Autoinflammatory diseases

Question 1

What are autoinflammatory diseases?

Answer 1

Heterogenous group of inherited disorders
No apparent autoimmune basis
Absense of high titre autoantibodies and antigen specific autoreactive T cells

Question 2

Examples of dominant autoinflammatory diseases

Answer 2

TRAP, Muckle-Wells

Question 3

Examples of recessive autoinflammatory diseases

Answer 3

FMF, DIRA, DITRA

Question 4

Resolution

Answer 4

Resolution is very important in inflammation - can have persistence of the trigger or lack of counter regulatory pathways

Question 5

DIRA

Answer 5

IL-1 antagonist - deficiency of IL-1RA
Leads to osteomyelitis, periostitis and pustulosis
Treat with anakinra - leads to the majority of removal of skin symptoms

Question 6

Function of IL-1

Answer 6

Attracts neutrophils and keratinocytes are a source of neutrophils

Question 7

Gout

Answer 7

High urate levels
Forms crystals and activates inflammatory pathways
Uses a lot of IL-1
Usually starts in the big toe 
Uses anakinra for treatment
IS NOT GENETIC

Question 8

Psoriasis

Answer 8

Silvery, scaley rash on skin
Independent risk factor for heart disease
Neutrophils and T cells under the skin
Il-36 leads to IL-8 release which recruits neutrophils

Question 9

Pustular psoriasis

Answer 9

IL-36A DITRA

Increased IL-36y - needs an N terminal truncation for activation so needs IL-1B caspase