MEFV and familial Mediterranean fever: A 2014 study of Japanese FMF patients also demonstrated that the serum IL-18 level in FMF patients in remission was significantly higher in typical FMF with M694I mutation than in atypical FMF with exon2 variants including E148Q.47 Based on our present data and previous findings, we speculate that in FMF, a pathogenic mutation in exon 10 facilitates IL-18-dependent subclinical inflammation via an aberrant regulation of the pyrin inflammasome cascade.