epitope:
A. Expressed by synovial macrophages and pivotal in cytokine cascade that leads to inflammation and damage in rheumatoid arthritis

B. Region of HLA DR beta chain that predisposes to development of rheumatoid arthritis

C. Expresses PADI enzymes capable of deiminating arginine to form citrullinated proteins

D. Binds to citrullinated proteins and has ~95% specificity for development of rheumatoid arthritis

E. Binds to Fc region of IgG

A

B. Region of HLA DR beta chain that predisposes to development of rheumatoid arthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
84
Q

RhA - P gingivalis:
A. Expressed by synovial macrophages and pivotal in cytokine cascade that leads to inflammation and damage in rheumatoid arthritis

B. Region of HLA DR beta chain that predisposes to development of rheumatoid arthritis

C. Expresses PADI enzymes capable of deiminating arginine to form citrullinated proteins

D. Binds to citrullinated proteins and has ~95% specificity for development of rheumatoid arthritis

E. Binds to Fc region of IgG

A

C. Expresses PADI enzymes capable of deiminating arginine to form citrullinated proteins

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
85
Q

RhA - anti-CCP antibody or ACPA:
A. Expressed by synovial macrophages and pivotal in cytokine cascade that leads to inflammation and damage in rheumatoid arthritis

B. Region of HLA DR beta chain that predisposes to development of rheumatoid arthritis

C. Expresses PADI enzymes capable of deiminating arginine to form citrullinated proteins

D. Binds to citrullinated proteins and has ~95% specificity for development of rheumatoid arthritis

E. Binds to Fc region of IgG

A

D. Binds to citrullinated proteins and has ~95% specificity for development of rheumatoid arthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
86
Q

RhA - TNF alpha:
A. Expressed by synovial macrophages and pivotal in cytokine cascade that leads to inflammation and damage in rheumatoid arthritis

B. Region of HLA DR beta chain that predisposes to development of rheumatoid arthritis

C. Expresses PADI enzymes capable of deiminating arginine to form citrullinated proteins

D. Binds to citrullinated proteins and has ~95% specificity for development of rheumatoid arthritis

E. Binds to Fc region of IgG

A

A. Expressed by synovial macrophages and pivotal in cytokine cascade that leads to inflammation and damage in rheumatoid arthritis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
87
Q

RhA - rheumatoid factor:
A. Expressed by synovial macrophages and pivotal in cytokine cascade that leads to inflammation and damage in rheumatoid arthritis

B. Region of HLA DR beta chain that predisposes to development of rheumatoid arthritis

C. Expresses PADI enzymes capable of deiminating arginine to form citrullinated proteins

D. Binds to citrullinated proteins and has ~95% specificity for development of rheumatoid arthritis

E. Binds to Fc region of IgG

A

E. Binds to Fc region of IgG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
88
Q

Clinical features of SLE?

Clue: CNS, skin, heart, kidneys, mediastinum, haematological, joint

A 
Seizures
butterfly rash, discoid lupus
endocarditis, myocarditis
glomerulonephritis
serositis, pleuritis, pericarditis
haemolytic anaemia, leukopenia, thrombcytopenia
arthritis
lymphadenopathy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
89
Q

SLE - incidence and prevalence?

A

1:2000
female preponderance
incidence highest in 2nd and 3rd decades
genetic predisposition

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
90
Q

SLE - hypersensitivity type? describe pathogenesis

A

Type III

1) antibodies bind to antigen to form immune complexes
2) immune complexes deposit in tissues (skin, joints, kidney)
3) activation of complement
4) stimulation of cells expressing Fc and complement receptors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
91
Q

SLE - what mechanisms are there for development of autoantibodies?

A

1) decreased apoptotic material clearance - polymorphsisms in complement/MBL/CRP genes
2) abnormal cellular activation - polymorphisms in cytokines
3) loss of tolerance & b cell hyperactivity
4) antibodies against intracellular proteins eg nuclear antigens (DNA, histones, snRNP) and cytoplasmic antigens (ribosome, scRNP)

92
Q

CRP

a) where is it produced?
b) what stimulates its production?
c) what is the half life?
d) when does it rise?

A

a) acute phase protein from liver
b) IL6
c) 6 hours (short)
d) rapid rise in response to infection/inflammation

93
Q

ESR

a) what does it measure?
b) when does it rise?

A

a) the rate of fall of erythrocytes through plasma
b) increase in fibrinogen within plasma allows red cells to fall more quickly. patients normally have high CRP
- or high total Ig leads to clumping of red cells (rouleaus) which fall more quickly. normal CRP. myeloma, SLE, Sjogrens

94
Q

Antiphospholipid syndrome

1) symptoms?
2) investigations?

A

1) recurrent venous or arterial thrombosis; recurrent miscarriage
2) anticardiolipin antibody - immunoglobulins directed against phopsholipids and beta2 glycoprotein-1; lupus anti-coagulant - prolongation of phopsholipid-dependent coagulation

95
Q

SLE - screen for anti-dsDNA. specificity? why are they useful?

A

95% specific to SLE

useful in disease monitoring (increase associated with disease activity)

96
Q

complement profiles in SLE. C3 and C4 in:

  • active disease?
  • severe active disease?
  • inactive disease?
A
  • normal and low
  • low and low
  • normal and normal

(as immune complexes deplete complement stores)

97
Q

In SLE moderately active disease - CRP:

A. Usually HIGH

B. Generally NORMAL

C. Usually LOW

A

b. Generally NORMAL

98
Q

In SLE moderately active disease - ESR:

A. Usually HIGH

B. Generally NORMAL

C. Usually LOW

A

A. Usually HIGH

99
Q

In SLE moderately active disease - anti-dsDNA:

A. Usually HIGH

B. Generally NORMAL

C. Usually LOW

A

A. Usually HIGH

100
Q

In SLE moderately active disease - C4:

A. Usually HIGH

B. Generally NORMAL

C. Usually LOW

A

C. Usually LOW

101
Q

Systemic sclerosis

A

Inflammation with Th17 and Th2

  • cytokines lead to activation of fibroblasts and development of fibrosis (polymorphisms in T1 collagen a2 chains and fibrillin1)
  • cytokines lead to activation of endothelial cells and contribute to microvascular disease
  • loss of B cell tolerance to nuclear antigens
102
Q

What is CREST?

