Treatment of Polyarteritis Nodosa in Deficiency of Adenosine Deaminase 2 (DADA2)
Polyarteritis nodosa presenting in the setting of deficiency of adenosine deaminase 2 (DADA2) is a distinct, genetically driven entity. Identifying this underlying cause changes both the diagnostic workup and the choice of treatment — standard PAN management may be insufficient or inappropriate.
Clinical scenario
DADA2 presents with a PAN-like vasculitis, often beginning in childhood. Characteristic features include recurrent strokes and skin changes. The diagnosis is established through ADA2 enzyme activity assays or ADA2 gene sequencing. ADA2 mutations have been identified in patients who were initially classified as having systemic PAN, making DADA2 an important consideration when PAN has atypical features or an early onset.
Treatment approach (first-line)
Patients with PAN-like vasculitis attributable to DADA2 are managed with a specific class of targeted biologic therapy rather than conventional immunosuppression alone.
The complete regimen — including agent selection, initiation criteria, and follow-up guidance — is detailed in the full structured protocol.
References
DADA2 was first described in a series of patients with an early-onset (often childhood) PAN-like vasculitis.
DADA2 is characterized by recurrent strokes and skin changes and diagnosed using ADA2 sequencing or ADA2 functional assays, and ADA2 mutations have been identified in patients diagnosed as having systemic PAN.
For patients with clinical manifestations of deficiency of adenosine deaminase 2 (DADA2), we strongly recommend treatment with tumor necrosis factor inhibitors over glucocorticoids alone.
DOI: 10.1002/art.41776
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