Detailed criteria for the diagnosis of familial Mediterranean fever

Major criteria

Typical attacks

1. Peritonitis (generalized)

2. Pleuritis (unilateral) or pericarditis

3. Monoarthritis (hip, knee, ankle)

4. Fever alone

Minor criteria

1-3. Incomplete attacks involving one or more of the following sites:

1. Abdomen

2. Chest

3. Joint

4. Exertional leg pain

5. Favorable response to colchicine

Supportive criteria

1. Family history of FMF

2. Appropriate ethnic origin

3. Age <20 years at disease onset

4-7. 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 the white blood cell count, erythrocyte sedimentation rate, serum amyloid A, and/or fibronogen

8. Episodic proteinuria/hematuria

9. Negative laparotomy or removal of normal appendix

10. Consanguinity of parents

FMF: familial Mediterranean fever.

Adapted from: Livneh A, Langevitz P, Zemer D, et al. Arthritis Rheum 1997; 40:1879.

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Detailed criteria for the diagnosis of familial Mediterranean fever