What antibody tests do you do?

A 
Limited cutaneous systemic sclerosis
-skin involvement does not progress beyond forearms (may involve peri-oral skin)
CREST
calcinosis
Raynaud's
oEsophageal dysmotility
sclerodactyly
telangiectasia
\+primary pul HTN
-2) anticentromere antibodies
103
Q

diffuse cutaneous systemic sclerosis - what is it/symptoms?

what antibody tests?

A

skin involvement does not progress beyond forearms
-CREST features
more extensive GI disease
interstitial pul disease
scleroderma kidney/renal crisis
-2) anti-topoisomerase antibodies (scl70), RNApol, fibrillarin

104
Q

what hypersensitivity type is dermatomyositis?

what immune cells are involved?

A
  • Type III - immune complex mediated vasculitis

- perivascular CD4 T cells and B cells within muscle

105
Q

what hypersensitivity type is polymyositis?

what immune cells are involved?

A
  • Type IV - CD8 T cells kill myofibres

- within muscle CD8 T cells surround HLA1 expressing myofibres

106
Q

symptoms of dermatomyositis and polymyositis?

A

Muscle - progressive and symmetrical weakness of proximal muscles. may involve pharyngeal or resp muscles
Skin - periorbital oedema with heliotrope discolouration of eye lids, scaley red rash on extensor surface of fingers, elbows, knees, Gottron’s papules
Lungs- may be associated with interstitial lung disease

fevers/arthritis/raynauds possible
strong association with presence of underlying malignancy

107
Q

What antibody investigations for:

1) dermatomyositis?
2) polymyositis?

A

Positive ANA

1) -anti-aminoacyl transfer RNA synthetase antibody eg Jo-1
2) anti-signal recognition peptide antibody (nuclear and cytoplasmic)
- anti-M2 (nuclear) DM>PM

108
Q

What statement most correctly describes ANA?
A. Screening test for a connective tissue disease

B. May be positive in SLE or in Sjogren’s syndrome but not usually in systemic sclerosis

C. Sometimes positive in patients with immune mediated myositis particularly if they have interstitial lung disease

A

A. Screening test for a connective tissue disease

109
Q

What statement most correctly describes anti-Ro?
A. Screening test for a connective tissue disease

B. May be positive in SLE or in Sjogren’s syndrome but not usually in systemic sclerosis

C. Sometimes positive in patients with immune mediated myositis particularly if they have interstitial lung disease

A

What statement most correctly describes ANA?

B. May be positive in SLE or in Sjogren’s syndrome but not usually in systemic sclerosis

110
Q

What statement most correctly describes anti-Jo-1 (t-RNA synthetase)?
A. Screening test for a connective tissue disease

B. May be positive in SLE or in Sjogren’s syndrome but not usually in systemic sclerosis

C. Sometimes positive in patients with immune mediated myositis particularly if they have interstitial lung disease

A

C. Sometimes positive in patients with immune mediated myositis particularly if they have interstitial lung disease

111
Q

What statement most correctly describes anti-Scl70?
A. Associated specifically with limited cutaneous form of systemic sclerosis

B. Associated specifically with diffuse cutaneous form of systemic sclerosis

C. Highly specific for diagnosis of SLE. Present in ~70% patients

A

B. Associated specifically with diffuse cutaneous form of systemic sclerosis

112
Q

What statement most correctly describes anti-dsDNA?
A. Associated specifically with limited cutaneous form of systemic sclerosis

B. Associated specifically with diffuse cutaneous form of systemic sclerosis

C. Highly specific for diagnosis of SLE. Present in ~70% patients

A

C. Highly specific for diagnosis of SLE. Present in ~70% patients

113
Q

What statement most correctly describes anti-centromere?
A. Associated specifically with limited cutaneous form of systemic sclerosis

B. Associated specifically with diffuse cutaneous form of systemic sclerosis

C. Highly specific for diagnosis of SLE. Present in ~70% patients

A

A. Associated specifically with limited cutaneous form of systemic sclerosis

114
Q

What are the 3 types of small vessel vasculitis associated with ANCA?

A

Microscopic polyangiitis

Granulomatosis with polyangiitis (Wegener’s granulomatosis)

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)

115
Q

1) What is ANCA?
2) What type of hypersensitivity is it?
3) What results from activation?

A

1) anti-neutrophil cytoplasmic antibodies
2) type II - antibody engagement with cell surface antigens may lead to neutrophil activation
3) activated neutrophils interact with endothelial cells causing damage to vessels –> vasculitis

116
Q

What does cANCA test for?

What antibodies is it associated with?

A
  • cytoplasmic fluorescence. Occurs in >90% of patients with granulomatous polyangiitis with renal involvement
  • associated with antibodies to enzyme proteinase 3
117
Q

p-ANCA what does it test for?

what antibody does it associate with?

A

perinuclear staining pattern
associated with microscopic polyangiitis and eosinophilic granulomatous polyangiitis (Churg-Strauss)
-associated with antibodies to myeloperoxidase
(less sensitive and specific than cANCA)

118
Q

ANA and vasculitis

A. Screening test for a connective tissue disease
or,
B. Associated with a subset of small vessel vasculitides including MPA, GPA and eGPA

A

A. Screening test for a connective tissue disease

119
Q

ANCA

A. Screening test for a connective tissue disease
or,
B. Associated with a subset of small vessel vasculitides including MPA, GPA and eGPA

A

B. Associated with a subset of small vessel vasculitides including MPA, GPA and eGPA

120
Q

What are the mononuclear immune cells of these organs called?

