The variant c.882‐2A>G (ADA2, NM_001282225.2) is reported in ClinVar and Varsome databases as pathogenic for polyarteritis nodosa or VAIHS and has been previously described to resemble ALPS (autoimmune lymphoproliferative syndrome) like phenotype by Alsutlan et al. 2018 (PMID 29271561).13 Here, ADA2 is linked to autoimmune lymphoproliferative syndrome.