Ethnic groups affected:
(1) Sephardic Jews
(2) Arabs
(3) Turks
(4) Armenians
Theory for pathogenesis:
(1) A deficiency of an enzyme that inhibits chemotaxis of neutrophils, which results in a diminished anti-inflammatory response to minor injury. One candidate molecule is C5a inhibitor.
(2) The chemotaxis causes activation of neutrophils with triggering of an inflammatory response.
(3) The affected gene is located on the short arm of chromosome 16 near the alpha-globulin gene.
Features of the recurrent attacks:
(1) generally severe, requiring bed rest
(2) remit spontaneously
(3) separated from the next by symptom-free intervals
Major Criteria:
(1) rectal temperature > 38°C for 12 to 72 hours
(2) recurrent pain of same type
(3) one or more of the following inflammatory conditions
(3a) unilateral pleuritis
(3b) pericarditis
(3c) generalized peritonitis
(3d) monoarthritis involving knee, ankle or hip
(3e) inflammation of the tunica vaginalis testis in males
(3f) erysipelas-like skin eruption
(3g) symmetric myalgia in calves, with extreme tenderness
Minor Criteria:
(1) clinical response to colchicine
(2) incomplete attacks (involving chest, abdomen or joints)
Supportive Criteria:
(1) family history of Familial Mediterranean Fever
(2) age of onset < 10 years of age