1) liver
2) kidney
3) bone
4) spleen
5) lung
6) neural tissue
7) connective tissue
8) skin
9) joints

A

1) Hupffer cells
2) mesangial cells
3) osteoclasts
4) sinusodial liining cell
5) alveolar macrophage
6) microglia
7) histiocyte
8) Langerhans cell
9) macrophage like synoviocytes

121
Q

oxidative killing:
A. Is mediated by Toll like receptors which recognise pathogen associated molecular patterns

B. May be mediated by antibodies, complement components or acute phase proteins and facilitates phagocytosis

C. Describes killing mediated by reactive oxygen species generated by action of the NADPH oxidase complex

D. May be mediated by bacteriocidal enzymes such as lysozyme

A

C. Describes killing mediated by reactive oxygen species generated by action of the NADPH oxidase complex

122
Q

pathogen recognition:
A. Is mediated by Toll like receptors which recognise pathogen associated molecular patterns

B. May be mediated by antibodies, complement components or acute phase proteins and facilitates phagocytosis

C. Describes killing mediated by reactive oxygen species generated by action of the NADPH oxidase complex

D. May be mediated by bacteriocidal enzymes such as lysozyme

A

A. Is mediated by Toll like receptors which recognise pathogen associated molecular patterns

123
Q

opsonisation
A. Is mediated by Toll like receptors which recognise pathogen associated molecular patterns

B. May be mediated by antibodies, complement components or acute phase proteins and facilitates phagocytosis

C. Describes killing mediated by reactive oxygen species generated by action of the NADPH oxidase complex

D. May be mediated by bacteriocidal enzymes such as lysozyme

A

B. May be mediated by antibodies, complement components or acute phase proteins and facilitates phagocytosis

124
Q

Non-oxidative killing:
A. Is mediated by Toll like receptors which recognise pathogen associated molecular patterns

B. May be mediated by antibodies, complement components or acute phase proteins and facilitates phagocytosis

C. Describes killing mediated by reactive oxygen species generated by action of the NADPH oxidase complex

D. May be mediated by bacteriocidal enzymes such as lysozyme

A

D. May be mediated by bacteriocidal enzymes such as lysozyme

125
Q

Defects of phagocyte mobilisation:

-failure of stem cells to differentiate along myeloid or lymphoid lineage

A

Reticular dysgensis - autosomal domiant SCID

–> mutation in mitochondrial energy metabolism enzyme adenylate kinase 2 (AK2)

126
Q

defects of phagocyte mobilisation:

-specific failure of neutrophil maturation (2)

A

1) Kostmann syndrome = autosomal recessive severe congenital neutropenia (mutation in HCLS1-associated protein x-1 (HAX1))
2) Cyclic neutropenia = automosal dominant episodic neutropenia every 4-6 weeks (mutation in neutrophil elastase (ELA-2))

127
Q

What does a deficiency of CD18 (beta2 integrin subunit) result in?

A

leukocyte adhesion deficiency - neutrophils lack adhesion molecules and fail to exit the bloodstream (very high neurtophil counts in blood and absence of pus formation)

128
Q

Chronic granulomatous disease. How does it occur? What symptoms?

A

Failure of oxidative killing mechanisms:

  • 1) inability to generate oxygen free radicals due to deficiency of one of components of NADPH oxidase
    • -> absent respiratory burst
      2) excessive inflammation –> persistent neutrophil/macrophage accummulation + failure to degrade antigens
      3) granuloma formation
      3) lymphadenopathy and hepatosplenomegaly
129
Q

Investigation of chronic granulomatous disease

A 
Nitroblue tetrazolinum (NBT) test: changes colour in presence of hydrogen peroxide
dihydrorhodamine (DHR) flow cytometry test: fluoresces in presence of hydrogen peroxide
130
Q

Kostmann syndrome. What would happen to the:

  • 1) neutrophil count
  • 2) leukocyte adhesion markers
  • 3) NBT or DHR test
  • 4) pus
A
  • 1) absent
  • 2) normal
  • 3) absent (as no neutrophils)
  • 4) no
131
Q

Leukocyte adhesion deficiency. What would happen to the:

  • 1) neutrophil count
  • 2) leukocyte adhesion markers
  • 3) NBT or DHR test
  • 4) pus
A
  • 1) increased during infection
  • 2) absent
  • 3) normal
  • 4) no
132
Q

chronic granulomatous disease. What would happen to the:

  • 1) neutrophil count
  • 2) leukocyte adhesion markers
  • 3) NBT or DHR test
  • 4) pus
A
  • 1) normal
  • 2) normal
  • 3) abnormal
  • 4) yes
133
Q

phagocyte deficiency. recurrent infections with high neutrophil count on FBC but no abcess formation.
A. IFN gamma receptor deficiency

B. Leukocyte adhesion deficiency

C. Chronic granulomatous disease

D. Kostmann syndrome

A

B. Leukocyte adhesion deficiency

134
Q

Phagocyte deficiency. recurrent infections with hepatosplenomegaly and abnoral dihydrorhodamine test.
A. IFN gamma receptor deficiency

B. Leukocyte adhesion deficiency

C. Chronic granulomatous disease

D. Kostmann syndrome

A

C. Chronic granulomatous disease

135
Q

phagocyte deficiency. recurrent infections with no neutrophils on FBC.
A. IFN gamma receptor deficiency

B. Leukocyte adhesion deficiency

C. Chronic granulomatous disease

D. Kostmann syndrome

A

D. Kostmann syndrome

136
Q

phagocyte deficiency. infection with atypical mycobacterium. normal FBC.
A. IFN gamma receptor deficiency

B. Leukocyte adhesion deficiency

C. Chronic granulomatous disease

D. Kostmann syndrome

A

A. IFN gamma receptor deficiency

137
Q

What is the importance of IL12 and IFNg in phagocyte development?

