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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.