NLRP3 and cryopyrin-associated periodic syndrome: In a multicenter study of 45 patients with low penetrance NLRP3 variants including 19 with Q705K vs 28 CAPS patients with known pathogenic variants, the former patients had autoinflammatory features, such as fever (76%), headaches (73%), rash (80%), sensorineural hearing loss (18%), arthralgia (84%), abdominal pain (56%), diarrhea (18%), and elevated ESR/CRP(26%-34%) (9); these clinical findings and their frequencies are quite similar to those in our patients with NLRP3 variants (Table 3).