MVK and mevalonic aciduria: Biallelic pathogenic variants in MVK have also been found to result in an autoinflammatory disease, with a clinical spectrum ranging from periodic fever and autoinflammation to severe and early lethal clinical presentation as seen in patients with mevalonic aciduria.4, 5, 6 Patients with MKD usually excrete elevated levels of urinary mevalonic acid during disease flares.6