familial Mediterranean feveer

Question 1

what is familial Mediterranean fever?

Answer 1

autosomal recessive disorder, caused by mutations in the MEFV gene located on chromosome 16

Question 2

what is the other name for FMF ?

Answer 2

recurrent polyserositis

Question 3

what is the clinical picture of FMF ?

Answer 3

recurrent episodes of fever
painful serositis - pleuritis , synovitis or peritonitis
lower limb arthritis
erysipelas like rash
amylloidosis ( due to amyloiid A deposition in the kidney )

Question 4

what is the age of onset of FMF ?

Answer 4

always in people below the age of 25

Question 5

what is the effect of FMF on the kidneys ?

Answer 5

amyloid deposition

Question 6

how long do the paroxysmal attacks of FMF last for ?

Answer 6

48-96 hours with a peak happening at 12 hours followed by a plateau with resolution

Question 7

what is the abdominal clinical presentation of FMF consistent with ?

Answer 7

appendicitis or cholecystitis and may mimic renal colic

Question 8

what are the bowel movements like in the paroxysmal attacks of FMF ?

Answer 8

constipation during the attack
diarrhea after the attack resolves

Question 9

what type of vasculitis is associated with FMF ?

Answer 9

henoch schonlein purpura

Question 10

what is expected to be found on x-ray film of FMF ?

Answer 10

air-fluid levels

Question 11

what criteria is used to diagnose FMF ?

Answer 11

tel-hashomer and Livneh criteeria

Question 12

what can be used to prevent attacks of FMF ?

Answer 12

colchicine

Question 13

what is the next best step in management for colchicine resistant patients ?

Answer 13

biologic therpay
IL-1 - anakinra