title: Evaluation of disease severity in familial Mediterranean fever.
abstract: OBJECTIVE
  To establish a new, objective, statistically based severity score for familial Mediterranean fever (FMF).
  
  
  METHODS
  One hundred consecutive FMF patients were evaluated independently by 2 FMF experts for severity of their disease and were assigned to 1 of 3 severity levels: mild, intermediate, or severe. Nine candidate criteria, reflecting objective suffering and disability, were analyzed to determine their weight for patient placement in the 3 predefined severity groups.
  
  
  RESULTS
  Candidate criteria best differentiating between the 3 patient categories were the frequency of attacks, the number of sites affected during an attack and during the course of the disease, and the duration of the attacks. These criteria were applied in a classification-tree model to establish a new FMF-severity score (F-SS). The first set of F-SS (F-SS-1) was highly sensitive and specific. Integrating F-SS-1 with clinical parameters strongly associated with disease severity resulted in a simplified score, the second set of F-SS (F-SS-2).
  
  
  CONCLUSIONS
  New, useful, objective, and valid severity scores were established and found to distinguish between patients with mild, intermediate, and severe diseases with high sensitivity and specificity.
  
  
  CONCLUSIONS
  The F-SS established may be important for treatment decisions, prognosis evaluation, and comparative analysis of patient populations.
authors.affiliation: Sheba Medical Center, Tel-Hashomer and Sackler School of Medicine, Tel-Aviv University, Tel-Aviv, Israel.
authors.forename: Adam
authors.initials: A
authors.lastname: Mor
authors.forename: Yael
authors.initials: Y
authors.lastname: Shinar
authors.forename: Nurit
authors.initials: N
authors.lastname: Zaks
authors.forename: Pnina
authors.initials: P
authors.lastname: Langevitz
authors.forename: Angela
authors.initials: A
authors.lastname: Chetrit
authors.forename: Shmuel
authors.initials: S
authors.lastname: Shtrasburg
authors.forename: Einat
authors.initials: E
authors.lastname: Rabinovitz
authors.forename: Avi
authors.initials: A
authors.lastname: Livneh
mesh_terms: D000328:Adult; D015331:Cohort Studies; D004185:Disability Evaluation; D010505:Familial Mediterranean Fever / Q000145:classification / Q000473:pathology / Q000503:physiopathology*; D005260:Female; D006304:Health Status; D006801:Humans; D008297:Male; D012219:Rheumatology / Q000379:methods*; D012720:Severity of Illness Index; D016037:Single-Blind Method; D011795:Surveys and Questionnaires