Department of Rheumatology, University Medical Centre Ljubljana, Vodnikova cesta, 62 1000, Ljubljana, Slovenia.
Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
Curr Rheumatol Rep. 2021 Feb 10;23(3):14. doi: 10.1007/s11926-021-00983-2.
Polyarteritis nodosa is a rare disease characterized by the necrotizing inflammation of medium-sized arteries. Different etiopathogenetic and clinical variants of the disease have been recognized over the past decades. In the present paper, we review the clinical features, diagnosis, and treatment of the different subtypes of the disease.
The diagnosis of polyarteritis nodosa is primarily based on clinical findings, imaging, and histopathological investigations. Microbiological and genetic investigations complement the diagnostic work-up. Idiopathic and hereditary variants of polyarteritis nodosa are treated with immunomodulatory medications such as glucocorticoids, conventional immunomodulatory drugs (e.g., cyclophosphamide) and biologic agents (e.g., tumor necrosis factor inhibitors, interleukin 6 inhibitor), while hepatitis B virus-associated polyarteritis nodosa primarily requires antiviral therapy combined with plasma exchange. PAN is a disease with heterogeneous presentations, severity, and therapeutic approaches. The overall prognosis of this disease is improving, mainly due to early diagnosis and more effective treatments. Treatment choices are guided mainly by the disease subtype and severity. In this review, we have presented the current knowledge on PAN clinical variants, their classification, diagnosis, and treatment approaches.
结节性多动脉炎是一种罕见的疾病，其特征为中等大小动脉的坏死性炎症。在过去几十年中，已经认识到该病具有不同的病因发病机制和临床变异型。本文回顾了该病不同亚型的临床特征、诊断和治疗。
结节性多动脉炎的诊断主要基于临床发现、影像学和组织病理学研究。微生物学和遗传学研究补充了诊断工作。特发性和遗传性结节性多动脉炎采用免疫调节药物治疗，如糖皮质激素、传统免疫调节剂（如环磷酰胺）和生物制剂（如肿瘤坏死因子抑制剂、白细胞介素 6 抑制剂），而乙型肝炎病毒相关性结节性多动脉炎主要需要抗病毒治疗联合血浆置换。PAN 是一种具有异质性表现、严重程度和治疗方法的疾病。该病的总体预后正在改善，主要是由于早期诊断和更有效的治疗。治疗选择主要取决于疾病亚型和严重程度。本文介绍了目前关于 PAN 临床变异型、分类、诊断和治疗方法的知识。
