Dysregulation of NOD/RIPK2 signaling is implicated in the development of several inflammatory diseases, including sarcoidosis, Blau syndrome, Crohn’s disease (CD), and ulcerative colitis (UC) (Hugot et al., 2001; Lesage et al., 2002; Hysi et al., 2005; McGovern et al., 2005; Tanabe et al., 2006; Stronati et al., 2008; Negroni et al., 2009; Stronati et al., 2010; Rose et al., 2011; Strober et al., 2014; Honjo et al., 2021). This evidence concerns the gene RIPK2 and Blau syndrome.