Schematic representation of the NOD2 protein (NP_071445 Schematic representation of the NOD2 protein (NP_071445.1) with the characteristic three domains structure and localisation of NOD2 mutations described in autoinflammatory granulomatous diseases. Schematic representation of the NOD2 protein (NP_071445.1) with the characteristic three domains structure and localisation of NOD2 mutations described in autoinflammatory granulomatous diseases. Red squares highlight mutations associated with BS, whereas the grey colour identifies EOS-related mutations, on the basis of the Infevers database data (CARD, caspase recruitment domain’ LRR, leucine rich repeat; NACHT, central nucleotide-binding and oligomerisation domain; http://fmf.igh.cnrs.fr/ISSAID/infevers/index.php). Francesco Caso et al. RMD Open 2015;1:e000097 Copyright © BMJ Publishing Group & EULAR. All rights reserved.