Renal transplantation in patients with cryopyrin-associated periodic syndrome: a case report and literature review. null

The cryopyrin-associated periodic syndrome (CAPS) is a rare autosomal dominant hereditary inflammatory disease clinically characterized by three overlapping types and associated with interleukin (IL)-1β. Clinical subtypes include: Familial Cold Autoinflammatory Syndrome (FCAS), Muckle-Wells Syndrome (MWS), and Neonatal-Onset Multisystem Inflammatory Disorder (NOMID), also known as Chronic Infantile Neurological Cutaneous and Articular Syndrome (CINCA). The estimated prevalence of the disease is 1/360,000, according to a French study, and it is caused by mutations in the NLRP3 gene(1). The clinical manifestations of CAPS include urticaria-like skin rash, sensorineural deafness, amyloidosis, skeletal abnormalities, and renal involvement that can ultimately progress to renal failure. In this article, we report a case of a CAPS patient with renal failure who underwent kidney transplantation at our hospital, with no recurrence observed during follow-up after surgery.