This particularly related to patients presenting for the first time with acute hyper-inflammation, and included various immunological diseases such as hemophagocytic lymphohistiocytosis (HLH) (6, 8, 9); and autoinflammatory diseases presenting with acute vasculitic features such as arterial stroke, digital gangrene, or other life and organ threatening vasculitic injury (e.g. deficiency of adenosine deaminase 2, DADA2; or STING associated vasculopathy with onset in infancy, SAVI, amongst several others). Here, ADA2 is linked to hemophagocytic syndrome.