Periodic Fevers

Question 1

Which part of the immune system is involved

Answer 1

Innate

Episodes are antigen-independent

Absence of infection and auto-abs

Question 2

FMF

Answer 2

Autosomal recessive

Age <20 (jews, arabs, turks, armenian, italian)

Duration: 1-3 days

Clinical features: erysipelas-like rash, sterile peritonitis, monoarthritis, pleuritis

Gene: MEFV

Protein: Pyrin

Question 3

TRAPS

Answer 3

Autosomal dominant or de novo

Age: <20, any ethic group

Duration: >7 dyas

Features: myalgias w/rash, conjunctivitis, periorbital edema, peritonitis, large joint arthritis

Gene: TNFRSF1A

Protein: TNF receptor type 1

Question 4

HIDS

Answer 4

Autosomal recessive

Age <1, dutch/french/european

Duration: 3-7 days

Features: maculopapular rash, abd pain, polyarthritis, spleomegaly, cervial adenopathy, aphthous ulcers

Gene: MVK

Protein: mevalonate kinase

Question 5

FCAS (Familial cold autoinflammatory syn)

Answer 5

Autosomal dominant

Age <1, europeans

Fevers: <2days

Clinical: cold induced urticarea, arthralgia, conjunctivitis