Livneh et al developed criteria for the diagnosis of Familial Mediterranean Fever (FMF) in a patient based on clinical findings. The authors are from Sheba Medical Center and Tel Aviv University in Israel.
Major criteria:
(1) typical attack with generalized peritonitis
(2) typical attack with unilateral pleuritis or pericarditis
(3) typical attack with monoarthritis of hip, knee or ankle
(4) typical attack with fever alone
Minor criteria:
(1) incomplete attack involving the abdomen
(2) incomplete attack involving the chest
(3) incomplete attack with monoarthritis
(4) exertional leg pain
(5) favorable response to therapeutic trial of colchicine
Supportive criteria:
(1) family history of Familial Mediterranean Fever
(2) appropriate ethnic origin
(3) age of disease onset < 20 years of age
(4) attack severe enough to require bed rest
(5) attack with spontaneous remission
(6) symptom-free intervals between attacks
(7) attack with transient inflammatory response (presence of one or more markers of leukocytosis, elevated ESR, elevated C reactive protein, etc.)
(8) episodic proteinuria and/or hematuria
(9) unproductive laparotomy or removal of a normal appendix
(10) consanguinity of parents
|
Major Criteria
|
Minor Criteria
|
Supportive Criteria
|
Diagnosis
|
|
>= 1
|
NA
|
NA
|
FMF present
|
|
0
|
>= 2
|
NA
|
FMF present
|
|
0
|
1
|
4 or 5 of first 5
|
FMF present
|
|
0
|
1
|
>=5
|
FMF present
|
