We diagnose FMF in patients with any one of the following:

• ≥ 1 major criteria
• ≥ 2 minor criteria
• 1 minor plus 5 supportive criteria
• 1 minor criterion plus ≥ 4 of the first five supportive criteria

Major criteria — Major criteria for the clinical diagnosis of FMF include the following:

• Typical attacks with peritonitis (generalized)
• Typical attacks with pleuritis (unilateral) or pericarditis
• Typical attacks with monoarthritis (hip, knee, ankle)
• Typical attacks with fever alone

Typical attacks are defined as recurrent (≥3 of the same type), febrile (≥38°C) and short (lasting between 12 hours and 3 days).

Minor criteria — Minor criteria for the clinical diagnosis of FMF include the following:

• Incomplete attacks involving chest pain
• Incomplete attacks involving monoarthritis
• Exertional leg pain
• Favorable response to colchicine

Incomplete attacks are defined as painful and recurrent attacks not fulfilling criteria for a typical attack.

Supportive criteria — Supportive criteria for FMF include the following:

• Family history of FMF
• Appropriate ethnic origin
• Age <20 years at disease onset
• Severe attack requiring bed rest
• Spontaneous remission of attack
• Symptom-free interval between attacks
• Attacks associated with transient inflammatory response with one or more abnormal lab results for white blood cell count, erythrocyte sedimentation rate, serum amyloid A, and/or fibrinogen
• Episodic proteinuria/hematuria
• Negative laparotomy or removal of normal appendix
• Consanguinity of parents

This approach is consistent with criteria developed at the Tel Hashomer Medical Center in Israel.