A

Infected macrophages produce IL12, inducing T cells to secrete IFNg. IFNg feeds back to macrophages and neutrophils, stimulating production of TNF–>NADPH oxidase activation

138
Q

Neutrophils:
A. Derived from monocytes and resident in peripheral tissues

B. Polymorphonuclear cells capable of phagocytosing pathogens and killing by oxidative and non-oxidative mechanisms

C. Lymphocytes that express inhibitory receptors capable of recognising HLA class I molecules and have cytotoxic capacity

D. Immature cells are adapted for pathogen recognition and uptake whilst mature cells are adapted for antigen presentation to prime T cells

A

B. Polymorphonuclear cells capable of phagocytosing pathogens and killing by oxidative and non-oxidative mechanisms

139
Q

Natural killer cells:
A. Derived from monocytes and resident in peripheral tissues

B. Polymorphonuclear cells capable of phagocytosing pathogens and killing by oxidative and non-oxidative mechanisms

C. Lymphocytes that express inhibitory receptors capable of recognising HLA class I molecules and have cytotoxic capacity

D. Immature cells are adapted for pathogen recognition and uptake whilst mature cells are adapted for antigen presentation to prime T cells

A

C. Lymphocytes that express inhibitory receptors capable of recognising HLA class I molecules and have cytotoxic capacity

140
Q

Dendritic cells:
A. Derived from monocytes and resident in peripheral tissues

B. Polymorphonuclear cells capable of phagocytosing pathogens and killing by oxidative and non-oxidative mechanisms

C. Lymphocytes that express inhibitory receptors capable of recognising HLA class I molecules and have cytotoxic capacity

D. Immature cells are adapted for pathogen recognition and uptake whilst mature cells are adapted for antigen presentation to prime T cells

A

D. Immature cells are adapted for pathogen recognition and uptake whilst mature cells are adapted for antigen presentation to prime T cells

141
Q

Macrophages:
A. Derived from monocytes and resident in peripheral tissues

B. Polymorphonuclear cells capable of phagocytosing pathogens and killing by oxidative and non-oxidative mechanisms

C. Lymphocytes that express inhibitory receptors capable of recognising HLA class I molecules and have cytotoxic capacity

D. Immature cells are adapted for pathogen recognition and uptake whilst mature cells are adapted for antigen presentation to prime T cells

A

A. Derived from monocytes and resident in peripheral tissues

142
Q

What are the 3 pathways of complement activation?

What complement do they activate? What is the outcome?

A

Classical, MBL (mannose-binding lectin), alternative.
C3
Leads to final common pathway C5-C9 –> membrane attack complex

143
Q

What is the classical pathway of complement activation?

A

Formation of antibody-antigen immune complexes results in change in antibody shape –> exposes binding site to C1
(also involves C4 and C2)
- dependent upon activation of acquired immune repsonse

144
Q

What is the mannonse binding lectin pathway of complement activation?

A

activated by direct binding of MBL to microbial cell surface carbohydrates –> directly stimulate clasical pathway (C4 and C2, not C1)
-not dependent upon acquired immune response

145
Q

What is the alternative pathway of complement activation?

A

Directly triggered by binding of C3 to bacterial cell wall components eg lipopolysaccharide of G-ve or teichoic acid of G+ve
-not dependent on acquired immune response

146
Q

Complement. C3:
A. Binding of immune complexes to this protein triggers the classical pathway of complement activation

B. Cleavage of this protein may be triggered via the classical, MBL or alternative pathways

C. Binds to microbial surface carbohydrates to activate the complement cascade in an immune complex independent manner

D. Part of the final common pathway resulting in the generation of the membrane attack complex

A

B. Cleavage of this protein may be triggered via the classical, MBL or alternative pathways

147
Q

Complement. C1:
A. Binding of immune complexes to this protein triggers the classical pathway of complement activation

B. Cleavage of this protein may be triggered via the classical, MBL or alternative pathways

C. Binds to microbial surface carbohydrates to activate the complement cascade in an immune complex independent manner

D. Part of the final common pathway resulting in the generation of the membrane attack complex

A

A. Binding of immune complexes to this protein triggers the classical pathway of complement activation

148
Q

Complement. C9:
A. Binding of immune complexes to this protein triggers the classical pathway of complement activation

B. Cleavage of this protein may be triggered via the classical, MBL or alternative pathways

C. Binds to microbial surface carbohydrates to activate the complement cascade in an immune complex independent manner

D. Part of the final common pathway resulting in the generation of the membrane attack complex

A

D. Part of the final common pathway resulting in the generation of the membrane attack complex

149
Q

Complement. MBL:
A. Binding of immune complexes to this protein triggers the classical pathway of complement activation

B. Cleavage of this protein may be triggered via the classical, MBL or alternative pathways

C. Binds to microbial surface carbohydrates to activate the complement cascade in an immune complex independent manner

D. Part of the final common pathway resulting in the generation of the membrane attack complex

A

C. Binds to microbial surface carbohydrates to activate the complement cascade in an immune complex independent manner

150
Q

What disease is associated with deficiency of the classical complement pathway?
How come?

A

SLE
Classical pathway involved in clearance of apoptotic/necrotic cells –> deficiency = increased load of self antigen esp. nuclear components. Complement activation normally promotes clearance of immune complexes by erythrocytes thus deficiencies result in deposition of immune complexes which stimulates local inflammation in skin, kidneys and joints

151
Q

If there is a defect in the terminal (“common”) pathway, which kind of bacteria are most likely to take advantage? Give 3 example organisms.

