In conclusion, we showed that in CAPS patients: (1) CKD is relatively common; (2) development of CKD may be due to a familial risk beyond the mutational status and/or the result of a potential second hit; (3) anti-IL-1 therapy significantly improves inflammatory parameters and symptom burden; (4) development of CAPS-associated CKD may be prevented by therapies targeting IL-1, while renal outcome may not be predicted once renal damage is already present. The gene discussed is IL1B; the disease is cryopyrin-associated periodic syndrome.