For instance, A20 haploinsufficiency is caused by mutations in the TNFAIP3, and is an auto-inflammatory disease mediated by the NFkB pathway with many clinical similarities to Behçet’s disease; however, it is typically juvenile onset with excess production of proinflammatory cytokines during flares [50, 51]. Here, TNFAIP3 is linked to Behcet disease.