A

Inability to use complement to lyse encapsulated bacteria
Neisseria meningitis
Streptococcus pneumonia
Haemophilius influenza

152
Q

Complement deficiency. Membranoproliferative nephritis and bacterial infections:
A. C9 deficiency

B. C3 deficiency with presence of a nephritic factor

C. MBL deficiency

D. C1q deficiency

A

B. C3 deficiency with presence of a nephritic factor

153
Q

Complement deficiency. Meningococcus meningitis with family history of sibling dying of same condition aged 6:
A. C9 deficiency

B. C3 deficiency with presence of a nephritic factor

C. MBL deficiency

D. C1q deficiency

A

A. C9 deficiency

154
Q

Complement deficiency. Severe childhood onset SLE with normal levels of C3 and C4:
A. C9 deficiency

B. C3 deficiency with presence of a nephritic factor

C. MBL deficiency

D. C1q deficiency

A

D. C1q deficiency

155
Q

Complement deficiency. Recurrent infections when receiving chemotherapy but previously well:
A. C9 deficiency

B. C3 deficiency with presence of a nephritic factor

C. MBL deficiency

D. C1q deficiency

A

C. MBL deficiency

156
Q

CD8+ T cells recognise peptide presented by?

A

HLA class I molecules (HLA-A, HLA-B, HLA-C)

157
Q

CD4+ T cells recognise peptide presented by?

A

HLA class II molecules (HLA-DR, HLA-DP, HLA-DQ)

158
Q 
Adaptive immune system. Th1 cells:
A. Express receptors that recognise peptides usually derived from intracellular proteins and expressed on HLA class I molecules

B. Subset of lymphocytes that express Foxp3 and CD25

C. Subset of cells that express CD4 and secrete IFN gamma and IL-2

D. Play an important role in promoting germinal centre reactions and differentiation of B cells into IgG and IgA secreting plasma cells

A

C. Subset of cells that express CD4 and secrete IFN gamma and IL-2

159
Q 
Adaptive immune system. CD8 T cells:
A. Express receptors that recognise peptides usually derived from intracellular proteins and expressed on HLA class I molecules

B. Subset of lymphocytes that express Foxp3 and CD25

C. Subset of cells that express CD4 and secrete IFN gamma and IL-2

D. Play an important role in promoting germinal centre reactions and differentiation of B cells into IgG and IgA secreting plasma cells

A

A. Express receptors that recognise peptides usually derived from intracellular proteins and expressed on HLA class I molecules

160
Q 
Adaptive immune system. T follicular helper (Tfh) cells:
A. Express receptors that recognise peptides usually derived from intracellular proteins and expressed on HLA class I molecules

B. Subset of lymphocytes that express Foxp3 and CD25

C. Subset of cells that express CD4 and secrete IFN gamma and IL-2

D. Play an important role in promoting germinal centre reactions and differentiation of B cells into IgG and IgA secreting plasma cells

A

D. Play an important role in promoting germinal centre reactions and differentiation of B cells into IgG and IgA secreting plasma cells

161
Q 
Adaptive immune system. T regulatory cells:
A. Express receptors that recognise peptides usually derived from intracellular proteins and expressed on HLA class I molecules

B. Subset of lymphocytes that express Foxp3 and CD25

C. Subset of cells that express CD4 and secrete IFN gamma and IL-2

D. Play an important role in promoting germinal centre reactions and differentiation of B cells into IgG and IgA secreting plasma cells

A

B. Subset of lymphocytes that express Foxp3 and CD25

162
Q

pre-B cells:
A. Exist within the bone marrow and develop from haematopoietic stem cells

B. Cell dependent on the presence of CD4 T cell help for generation.

C. Are generated rapidly following antigen recognition and are not dependent on CD4 T cell help

D. Divalent antibody present within mucous which helps provide a constitutive barrier to infection

A

A. Exist within the bone marrow and develop from haematopoietic stem cells

163
Q

IgA:
A. Exist within the bone marrow and develop from haematopoietic stem cells

B. Cell dependent on the presence of CD4 T cell help for generation.

C. Are generated rapidly following antigen recognition and are not dependent on CD4 T cell help

D. Divalent antibody present within mucous which helps provide a constitutive barrier to infection

A

D. Divalent antibody present within mucous which helps provide a constitutive barrier to infection

164
Q

IgG secreting plasma cells:
A. Exist within the bone marrow and develop from haematopoietic stem cells

B. Cell dependent on the presence of CD4 T cell help for generation.

C. Are generated rapidly following antigen recognition and are not dependent on CD4 T cell help

D. Divalent antibody present within mucous which helps provide a constitutive barrier to infection

A

B. Cell dependent on the presence of CD4 T cell help for generation.

165
Q

IgM secreting plasma cells:
A. Exist within the bone marrow and develop from haematopoietic stem cells

B. Cell dependent on the presence of CD4 T cell help for generation.

C. Are generated rapidly following antigen recognition and are not dependent on CD4 T cell help

D. Divalent antibody present within mucous which helps provide a constitutive barrier to infection

A

C. Are generated rapidly following antigen recognition and are not dependent on CD4 T cell help

166
Q

3 functions of antibodies?

A
  • 1) identification of pathogens and toxins
  • 2) interact with other components of immune response to remove pathogens (complement, phagocytes, NK cells)
  • 3) defence against bacteria of all kinds
167
Q

Which region of the antibody is the antigen binding regions?

A

Fab

168
Q

Which region of the antibody is the constant region of the heavy chain determining effector function?

A

Fc

169
Q

What is the mutation in reticular dysgenesis?

What does fails to produce?

A
  • mutation in mitochondrial energy metabolism enzyme adenylate kinase 2 (AK2)
  • failure in production of: neutrophils, lymphocytes, monocyte/macrophage, platelets
170
Q

Phenotype of X-linked SCID?

Where is the mutation?

A

Very low or absent T cell no.; normal or increased B cell no.; poorly developed lymphoid tissue and thymus
Mutation of gamma chain of IL-2 receptor on Xq13.1

171
Q

What is DiGeorge syndrome? What has been deleted? Symptoms?

A

1) development defect of pharyngeal pouch
2) 22q11.2 deletion
3) high forehead; low set, abnormally folded ears, cleft palate, small mouth and jaw. hypocalcaemia, oesophageal atresia, T cell lymphopenia, complex congenital heart disease.
Normal B cell, reduced T cell

172
Q

Severe recurrent infections from 3 months, CD4 and CD8 T cells absent, B cell present, IgM present, IgA and IgG absent.

