Abstract

Deficiency of adenosine deaminase 2 (DADA2) is a newly described entity of monogenic vasculitis with multisystem involvement and prominent neurological features. With this report, we are adding to the growing spectrum of cases of DADA2 with two adult cases of early-onset recurrent cerebrovascular events with multisystem involvement. These cases highlight the need for high suspicion of this diagnosis in adults presenting late with symptoms compatible with DADA2. We further report the futility and probable harm of antiplatelet agents in DADA2. Tumour necrosis factor inhibitors have been shown to be beneficial for the treatment and prevention of severe manifestations of this condition.