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Review
. 2022 Apr 1;34(2):229-233.
doi: 10.1097/MOP.0000000000001106.

Polyarteritis nodosa

Yelda Bilginer  1 , Seza Ozen
Affiliations
Review

Polyarteritis nodosa

Yelda Bilginer et al. Curr Opin Pediatr. .

Abstract

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.

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References

    1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65:1–11.
    1. Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood TR. Incidence of Henoch-Schonlein purpura, Kawasaki disease, and rare vasculitides in children of different ethnic origins. Lancet 2002; 360:1197–1202.
    1. Ozen S, Ruperto N, Dillon MJ, et al. EULAR/PReS endorsed consensus criteria for the classification of childhood vasculitides. Ann Rheum Dis 2006; 65:936–941.
    1. Karadag O, Erden A, Bilginer Y, et al. A retrospective study comparing the phenotype and outcomes of patients with polyarteritis nodosa between UK and Turkish cohorts. Rheumatol Int 2018; 38:1833–1840.
    1. Tekgoz N, Aydin F, Kurt T, et al. Clinical features and outcomes of childhood polyarteritis nodosa: a single referral center experience. Mod Rheumatol 2021; 31:1142–1147.

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Supplementary concepts