In light of the data by now available, we consider the genetic analysis of CECR1 gene suggested in the following clinical pictures: patients with an inflammatory vasculopathy with early onset in infancy, patients with a diagnosis of PAN or cPAN with early onset and/or severe organ involvement (above all stroke), especially in case of a positive family history or consanguinity/endogamy in the parents. This evidence concerns the gene ADA2 and polyarteritis nodosa.