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The changing face of polyarteritis nodosa and necrotizing vasculitis

Nature Reviews Rheumatology volume 13, pages 381–386 (2017)Cite this article

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Abstract

Polyarteritis nodosa (PAN) is a vasculitic disease characterized primarily by necrotizing vasculitis — inflammatory lesions in blood vessels that lead to vessel wall necrosis. Our understanding of PAN and necrotizing vasculitis has evolved over time. In addition to PAN, necrotizing vasculitis is now a recognized feature of a broad range of diseases with different aetiopathogenesis. For example, necrotizing vasculitis associated with hepatitis B virus infection has a different aetiopathogeneis to PAN and is now classified as a separate disease. Additionally, although 'classic' PAN is not an inherited disease, mutations in specific genes, such as ADA2 (also known as CECR1), can result in a necrotizing vasculopathy similar to PAN. The literature also suggests that the course of PAN differs in childhood-onset disease and in cases confined to the skin (so-called cutaneous PAN). Dissecting PAN and other autoinflammatory diseases with PAN-like features has enabled more-specific therapies and might also help us better understand the pathogenesis of these devastating conditions.

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Figure 1: PAN and PAN-like diseases associated with necrotizing vasculitis.
Figure 2: Timeline: the evolution of our understanding of polyarteritis nodosa.

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  1. Department of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Sihhiye, 06100, Ankara, Turkey

    Seza Ozen

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Correspondence to Seza Ozen.

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S.O. declares that she has acted as a consultant for Novartis and R-Pharm, and has received honoraria from Sobi.

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Ozen, S. The changing face of polyarteritis nodosa and necrotizing vasculitis. Nat Rev Rheumatol 13, 381–386 (2017). https://doi.org/10.1038/nrrheum.2017.68

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