Besides the classical A20 haploinsufficiency or BD phenotypes, additional atypical phenotypes have been reported in TNFAIP2 mutant patients, including diabetes, psoriasis, Crohn’s disease, IBD, systematic lupus erythematosus (SLE), lupus nephritis, and neurological dysfunction (Figure 5). This evidence concerns the gene TNFAIP3 and lupus nephritis.