Key result
Childhood polyarteritis nodosa requires differentiation from adenosine deaminase-2 deficiency, as treatment strategies differ, though large patient numbers for high-evidence treatment are lacking.
Distinguishing deficiency of ADA2 from childhood polyarteritis nodosa is crucial as treatment strategies differ.
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PURPOSE OF REVIEW: The aim is to review recent reports on childhood polyarteritis nodosa, including recent reports on treatment and outcome. Recently deficiency of adenosine deaminase-2 (ADA2), which may present as a polyarteritis nodosa-mimic, is becoming an important part of our practice. We also aim to highlight differences of childhood polyarteritis nodosa with deficiency of ADA2 as well as adult-onset disease. RECENT FINDINGS: The few recent childhood series confirm the systemic nature of this vasculitis with predominantly medium-vessel involvement. American College of Rheumatology Vasculitis foundation has suggested recommendations for the management of this vasculitis. Unfortunately, we lack large patient numbers to provide us high evidence for the treatment of these patients. However, for induction mycophenolate mofetil or shorter courses of cyclophosphamide can be considered.Deficiency of ADA2 is now in the differential diagnosis of polyarteritis nodosa patients presenting with a family history and/or stroke with hematological and/or immunological abnormalities. SUMMARY: We need collaborative work to define management and treatment strategies for childhood polyarteritis nodosa. Distinguishing deficiency of ADA2 is important because the treatment is different.
Childhood PAN series confirm systemic features distinct from ADA2 deficiency; leaves open pediatric-specific management strategies.
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Bilginer et al. (2022) conducted a review in Childhood polyarteritis nodosa. Treatment strategies was evaluated. Childhood polyarteritis nodosa requires differentiation from adenosine deaminase-2 deficiency, as treatment strategies differ, though large patient numbers for high-evidence treatment are lacking.
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