Criteria

Diagnostic criteria for FMF[61]

Major criteria

Typical attacks:

  • Peritonitis (generalised)

  • Pleuritis (unilateral) or pericarditis

  • Monoarthritis (hip, knee, ankle).

Minor criteria

One to three incomplete attacks involving one or more of the following sites:

  • Abdomen

  • Chest

  • Joint

  • Exertional leg pain

  • Favourable response to colchicine.

Supportive criteria

  • Family history of FMF

  • Appropriate ethnic origin

  • Age <20 years at disease onset

  • Features of attacks:

    • Severe, requiring bed rest

    • Spontaneous remission

    • Symptom-free interval

  • Transient inflammatory response, with one or more abnormal test result(s) for WBC count, C-reactive protein, erythrocyte sedimentation rate, serum amyloid A, and/or fibrinogen

  • Episodic proteinuria/haematuria

  • Unproductive laparotomy or removal of ‘white’ appendix

  • Consanguinity of parents.

The requirements for the diagnosis of FMF are either:

  • One or more major criterion

  • Two or more minor criteria

  • One minor criterion plus five or more supportive criteria

  • One minor criterion plus four or more of the first five supportive criteria.

Typical attacks are defined as recurrent (≥3 of the same type), febrile (rectal temperature of 38°C (100°F) or higher), and short (lasting between 12 hours and 3 days).

Incomplete attacks are defined as painful and recurrent, differing from typical attacks in 1 or 2 features, as follows:

  • The temperature is normal or lower than 38°C (100°F)

  • The attacks are longer or shorter than specified (but no shorter than 6 hours or longer than a week)

  • No signs of peritonitis are recorded during the abdominal attacks

  • The abdominal attacks are localised

  • The arthritis is in joints other than those specified.

Attacks are not counted if they do not fit the definition of either typical or incomplete attacks.

Diagnostic criteria for FMF in childhood[63]

Children who have two or more of the following:

  • Fever: axillary temperature of >38°C, 6–72 hours of duration, ≥3 attacks

  • Abdominal pain: 6-72 hours of duration, ≥3 attacks

  • Chest pain: 6-72 hours of duration, ≥3 attacks

  • Arthritis: 6-72 hours of duration, ≥3 attacks, oligoarthritis

  • Family history of FMF.

Use of this content is subject to our disclaimer