The disease is caused by a gain-of-function mutation in MEFV (Mediterranean FeVer) gene, causing an excessive activation of pyrin inflammasome and thus overproduction of proinflammatory cytokines.1 Spondyloarthropathies accompanying FMF include inflammatory arthritides, sacroiliitis, spondylitis, enthesitis, and extraskeletal manifestations, and in children, enthesitis-related arthritis (ERA).2 Although the evidence supporting the role of innate immunity is overwhelming, the exact pathogenesis of the aforementioned diseases is yet to be determined. This evidence concerns the gene MEFV and enthesitis-related juvenile idiopathic arthritis.