Familial Mediterranean Fever in Iran: A Report from FMF Registration Center

الملخص EN

Background.

Familial Mediterranean fever (FMF) is a periodic AR autoinflammatory disorder.

This comprehensive study describes FMF in Iran as a country near Mediterranean area.

Materials and Methods.

From the country FMF registration center 403 patients according to Tel-Hashomer criteria enrolled this study, 239 patients had MEFV gene mutations analyses.

Data, if needed, was analyzed by SPSS v20.

Results.

175 patients (43.4%) were female and 228 patients (56.6%) were male.

The mean age was 21.3 years.

Abdominal pain was in 93.3% patients and 88.1% had fever.

Abdominal pain was the main complaint of patients in (49.6%).

The mean interval between attacks was 36.5±29.6 days and the mean duration of every episodes was 43.3±34.5 hours.

15.1% of patients had positive family history and 12.7% had previous surgery; in 52.3% of patients delay in diagnosis was more than three years.

12 common MEFV gene mutations were analyzed, 21.33% were without mutations, 39.7% had compound heterozygote, 25.52% showed heterozygous, and 13.38% showed homozygous results.

The most common compound genotype was M694V-V726A (% 10.46) and in alleles M694V (% 20.9) and V726A (% 12.7) were the most frequent mutations, respectively.

Conclusion.

M694V was the most common mutation, and the most common compound genotype was M694V-V726A.

Our genotype results are similar to Arabs and in some way to Armenians, erysipelas-like skin lesions are not common in this area, and clinical criteria are the preferred methods in diagnosis of FMF.