A. Bare lymphocyte syndrome type II

B. X-linked SCID

C. DiGeorge syndrome

D. IFN gamma receptor deficiency

A

B. X-linked SCID

173
Q

Young adult with chronic infection with Mycobacterium mariunum.

A. Bare lymphocyte syndrome type II

B. X-linked SCID

C. DiGeorge syndrome

D. IFN gamma receptor deficiency

A

D. IFN gamma receptor deficiency

174
Q

Recurrent infections in childhood, abnormal facial features, congenital heart disease, normal B cells, low T cells, low IgA and IgG

A. Bare lymphocyte syndrome type II

B. X-linked SCID

C. DiGeorge syndrome

D. IFN gamma receptor deficiency

A

C. DiGeorge syndrome

175
Q

14 month baby with two recent serious bacterial infectinos. T cells present - but only CD8+ population. B cells present. IgM present but IgG absent

A. Bare lymphocyte syndrome type II

B. X-linked SCID

C. DiGeorge syndrome

D. IFN gamma receptor deficiency

A

A. Bare lymphocyte syndrome type II

176
Q

Adult with bronchiectasis, recurrent sinusitis and development of atypical SLE

A. IgA deficiency

B. Common variable immunodeficiency

C. Bruton’s X linked hypogammaglobulinaemia

D. X linked hyper IgM syndrome due to CD40ligand mutation

A

B. Common variable immunodeficiency

177
Q

Recurrent bacterial infections in a child, episode of pneumocystis pneumonia, high IgM, absent IgA and IgG

A. IgA deficiency

B. Common variable immunodeficiency

C. Bruton’s X linked hypogammaglobulinaemia

D. X linked hyper IgM syndrome due to CD40ligand mutation

A

D. X linked hyper IgM syndrome due to CD40ligand mutation

178
Q

1 year old boy. Recurrent bacterial infections. CD4 and CD8 T cells present. B cells abesnt, IgG, IgA, IgM absent

A. IgA deficiency

B. Common variable immunodeficiency

C. Bruton’s X linked hypogammaglobulinaemia

D. X linked hyper IgM syndrome due to CD40ligand mutation

A

C. Bruton’s X linked hypogammaglobulinaemia

179
Q

Recurrent respiratory tract infections, absent IgA, normal IgM and IgG

A. IgA deficiency

B. Common variable immunodeficiency

C. Bruton’s X linked hypogammaglobulinaemia

D. X linked hyper IgM syndrome due to CD40ligand mutation

A

A. IgA deficiency

180
Q

2 indications for bone marrow transplant?

A
  1. life threatening primary immunodeficiency eg SCID, leukocyte adhesion defect
  2. haematological malignancy
181
Q

Indications for antibody replacement?

A
  1. Primary antibody deficiency eg X linked agammaglobulinaemia, X-linked hyper IgM syndrome, common variable immune deficiency
  2. secondary antibody deficiency eg haematological malignancies (CLL, multiple myeloma), after BMT
182
Q

How does ipilimumab work?

A

Antibody specific for cytotoxic T-lymphocyte associated protein 4 (CTLA-4), blocking immune checkpoint, allowing T cell activation

Used in advanced melanoma

183
Q

How do pembrolizumab and nivolumab work?

A

Antibodies specfic for PD-1 (programmed cell death 1), blocks immune checkpoint, allowing T cell activation

Used in advanced melanoma

184
Q

Boosting the immune response. IFN alpha:

A. Post-transplant lymphoproliferative disorder

B. Part of treatment for Hepatitis C

C. X linked hyper IgM syndrome

D. X linked SCID

E. Chronic granulomatous disease

F. Immunosuppressed seronegative individual after chicken pox exposure

G. Metastatic melanoma

A

C. X linked hyper IgM syndrome

185
Q

Boosting the immune response. Bone marrow transplant:

A. Post-transplant lymphoproliferative disorder

B. Part of treatment for Hepatitis C

C. X linked hyper IgM syndrome

D. X linked SCID

E. Chronic granulomatous disease

F. Immunosuppressed seronegative individual after chicken pox exposure

G. Metastatic melanoma

A

D. X linked SCID

186
Q

Boosting the immune response. IFN gamma:

A. Post-transplant lymphoproliferative disorder

B. Part of treatment for Hepatitis C

C. X linked hyper IgM syndrome

D. X linked SCID

E. Chronic granulomatous disease

F. Immunosuppressed seronegative individual after chicken pox exposure

G. Metastatic melanoma

A

E. Chronic granulomatous disease

187
Q

Boosting the immune response. EBV-specific CD8 T cells:

A. Post-transplant lymphoproliferative disorder

B. Part of treatment for Hepatitis C

C. X linked hyper IgM syndrome

D. X linked SCID

E. Chronic granulomatous disease

F. Immunosuppressed seronegative individual after chicken pox exposure

G. Metastatic melanoma

A

A. Post-transplant lymphoproliferative disorder

188
Q

Boosting the immune response. Human normal immunoglobulin:

A. Post-transplant lymphoproliferative disorder

B. Part of treatment for Hepatitis C

C. X linked hyper IgM syndrome

D. X linked SCID

E. Chronic granulomatous disease

F. Immunosuppressed seronegative individual after chicken pox exposure

G. Metastatic melanoma

A

C. X linked hyper IgM syndrome

189
Q

Boosting the immune response. Pembrolizumab:

A. Post-transplant lymphoproliferative disorder

B. Part of treatment for Hepatitis C

C. X linked hyper IgM syndrome

D. X linked SCID

E. Chronic granulomatous disease

F. Immunosuppressed seronegative individual after chicken pox exposure

G. Metastatic melanoma

A

G. Metastatic melanoma

190
Q

Boosting the immune response. Varicella zoster immunoglobulin:

A. Post-transplant lymphoproliferative disorder

B. Part of treatment for Hepatitis C

C. X linked hyper IgM syndrome

D. X linked SCID

E. Chronic granulomatous disease

F. Immunosuppressed seronegative individual after chicken pox exposure

G. Metastatic melanoma

A

F. Immunosuppressed seronegative individual after chicken pox exposure

191
Q

How do corticosteroids exert their anti-inflammatory effects?

