MEFV mutations - therapeutic guides or red herrings?

Background/question Familial Mediterranean Fever (FMF) is a hereditary inflammatory disorder characterised by self-resolving attacks of fever and serositis common in populations from Mediterranean ancestry [1]. Mutations affecting MEFV gene is believed to be responsible for the disease phenotype[1]. The correlation between the genotype and phenotype is not very strong, indicating the presence of other modifying factors which alter clinical manifestation. We describe 2 children with autoinflammtory symptoms, who had MEFV mutations, the significance of which we are unsure of.