Table 2.

Classification criteria for the clinical diagnosis of familial Mediterranean fever (FMF).

Tel Hashomer Criteria	Livneh’s Criteria
Major	Major
Recurrent fevers + peritonitis/pleurisy/serositis	Typical attack of peritonitis
AA-amyloidosis	Typical attack of unilateral pleuritic or pericarditis
Favourable response to prophylaxis with colchicine	Typical attack of monoarthritis
Minor	Fever (rectal temperature of 38 °C or higher) alone
Recurrent fevers	Minor
Erysipelas-like erythema	Incomplete attack involving the abdomen
Family history of FMF in a first-degree relative	Incomplete attack involving the chest
	Incomplete attack involving one large joint
	Exertional leg pain
	Favourable response to prophylaxis with colchicine
	Supportive
	Family history of familial Mediterranean fever
	Typical ethnic origin (Armenian, Turkish, Arabian, Sephardic Jew)
	Age less than 20 years at disease onset
	Severity of attacks requiring bed rest
	Spontaneous remission of attacks
	Symptom-free intervals between attacks
	Transient increase of inflammatory parameters during attacks
	Episodic proteinuria or hematuria
	Surgical removal of a “white” appendix
	Consanguinity of parents

Diagnosis of FMF is made when 2 major criteria or 1 major and 2 minor criteria are satisfied (according to the Tel Hashomer criteria); diagnosis requires ≥ 1 major criteria, or ≥2 minor criteria, or 1 minor criterion plus ≥ 5 supportive criteria or 1 minor criterion plus ≥ 4 of the “first” five supportive criteria (according to Livneh’s criteria). Note: “Incomplete” attacks are defined as painful and recurrent flares that differ from typical attacks in 1 or 2 features, as follows: (a) normal temperature or lower than 38 °C; (b) attacks longer than 1 week or shorter than 6 h; (c) no signs of peritonitis recorded during acute abdominal attacks.