A

Inhibits phospholipase A2 (repsonsible for breaking down phospholipids to arachidonic acid –> prostaglandins and leukotrienes)

192
Q

3 effect of corticosteroids on phagocytes?

A
  1. decreased traffic of phagocytes to inflamed tissue
  2. decreased phagocytosis
  3. decreased release of proteolytic enzymes
193
Q

4 effects of corticosteroids on lymphocyte function?

A
  1. lymphopenia (sequestration of lymphocytes in lymphoid tissue)
  2. blocks cytokine gene expression
  3. decreased antibody production
  4. promotes apoptosis
194
Q

side effects of corticosteroids?

A

METABOLIC eg diabetes, central obesity, moon face, lipid abnormalities, osteoporosis, hirsutism, adrenal suppresion
OTHER EFFECTS cataracts, glaucoma, peptic ulceration, pancreatitis, avascular necrosis
IMMUNOSUPPRESSION

195
Q

Cyclophosphamide

  • 1) mechanism of action?
  • 2) major indications?
  • 3) side effects?
A

1) alkylates guanine base of DNA –> damages DNA+prevent cell replication (affects B>T cells)
2) multisystem connective tissue disease or vasculitis with severe end-organ involvement; anti-cancer agent
3) toxic to proliferating cells; haemorrhagic cystitis; malignancy; infection (Pneumocystis Jiroveci)

196
Q

Azathioprine

  • 1) mechanism of action?
  • 2) major indications?
  • 3) side effects?
A

1) metabolised by liver to 6 mercaptopurine; blocks de novo purine synthesis –> prevents DNA replication (preferentially inhibits T cell activation and proliferation)
2) transplantation; AI disease; auto-inflammatory disease
3) bone marrow suppression (check TPMT activity or gene variants before treatment); hepatotoxicity; infection

197
Q

Mycophenolate mofetil

  • 1) mechanism of action?
  • 2) major indications?
  • 3) side effects?
A

1) blocks de novo nucleotide synthesis–> prevent DNA replication (prevent T>B cell proliferation)
2) transplantation alternative to azathioprine; used in auto-immune disease and vasculitis as alternative to cyclophosphamide
3) bone marrow suppression; infection (herpes virus, JC virus=PML)

198
Q

Indications for plasmapheresis?

A

Severe antibody-mediated disease eg Goodpasture’s syndrome (anti-glomerular BM antibodies); severe acute myasthenia gravis (anti-AChR antibodies); severe vascular rejection (anti-HLA antibodies to donor)
MANY ARE TYPE II HYPERSENSITIVITY REACTIONS

199
Q

Cyclosporin and tacrolimus:

-1) method of action?

A
  • 1) calcineurin inhibitors –> decreased IL2 production –> less lymphocyte proliferation and effector function
  • 2) nephrotoxicity, HTN, neurotoxic, dysmorphism, T2DM
200
Q

Tofacitinib:

-method of action?

A

JAK inhibitor (linked to cytokine receptor) –> inhibit cytokine production response

201
Q

Apremilast

-method of action?

A

PDE4 inhibitor –> PKA production –>prevent activation of TFs –> decrease cytokine production

202
Q

Immunosuppression s/e. Cyclophosphamide?

A. Osteoporosis

B. Infertility

C. Progressive multifocal leukoencephalopathy

D. Neutropenia particularly if TPMT low

E. Hypertension

A

B. Infertility

203
Q

Immunosuppression s/e.
Prednisolone?

A. Osteoporosis

B. Infertility

C. Progressive multifocal leukoencephalopathy

D. Neutropenia particularly if TPMT low

E. Hypertension

A

A. Osteoporosis

204
Q

Immunosuppression s/e.
Azathioprine?

A. Osteoporosis

B. Infertility

C. Progressive multifocal leukoencephalopathy

D. Neutropenia particularly if TPMT low

E. Hypertension

A

D. Neutropenia particularly if TPMT low

205
Q

Immunosuppression s/e.
Cyclosporin?

A. Osteoporosis

B. Infertility

C. Progressive multifocal leukoencephalopathy

D. Neutropenia particularly if TPMT low

E. Hypertension

A

E. Hypertension

206
Q

Immunosuppression s/e.
Mycophenolate mofetil?

A. Osteoporosis

B. Infertility

C. Progressive multifocal leukoencephalopathy

D. Neutropenia particularly if TPMT low

E. Hypertension

A

C. Progressive multifocal leukoencephalopathy

207
Q

What is basiliximab?

  • 1) indication?
  • 2) action?
  • 3) toxicity?
A

Antibody directed at CD25 (IL-2 alpha chain)

1) prophylaxis of allograft rejection, before and after transplant surgery
2) inhibits T cell proliferation
3) infused reactions, infection, LT risk of malignancy

208
Q

What is abatacept?

  • 1) indication?
  • 2) action?
  • 3) toxicity?
A

CTLA4-Ig fusion protein to block CD28

1) RhA. IV monthly, subcut weekly
2) reduce T cell activation
3) infusion rx, infection, caution wrt malignancy

209
Q

What is a cytokine storm?

Clinical features?

A

Potentially fatal immune reaction with a positive feedback loop (cytokines stimulate lymphocyte and macrophage migration and activation leading to production of more cytokines)
fevers, myalgias, increased vascular permeability (pul, cerebral oedema, cardiovascular collapse, poor peripheral perfusion, shock)

210
Q

Rituximab what is it?

  • 1) indication?
  • 2) action?
  • 3) toxicity?
A

CD20 antibody

1) lymphoma, RhA, SLE (2 IV doses every 6-12 months)
2) depletes mature B cells
3) infusion rx, infection exacerbation CV disease

211
Q

Natalizumab what is it?

