A mutation in NLRP1 was also identified in systemic juvenile idiopathic arthritis patients resulting in a novel monogenic disorder autoinflammation with arthritis and dyskeratosis (AIADK)24, while a patient presenting with corneal intraepithelial dyskeratosis, mucosal inflammation, tooth abnormalities and eczema further expands the association of NLRP1 with autoimmune arthritis and eye disease25. Here, NLRP1 is linked to systemic-onset juvenile idiopathic arthritis.