Nevertheless, in addition to HRFs, the concept of autoinflammation has now been extended to a number of clinical entities encompassing more recently identified Mendelian diseases such as deficiency of adenosine deaminase-2 (DADA-2) and monogenic interferonopathies; as well as diseases with a polygenic form of inheritance (such as Behçet’s and Still’s disease) (2). The gene discussed is ADA2; the disease is hyperinsulinemic hypoglycemia, familial, 4.