Acknowledging that the difficulties in linking genotype and phenotype in FMF are caused by an incomplete understanding of the molecular pathogenic mechanism underlying FMF, a recently reported consensus-driven pathogenicity classification was able to classify most variants in three genes causing other AID (MVK, NLRP3 and TNFRSF1A), but almost half of the MEFV variants (42.4%) could not be classified or were classified as ‘variants of uncertain significance’.5 This evidence concerns the gene TNFRSF1A and familial Mediterranean fever.