  • 1) indications?
  • 2) dosing?
  • 3) toxicity?
A

antibody specific for alpha integrin

1) highly active RRMS (IV every 4 weeks)
2) inhibits T cell migration
3) infusion rx, infection (PML), hepatotoxic, malignancy

212
Q

What is tocilizumab?

  • 1) indications?
  • 2) action?
  • 3) toxicity?
A

Antibody directed at IL-6R

1) Castleman’s disease, RhA (IV once a month)
2) reduces macrophage, T cell, B cell, neutrophil activation
3) infusion rx, infection, hepatotoxicity, elevated lipids, malignancy

213
Q

Agents directed at cell surface molecules.
Basiliximab (anti-IL2 R)

A. Inhibits T cell migration but may only be used in highly active remitting/relapsing MS

B. Inhibits T cell activation and is effective in rheumatoid arthritis

C. Depletes B cells and is effective in treatment of B cell lymphomas and rheumatoid arthritis

D. Inhibits function of lymphoid and myeloid cells and used in management of rheumatoid arthritis

E. Antibody specific for CD25 which inhibits T cell activation and is used to prevent rejection

A

E. Antibody specific for CD25 which inhibits T cell activation and is used to prevent rejection

214
Q

Agents directed at cell surface molecules.
Abatacept (CTLA4-Ig fusion protein)

A. Inhibits T cell migration but may only be used in highly active remitting/relapsing MS

B. Inhibits T cell activation and is effective in rheumatoid arthritis

C. Depletes B cells and is effective in treatment of B cell lymphomas and rheumatoid arthritis

D. Inhibits function of lymphoid and myeloid cells and used in management of rheumatoid arthritis

E. Antibody specific for CD25 which inhibits T cell activation and is used to prevent rejection

A

B. Inhibits T cell activation and is effective in rheumatoid arthritis

215
Q

Agents directed at cell surface molecules.
Rituximab (anti-CD20)

A. Inhibits T cell migration but may only be used in highly active remitting/relapsing MS

B. Inhibits T cell activation and is effective in rheumatoid arthritis

C. Depletes B cells and is effective in treatment of B cell lymphomas and rheumatoid arthritis

D. Inhibits function of lymphoid and myeloid cells and used in management of rheumatoid arthritis

E. Antibody specific for CD25 which inhibits T cell activation and is used to prevent rejection

A

C. Depletes B cells and is effective in treatment of B cell lymphomas and rheumatoid arthritis

216
Q

Agents directed at cell surface molecules.
Natalizumab (anti-alpha4 integrin)

A. Inhibits T cell migration but may only be used in highly active remitting/relapsing MS

B. Inhibits T cell activation and is effective in rheumatoid arthritis

C. Depletes B cells and is effective in treatment of B cell lymphomas and rheumatoid arthritis

D. Inhibits function of lymphoid and myeloid cells and used in management of rheumatoid arthritis

E. Antibody specific for CD25 which inhibits T cell activation and is used to prevent rejection

A

A. Inhibits T cell migration but may only be used in highly active remitting/relapsing MS

217
Q

Agents directed at cell surface molecules.
Tocilizumab (anti-IL6 R)

A. Inhibits T cell migration but may only be used in highly active remitting/relapsing MS

B. Inhibits T cell activation and is effective in rheumatoid arthritis

C. Depletes B cells and is effective in treatment of B cell lymphomas and rheumatoid arthritis

D. Inhibits function of lymphoid and myeloid cells and used in management of rheumatoid arthritis

E. Antibody specific for CD25 which inhibits T cell activation and is used to prevent rejection

A

D. Inhibits function of lymphoid and myeloid cells and used in management of rheumatoid arthritis

218
Q

Which vaccines cannot be used in immunosuppressed patients?

A

Polio, BCG, yellow fever, measles (ie live vaccines)

219
Q

Inflixiamb, adalimumab, certolizumab, golimumab

  • 1) indication?
  • 2) mechanism of action?
  • 3) toxicity?
A

1) RhA, ankylosing spodylitis, psoriasis and psoriatic arthritis, IBD (subcut or IV)
2) inhibit TNF alpha
3) infusion or injection site reaction; infection; lupus-like conditions; demyelination; malignancy

220
Q

Etanercept

  • 1) indication?
  • 2) action?
  • 3) toxicity?
A

1) RhA, ank. spond., psoriasis and psoriatic arthritis (subcut weekly)
2) inhibits TNF alpha and TNF beta
3) injection site reaction, infection, lupus-like conditions, demyelination, malinancy

221
Q

Ustekinumab

  • 1) indication?
  • 2) mechanism?
  • 3) toxicity?
A

Antibody to p40 subunit of IL-12 and IL23.

1) psoriasis, psoriatic arthritis (subcut every 12 weeks)
2) inhibits IL-12 and IL-23
3) injection site reaction, infection, malignancy

222
Q

Denosumab

  • 1) indication?
  • 2) mechanism?
  • 3) toxicity?
A

Antibody directed at RANK ligand

1) osteoporosis (subcut every 6 months)
2) inhibits RANK mediated osteoclast differentiation and function
3) injection site reaction; infection; avascular necrosis of jaw

223
Q

Immunosuppression. Psoriasis?

A. Inhibit RANK ligand

B. Inhibit IL12/23 or TNF alpha

C. Inhibit IL6 or TNF alpha or deplete B cells

A

B. Inhibit IL12/23 or TNF alpha

224
Q

Immunosuppression. RhA?

A. Inhibit RANK ligand

B. Inhibit IL12/23 or TNF alpha

C. Inhibit IL6 or TNF alpha or deplete B cells

A

C. Inhibit IL6 or TNF alpha or deplete B cells

225
Q

Immunosuppression. Osteoporosis?

A. Inhibit RANK ligand

B. Inhibit IL12/23 or TNF alpha

C. Inhibit IL6 or TNF alpha or deplete B cells

A

A. Inhibit RANK ligand

Year 5 (9 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions