The changing face of polyarteritis nodosa and necrotizing vasculitis
The changing face of polyarteritis nodosa and necrotizing vasculitis
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DOI：
10.1038/nrrheum.2017.68
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发表时间：
2017-06-01
影响因子：
33.7
通讯作者：
Ozen, Seza
中科院分区：
文献类型：
--
作者：
Ozen, Seza
结节性多动脉炎(PAN)是一种以坏死性血管炎为主要特征的血管性疾病--血管中的炎性损害导致血管壁坏死。我们对PAN和坏死性血管炎的理解是随着时间的推移而演变的。除了PAN，坏死性血管炎现在是一系列具有不同病因的疾病的公认特征。例如，与乙肝病毒感染相关的坏死性血管炎与PAN有不同的病因，现在被归类为一种单独的疾病。此外，尽管典型的PAN不是一种遗传性疾病，但特定基因的突变，如ADA2(也称为CECR1)，可导致类似于PAN的坏死性血管病变。文献还表明，PAN的病程在儿童期起病和局限于皮肤(所谓的皮肤病)的病例中有所不同。解剖PAN和其他具有PAN样特征的自体炎症性疾病可以实现更特异的治疗，也可能有助于我们更好地理解这些破坏性疾病的发病机制。
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.