Key result
Cluster analysis of 1,168 patients with familial Mediterranean fever identified 3 distinct disease subtypes with varying clinical manifestations, M694V carriage, and amyloidosis prevalence.
Why the study?
Are there distinct clinical subgroups with different prognoses among patients with familial Mediterranean fever?
Observational (n=1,168)
Are there distinct clinical subgroups with different prognoses among patients with familial Mediterranean fever?
Patients with familial Mediterranean fever can be clustered into three distinct clinical and genetic patterns that may have different prognostic significance.
FMF subtyping may refine amyloidosis risk assessment; hypothesis-generating and requires prospective validation.
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. CONCLUSION: Patients with FMF can be clustered into distinct patterns of clinical and genetic manifestations and these patterns may have different prognostic significance.
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Akar et al. (2015) conducted an observational in Familial Mediterranean fever (n=1,168). Disease subtypes was evaluated on Clinical subgroups (clusters). Cluster analysis of 1,168 patients with familial Mediterranean fever identified 3 distinct disease subtypes with varying clinical manifestations, M694V carriage, and amyloidosis prevalence.
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