Table 1 Clinical diagnostic criteria for familial Mediterranean fever (FMF)

Tel-Hashomer Criteria (adapted from reference #3: Livneh A, et al. Arthritis Rheum 1997;40:1879–1885)

Long Version

Major Criteria

 Typical attacks (≥3 of the same type, rectal temp ≥38 °C, attacks lasting 12 h to 3 days)

 1.Peritonitis

 2.Pleuritis (unilateral) or pericarditis

 3.Monoarthritis (hip, knee, ankle)

 4.Fever alone

Minor Criteria

 1. Incomplete attacks (typical attacks including one of the following sites: abdomen, chest or joint with 1 or 2 of the following exceptions: 1) Temperature < 38 °C, 2) attacks lasting 6–12 h or 3–7 days, 3) no signs of peritonitis during abdominal attacks, 4) localized abdominal pain, 5) arthritis in joints other than hip, knee or ankle)

 2. Exertional leg pain

 3. Favorable response to colchicine

Supportive Criteria

 1. Family history of FMF

 2. Appropriate ethnic origin

 3. Age <20 year at disease onset

 4–7 are related to features of attacks:

 4. Severe, requiring bed rest

 5. Spontaneous remission

 6. Symptom-free interval

 7. Transient inflammatory response with one or more abnormal test result(s) for white blood cell count, erythrocyte sedimentation rate, serum amyloid A, and /or fibrinogen

 8. Episodic proteinuria/hematuria

 9. Unproductive laparotomy or removal of “white” appendix

 10. Consanguinity of parents

 An FMF diagnosis requires ≥1 major criteria, or ≥2 minor criteria, or 1 minor criteria plus ≥5 supportive criteria, or 1 minor criteria plus ≥4 of the first 5 supportive criteria.

Short Version

Major criteria

 1.Recurrent febrile attacks accompanied by peritonitis, synovitis or pleuritis.

 2.Amyloidosis of the AA-type without predisposing disease.

 3.Favorable response to continuous colchicine treatment.

Minor criteria

 4. Recurrent febrile attacks

 5. Erysipelas-like erythema

 6. FMF in a first degree relative

Definitive diagnosis: 2 major or 1 major and 2 minor.

Probable diagnosis: 1 major and 1 minor

Yalçinkaya Pediatric Criteria (adapted from reference #4: Yalçinkaya F, et al. Rheumatology (Oxford, England) 2009;48:395–8)