L9 - Autoimmune disease

Question 1

What are the 2 types of autoimmune disease?

Answer 1

organ specific

non-organ specific

Question 2

What is IPEX syndrome?

Answer 2

Foxp3 gene - transcription factor

total absence or clear reduction of T-cells

problems in endocrine organs

Question 3

What are the 4 ways that regulatory T cells work?

Answer 3

inhibitory cytokines - TGFb and IL-10 calm down effector T-cells

cytolysis - release granzymes

metabolic disruption - high levels of cAMP calm effector T-cells, cytokine deprivation

targeting dendritic cells - inhibition of maturation & function

Question 4

What is an example of an organ-specific autoimmune disease?

Answer 4

Pemphigus vulgaris

Question 5

What is Pemphigus vulgaris?

Answer 5

blistering disease within epidermis

loss of water

electrolyte disruption

Question 6

Where does Pemphigus vulgaris often begin?

Answer 6

erosions of the oral mucosa

Question 7

What is the histology of pemphigus vulgaris?

Answer 7

loss of normal epithelial adhesion = detachment

Question 8

what is the autoantigen responsible for cell-cell adhesion in the epidermis?

Answer 8

DESMOSOMES

Question 9

In pemphigus vulgaris, why are there depositions of IgG and complement around keratinocytes?

Answer 9

connects the keratinocytes so it can form a layer and blister off

Question 10

At what skin layer does pemphigus vulgaris occur?

Answer 10

above the stratum basale

Question 11

What is immunosuppressive therapy used for dogs with Pemphigus vulgaris?

Answer 11

cyclosporin A

Question 12

What is the blistering dieased called when the detachment occurs above the stratum spinosum?

Answer 12

Pemphigus foliaceus

Question 13

Why is diagnosing pemphigus foliaceus an issue?

Answer 13

misdiagnose for eczema

Question 14

What is the auto-antibody for pemphigus foliaceus?

Answer 14

anti-Dsg1

destroy desmosomes

Question 15

What is Fogo Selvagem?

Answer 15

endemic P. foliaceus

river valleys of Brazil

black fly bites

Question 16

What is therapy for Pemphigus vulgaris?

Answer 16

Steroids (in combo with immunosuppressants)

Question 17

What is SLIT?

Answer 17

Sublingual Immunotherapy

under tongue

improves atopic dermatitis to dust mites

Question 18

What is Rituximab?

Answer 18

takes out B-cells which form the auto-antibodies

helps P. vulgaris

Question 19

Why is Rituximab safe?

Answer 19

takes out B-cells BUT does NOT touch plasma cells

still have memory cells

Question 20

What is an example of systemic autoimmune disease?

Answer 20

lupus erythematosus

Question 21

What is Lupus?

Answer 21

multisystem

young women

autoantibodies to nuclear antigens

Question 22

What does Lupus effect?

Answer 22

joints - arthritis
kidnye
heart
vasculitis allergica

Question 23

What is vasculitis?

Answer 23

leaky vessles - inflammation on wall

Question 24

What is chronic discoif LE?

Answer 24

very common lesion on lupus patients - leaves scar

Question 25

What triggers chronic-discoid LE?

Answer 25

UV
infection
cold exposure
stress

Question 26

What are clinical manifestation of neonatal lupus?

Answer 26

rash
anemia
leukopenia
thrombocytopenia
congenital atrioventricular block

Question 27

does neonatal lupus last?

Answer 27

No

when baby develops its own antibodies it goes away

Question 28

What inhibits Anti-Ro binding in lupus?

Answer 28

EBNA-1

Epstein-Barr virus nuclear antigen

Question 29

What happens when there is UV exposure in Lupus patient?

Answer 29

some skin cells go into apoptosis

no clearance of “burnt” cells

induces exposure of “Ro”

Question 30

What cytokine induces apoptosis in lupus

Answer 30

IL-18

Question 31

What doe HPK in Lupus patients produce upon stimulation with IL-18?

Answer 31

TNFa
drives the apoptosis

Infliximab could prevent this

Question 32

What is therapy for Lupus?

Answer 32

protect from UV

chloroquin - prevetns action of TLR7/9

immunosuppression

Question 33

What is systemic therapy for Lupus?

Answer 33

MTX