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Extended report
FMF50: a score for assessing outcome in familial Mediterranean fever
  1. Seza Ozen1,
  2. Erkan Demirkaya2,
  3. Ali Duzova1,
  4. Ozlem Erdogan3,
  5. Eren Erken4,
  6. Ahmet Gul5,
  7. Ozgur Kasapcopur6,
  8. Timucin Kasifoglu7,
  9. Bunyamin Kisacik8,
  10. Huri Ozdogan5,
  11. Mehmet Tunca9,
  12. Cengizhan Acikel10,
  13. for the FMF Arthritis Vasculitis and Orphan disease Research in pediatric rheumatology (FAVOR) and Turkish FMF study group
  1. 1Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey
  2. 2Department of Pediatrics, Gulhane Military Academy, Ankara, Turkey
  3. 3Department of Pediatrics, Sami Ulus Children's Hospital, Ankara, Turkey
  4. 4Department of Rheumatology, Cukurova University, Adana, Turkey
  5. 5Department of Rheumatology, Istanbul University, Capa Medical Faculty, Istanbul, Turkey
  6. 6Department of Pediatrics and Rheumatology, Istanbul University Cerrahpasa Medical Faculty, Istanbul, Turkey
  7. 7Department of Rheumatology, Osman Gazi University Eskisehir, Eskisehir, Turkey
  8. 8Department of Rheumatology, Gaziantep University Faculty of medicine, Gaziantep, Turkey
  9. 9Department of Internal Medicine, Dokuz Eylul University, Izmir, Turkey
  10. 10Gulhane Military Academy, Ankara, Turkey
  1. Correspondence to Professor Seza Ozen, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara 6100, Turkey; sezaozen{at}


Background Colchicine is the main treatment for familial Mediterranean fever (FMF). However, biological agents and other treatments are available for patients who are unable to receive optimal treatment.

Objective To develop outcome criteria that define response to treatment.

Methods Two rounds of Delphi exercise were followed by a consensus conference enabling the definition of the criteria to be employed. Data for patients with FMF responding and resistant to their treatment were obtained from the FMF Arthritis Vasculitis and Orphan disease Research in paediatric rheumatology (FAVOR) website. The suggested criteria were analysed and validated in this patient cohort. Sensitivity/specificity measures and the ability of the score to discriminate between patients with active and inactive disease via the best cut-off score were calculated by a receiver operating characteristic analysis.

Results Compliance with the maximum dose of the drug was considered essential for evaluation of the patients. Seven criteria were suggested in the consensus conference. The performance of each criterion, in differentiating between resistant and responsive patients, was tested. The final set of criteria was defined as at least 50% improvement in five of six criteria, without worsening in any one defined response to treatment with a very high sensitivity and specificity. The items of this FMF50 included:

1. Percentage change in the frequency of attacks with the treatment.

2. Percentage change in the duration of attacks with the treatment.

3. Patients/parents’ global assessment of disease severity (10 cm visual analogue scale (VAS)).

4. Physicians’ global assessment of disease severity (10 cm VAS).

5. Percentage change in arthritis attacks with the treatment.

6. Percentage change in C-reactive protein, erythrocyte sedimentation rate or serum amyloid A level with the treatment.

Conclusions The FMF50 produced is a user-friendly measurement tool to guide physicians and can be used in clinical trials.

  • Familial Mediterranean Fever
  • Outcomes research
  • Treatment

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