Table 3.

Classification Criteria for the Clinical Diagnosis of Familial Mediterranean Fever (FMF) according to Livneh et al. Diagnosis of FMF requires ≥ 1 major criteria, or ≥ 2 minor criteria, or 1 minor criterion plus ≥5 supportive criteria (family history of FMF, appropriate ethnic origin, age less than 20 years at disease onset, severity of attacks requiring bed rest, spontaneous remission of symptoms, presence of symptom-free intervals, transient elevation of inflammatory markers, episodic proteinuria or hematuria, nonproductive laparotomy with removal of a “white” appendix, consanguinity of parents) or 1 minor criterion plus ≥ 4 of the “first” five supportive criteria. “Incomplete” attacks are defined as painful and recurrent flares that differ from typical attacks in 1 or 2 features, as follows: 1) normal temperature or lower than 38°C; 2) attacks longer than 1 week or shorter than 6 hours; 3) no signs of peritonitis recorded during acute abdominal complaint.

Livneh’s criteria
Major criteria
Typical attack of generalized peritonitis
Typical attack of unilateral pleuritis/pericarditis
Typical attack of monoarthritis
Presence of fever alone (rectal temperature of 38°C or higher)
Minor criteria
Incomplete attack involving abdomen
Incomplete attack involving chest
Incomplete attack involving one large joint
Exertional leg pain
Favorable response to colchicine