Granulomatous autoinflammatory diseases include Blau syndrome (BS, OMIM 186580) and early-onset sarcoidosis (EOS, OMIM 609464); both are caused by mutations in the NOD2/CARD15 gene, with subsequent dysregulation of the inflammatory response and formation of noncaseous granulomas (Table 1) [117]. The gene discussed is NOD2; the disease is sarcoidosis.