title: Different disease subtypes with distinct clinical expression in familial Mediterranean fever: results of a cluster analysis.
abstract: OBJECTIVE
  The aim of this study was to evaluate whether there are clinical subgroups that may have different prognoses among FMF patients.
  
  
  METHODS
  The cumulative clinical features of a large group of FMF patients [1168 patients, 593 (50.8%) male, mean age 35.3 years (s.d. 12.4)] were studied. To analyse our data and identify groups of FMF patients with similar clinical characteristics, a two-step cluster analysis using log-likelihood distance measures was performed. For clustering the FMF patients, we evaluated the following variables: gender, current age, age at symptom onset, age at diagnosis, presence of major clinical features, variables related with therapy and family history for FMF, renal failure and carriage of M694V.
  
  
  RESULTS
  Three distinct groups of FMF patients were identified. Cluster 1 was characterized by a high prevalence of arthritis, pleuritis, erysipelas-like erythema (ELE) and febrile myalgia. The dosage of colchicine and the frequency of amyloidosis were lower in cluster 1. Patients in cluster 2 had an earlier age of disease onset and diagnosis. M694V carriage and amyloidosis prevalence were the highest in cluster 2. This group of patients was using the highest dose of colchicine. Patients in cluster 3 had the lowest prevalence of arthritis, ELE and febrile myalgia. The frequencies of M694V carriage and amyloidosis were lower in cluster 3 than the overall FMF patients. Non-response to colchicine was also slightly lower in cluster 3.
  
  
  CONCLUSIONS
  Patients with FMF can be clustered into distinct patterns of clinical and genetic manifestations and these patterns may have different prognostic significance.
authors.affiliation: Division of Rheumatology, Izmir Kâtip Çelebi University School of Medicine, Izmir, servet.akar@gmail.com.
authors.forename: Servet
authors.initials: S
authors.lastname: Akar
authors.affiliation: Department of Internal Medicine, Division of Rheumatology, Namik Kemal University School of Medicine, Tekirdag.
authors.forename: Dilek
authors.initials: D
authors.lastname: Solmaz
authors.affiliation: Department of Internal Medicine, Division of Rheumatology, Osmangazi University School of Medicine, Eskisehir.
authors.forename: Timucin
authors.initials: T
authors.lastname: Kasifoglu
authors.affiliation: Department of Internal Medicine, Division of Rheumatology, Osmangazi University School of Medicine, Eskisehir.
authors.forename: Sule Yasar
authors.initials: SY
authors.lastname: Bilge
authors.affiliation: Division of Rheumatology, Dokuz Eylul University School of Medicine.
authors.forename: Ismail
authors.initials: I
authors.lastname: Sari
authors.affiliation: Department of Internal Medicine, Izmir Kâtip Çelebi University School of Medicine and.
authors.forename: Zeynep Zehra
authors.initials: ZZ
authors.lastname: Gumus
authors.affiliation: Department of Internal Medicine, Dokuz Eylul University School of Medicine, Izmir, Turkey.
authors.forename: Mehmet
authors.initials: M
authors.lastname: Tunca
mesh_terms: D000328:Adult; D000686:Amyloidosis / Q000453:epidemiology / Q000209:etiology*; D001168:Arthritis / Q000453:epidemiology / Q000209:etiology*; D016000:Cluster Analysis; D010505:Familial Mediterranean Fever / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology*; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D063806:Myalgia / Q000453:epidemiology / Q000209:etiology*; D015995:Prevalence; D012042:Registries; D014421:Turkey / Q000453:epidemiology
keywords: cluster analysis; familial Mediterranean fever; marenostrin; phenotype