近几十年来，多动脉炎（PAN）的病情发生了重大变化。关于现代时期系统性PAN的原因、特征和预后的最新数据缺乏。本回顾性研究纳入了2005年至2019年期间被法国血管炎研究组转诊的系统性PAN患者。收集了病情特征、相关疾病及预后数据，并对复发和死亡的预测因素进行了分析。共纳入196名患者。主要临床症状为全身状况（84%）、神经系统（59%）、皮肤（58%）和肌肉骨骼（58%）表现。继发性PAN占55例（28%），其中包括骨髓增生异常综合征（9%）、实体肿瘤（7%）、淋巴瘤（4%）和自身炎症性疾病（4%）。没有患者活动性乙型肝炎病毒（HBV）感染。所有接受治疗的患者（98.5%）使用糖皮质激素（GCs），单独使用（41%）或与免疫抑制剂联合使用（59%），达到缓解的比例为90%。复发与年龄>65岁（HR 1.85；95% CI 1.12-3.08）、肠道受累（1.95；95% CI 1.09-3.52）和皮肤坏死性损害（HR 1.95；95% CI 1.24-3.05）独立相关。1年、5年和10年的总生存率分别为93%、87%和81%。多变量分析显示，年龄>65岁（HR 2.80；95% CI 1.23-6.37）、坏死性紫癜（HR 4.16；95% CI 1.62-10.70）、急性肾损伤（HR 4.89；95% CI 1.71-13.99）和继发性PAN（HR 2.98；95% CI 1.29-6.85）与死亡独立相关。PAN的病情发生了变化，HBV-PAN已经消失，继发性PAN逐渐出现。复发率仍然较高，尤其在年长的患者中伴有胃肠道和皮肤坏死病变，也存在较高的死亡率。 版权所有 © 2023。Elsevier Ltd出版。

The landscape of polyarteritis nodosa (PAN) has substantially changed during the last decades. Recent data regarding causes, characteristics, and prognosis of systemic PAN in the modern era are lacking.This retrospective study included patients with systemic PAN referred to the French Vasculitis Study Group between 2005 and 2019. Characteristics, associated conditions and outcomes were collected, and predictors of relapse and death were analyzed.196 patients were included. Main clinical symptoms were constitutional (84%), neurological (59%), skin (58%) and musculoskeletal (58%) manifestations. Secondary PAN accounted for 55 (28%) patients, including myelodysplastic syndrome (9%), solid cancer (7%), lymphoma (4%) and autoinflammatory diseases (4%). No patient had active HBV infection. All treated patients (98.5%) received glucocorticoids (GCs), alone (41%) or in combination with immunosuppressants (59%), with remission achieved in 90%. Relapses were independently associated with age >65 years (HR 1.85; 95% CI1.12-3.08), gastrointestinal involvement (1.95; 95% CI1.09-3.52) and skin necrotic lesions (HR 1.95; 95%CI 1.24-3.05). One-, 5- and 10-year overall survival rates were 93%, 87% and 81%, respectively. In multivariate analyses, age >65 years (HR 2.80; 95%CI 1.23-6.37), necrotic purpura (HR 4.16; 95%CI 1.62-10.70), acute kidney injury (HR 4.89; 95% 1.71-13.99) and secondary PAN (HR 2.98; 95%CI 1.29-6.85) were independently associated with mortality.Landscape of PAN has changed during the last decades, with the disappearance of HBV-PAN and the emergence of secondary PAN. Relapse rate remains high, especially in aged patients with gastrointestinal and cutaneous necrosis, as well as mortality.Copyright © 2023. Published by Elsevier Ltd.