Lecture 25: Immunodeficiency, Autoimmunity and autoinflammation

Question 1

What are the two main mechanisms of the innate immune system?

Answer 1

1) complement cascade

2) neutrophil and dendritic cell action

Question 1

True or false: the innate immune system produces memory cells

Answer 1

True

Question 1

What is the main mechanism of the adaptive immune system?

Answer 1

antibody production

Question 2

True or false: the adaptive immune system produces memory cells

Answer 2

False

Question 3

What is the name given to the proteins that opsonise pathogens?

Answer 3

acute phase proteins

Question 4

What are autoinflammatory diseases>

Answer 4

clinical disorders caused by defects or dysregulation of the innate immune system characterised by recurrent or continuous inflammation

Question 5

Give a common presentation of autoinflammatory disease:

Answer 5

neutrophilic urticarial dermatosis

Question 6

What is the name of the inflammasome associated with a range of autoinflammatory diseases?

Answer 6

NLRP3 inflammasome

Question 7

Give 3 autoinflammatory diseases associated with the NLRP3 inflammasome in order of increasing severity:

Answer 7

1) FCAS

2) Muckle Wells Syndrome

3) NOMID

Question 8

What is arthralgia?

Answer 8

joint pain

Question 9

Give three symptoms of FCAS:

Answer 9

1) cold induced rash

2) conjunctivitis

3) arthralgia

Question 10

Give three symptoms of Muckle Wells Syndrome:

Answer 10

1) hives

2) sensorineural deafness

3) amyloidosis

Question 11

Give two symptoms of NOMID:

Answer 11

1) progressed and chronic meningitis (including deafness and visual + intellectual damage)

2) destructive arthritis

Question 12

How does a mutation of the NLRP3 gene cause autoinflammatory disease?

Answer 12

it over-activates the NLRP3 inflammasome, promoting proteolytic cleavage, maturation and secretion of pro-inflammatory cytokines interleukin 1-beta and 18

Question 13

What 2 interleukins does the NLRP3 inflammasome secrete?

Answer 13

1) IL-1-beta

2) IL-18

Question 14

What causes autoimmune diseases?

Answer 14

antibodies or lymphocytes produced against substances naturally present in the body

Question 15

Give 5 causes of autoimmune disease:

Answer 15

1) genetics

2) immune regulation

3) environment

4) sex (hormonal influence)

5) age

Question 16

Are autoimmune diseases more common in men or women?

Answer 16

women

Question 17

Where in the body do lymphocytes undergo positive and negative selection to ensure tolerance to body cells is developed?

Answer 17

Thymus

Question 18

What cells ensure our immune system isn’t overactive or attacking body cells?

Answer 18

regulatory T cells

Question 19

How do autoreactive B cells cause damage?

Answer 19

they are directly cytotoxic and cause activation of the complement system

Question 20

How do autoreactive T cells cause damage?

Answer 20

they are directly cytotoxic and cause inflammatory cytokine production

Question 21

What is Hashimoto’s thyroiditis?

Answer 21

destruction of thyroid follicles by autoimmunity

Question 22

Does Hashimoto’s thyroiditis cause hyper or hypothyroidism?

Answer 22

hypothyroidism

Question 23

Does Grave’s disease cause hyper or hypothyroidism?

Answer 23

hyperthyroidism

Question 24

In Hashimoto’s thyroiditis, what 2 proteins do the autoantibodies mainly act against?

Answer 24

1) thyroid peroxidase

2) thyroglobulin

Question 25

What is Grave’s disease?

Answer 25

inappropriate stimulation of the thyroid gland by anti-TSH autoantibodies

Question 26

Give 3 common manifestations of systemic lupus erythematosus:

Answer 26

1) skin rash

2) erosion of joints

3) kidney failure

Question 27

What structures do autoantibodies in SLE target?

Answer 27

connective tissue

Question 28

Other than SLE, give 3 autoimmune diseases that target connective tissue:

Answer 28

1) scleroderma

2) polymyositis

3) Sjogrens syndrome

Question 29

Define ‘immunosuppression’:

Answer 29

the natural or artificial process which turns off the immune response partially or fully, accidentally or on purpose

Question 30

Define ‘immunodeficiency’:

Answer 30

the lack of an efficient immune system causing susceptibility to infections

Question 31

Give 7 causes of secondary immunodeficiencies:

Answer 31

1) stress

2) surgery/ burns

3) malnutrition

4) cancer

5) immunosuppressive drugs e.g. cancer therapies

6) irradiation

7) AIDS

Question 32

Give the most common cause of secondary immunodeficiencies:

Answer 32

Malnutrition

Question 33

Give an example of a primary immunodeficiency disease:

Answer 33

SCID (severe combined immunodeficiency)

Question 34

True or false: both B and T cells are affected in SCID

Answer 34

True

Question 35

Why is it defects in T cells are more dramatic than B cells?

Answer 35

B cells rely on T cells for mediation and activation

Question 36

What is the main symptom of immunodeficiency diseases?

Answer 36

recurrent infection