RT Journal Article
SR Electronic
T1 Deficiency of adenosine deaminase 2 (DADA2): two cases of multisystem vasculitis managed in a South Indian tertiary care centre
JF BMJ Case Reports
JO BMJ Case Reports
FD BMJ Publishing Group Ltd
SP e242963
DO 10.1136/bcr-2021-242963
VO 14
IS 8
A1 Avanish Jha
A1 Daisy Doley
A1 John Mathew
A1 Arvind G Hegde
YR 2021
UL http://casereports.bmj.com/content/14/8/e242963.abstract
AB 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.