ADORA1 and cryopyrin-associated periodic syndrome: Defibrotide, an adenosine receptor agonist with affinity to adenosine receptors A1 and A2 and secondary antithrombotic function, has been used as a proposed vascular cell modulator to target VEC injury and to reconstitute multiple VEC functions in a patient with intractable prothrombotic resistance to combined therapy, with effective remission of CAPS [11].