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lüll Current status of understanding the pathogenesis and management of patients with NOMID/CINCA Goldbach-Mansky RCurr Rheumatol Rep 2011[Apr]; 13 (2): 123-31Neonatal-onset multisystem inflammatory disease (NOMID)/chronic infantile neurologic, cutaneous, and arthritis (CINCA) syndrome is the most severe clinical phenotype in the spectrum of cryopyrin- (NLRP3/NALP3) associated periodic syndromes (CAPS). The study of patients with NOMID/CINCA has been instrumental in characterizing the extent of organ-specific inflammatory manifestations and damage that can occur with chronic interleukin (IL)-1beta overproduction. Mutations in CIAS1/NLRP3 lead to constitutive activation of the "NLRP3 inflammasome," an intracellular platform that processes and secretes increased amounts of IL-1beta. The pivotal role of IL-1beta in NOMID/CINCA has been demonstrated in several clinical studies using IL-1--blocking agents that lead to rapid resolution of the inflammatory disease manifestations. NOMID/CINCA is a monogenic autoinflammatory syndrome; and the discovery of the role of IL-1 in NOMID has led to the exploration in the role of IL-1 in other disorders including gout and Type II diabetes. The inflammation in NOMID/CINCA is continuous with intermittent flares, and organ manifestations encompus the central nervous system, eye, inner ear, and bones. This review discusses updates on the pathogenesis of NOMID/CAPS, emerging long term-outcome data regarding IL-1--blocking agents that have influenced our considerations for optimal treatment, and a monitoring approach tailored to the patient's disease severity and organ manifestations.|Adolescent[MESH]|Animals[MESH]|Antirheumatic Agents/*therapeutic use[MESH]|Carrier Proteins/genetics[MESH]|Child[MESH]|Cryopyrin-Associated Periodic Syndromes/*drug therapy/*genetics/pathology[MESH]|Drug Evaluation, Preclinical[MESH]|Humans[MESH]|Infant, Newborn[MESH]|Infant, Newborn, Diseases/*drug therapy/*genetics/pathology[MESH]|Inflammasomes/drug effects[MESH]|Interleukin 1 Receptor Antagonist Protein/*therapeutic use[MESH]|Interleukin-1beta/antagonists & inhibitors/metabolism[MESH]|Mutation[MESH]|NLR Family, Pyrin Domain-Containing 3 Protein[MESH]|Syndrome[MESH] |