BETHESDA, MD—Rheumatologists from the National Institutes of Health (NIH) and from Jerusalem, Israel, have identified deficiency of adenosine deaminase 2 (DADA2) as an important cause of familial polyarteritis nodosa, which tends to present in childhood and can manifest with hematologic, immunologic and inflammatory signs, says Chip Chambers, MD, founder and president of the DADA2 Foundation….
Jason Springer, MD, MS, served as one of the lead authors of the ACR/VF guidelines for polyarteritis nodosa (PAN), and talks about the recommendations specific to PAN here.
Since it was first described, the spectrum of disease caused by deficiency of adenosine deaminase 2 (DADA2) has been broadening. Features described include systemic vascular and inflammatory features and recurrent stroke, which overlap with childhood-onset polyarteritis nodosa (PAN). Previous data show that DADA2 has extensive genotypic and phenotypic variation.1 Results from a recently published study…
Two new studies that examine the effects of a genetic defect in adenosine deaminase 2 may advance a gene-based definition of vasculopathy