PT  - JOURNAL ARTICLE
AU  - Jha, Avanish
AU  - Doley, Daisy
AU  - Mathew, John
AU  - Hegde, Arvind G
TI  - Deficiency of adenosine deaminase 2 (DADA2): two cases of multisystem vasculitis managed in a South Indian tertiary care centre
AID  - 10.1136/bcr-2021-242963
DP  - 2021 Aug 01
TA  - BMJ Case Reports
PG  - e242963
VI  - 14
IP  - 8
4099  - http://casereports.bmj.com/content/14/8/e242963.short
4100  - http://casereports.bmj.com/content/14/8/e242963.full
SO  - BMJ Case Reports2021 Aug 01; 14
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.