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 arthritic joint disease.