Rare loss-of-function mutations in TNFAIP3 cause the autosomal dominant IEI familial Behçet-like autoinflammatory syndrome-1, an autoinflammatory disorder characterised by systemic inflammation, mucosal ulceration, polyarthritis, uveitis, and recurrent infections in some [95,96]. Here, TNFAIP3 is linked to uveitis.