Four distinct clinical phenotypes of vasculitis affecting medium-sized arteries.
Four distinct clinical phenotypes of vasculitis affecting medium-sized arteries.
复制标题
影响中等动脉的血管炎的四种不同的临床表型。
DOI：
10.1080/03009742.2018.1551965
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发表时间：
2019
影响因子：
2.1
通讯作者：
Harigae H.
中科院分区：
文献类型：
--
作者：
Shirai T;Shirota Y;Fujii H;Ishii T;Harigae H.
目的：在结节性多动脉炎（PAN）的范围内，皮肤PAN（cPAN）进一步分为轻度cPAN和重度cPAN，后者表现为溃疡、坏死或神经炎。由于很难区分严重的cPAN和系统性PAN，本研究评估了中型动脉坏死性动脉炎患者的临床特征。方法：本研究纳入了2008年至2017年在我院诊断为中型动脉坏死性动脉炎的41例患者。临床背景，实验室检查结果，治疗，复发率和死亡率evaluated.Results：36例患者被归类为有cPAN（轻度，15;溃疡，9;神经炎，8;两者，4），和5例表现为系统性血管炎。轻度cPAN的临床特征包括女性优势（84.6%）和年轻（中位数31岁）;全身性PAN的临床特征包括年龄较大（中位数71岁）和炎症标志物水平较高。重度cPAN表现为中间表型。用于治疗轻度cPAN、重度cPAN和全身性PAN的泼尼松龙中位剂量分别为20.0、40.0和40.0 mg/天。20.0%的轻度cPAN、90.5%的重度cPAN和80.0%的全身性PAN患者使用了免疫抑制剂。结论：轻度cPAN、重度cPAN（溃疡型）、重度cPAN（神经炎型）和全身性PAN的临床特点各有不同。特别是，重度cPAN（溃疡型）患者的复发率更高，表明联合治疗的重要性。
Objective: Within the spectrum of polyarteritis nodosa (PAN), cutaneous PAN (cPAN) is further classified into mild cPAN and severe cPAN which presents with ulcers, necrosis, or neuritis. As distinguishing between severe cPAN and systemic PAN can be difficult, this study evaluated the clinical characteristics of patients with necrotizing arteritis of medium-sized arteries.Methods: Forty-one patients diagnosed with necrotizing arteritis of medium-sized arteries between 2008 and 2017 at our institution were enrolled in this study. Clinical background, laboratory findings, treatments, and rates of relapse and death were evaluated.Results: Thirty-six patients were classified as having cPAN (mild, 15; ulcer, nine; neuritis, eight; both, four), and five cases manifested systemic vasculitis. Clinical characteristics of mild cPAN included female predominance (84.6%) and younger age (median 31 years); those of systemic PAN included older age (median 71 years) and higher levels of inflammatory markers. Severe cPAN manifested with intermediate phenotypes. The median doses of prednisolone used to treat mild cPAN, severe cPAN, and systemic PAN were 20.0, 40.0, and 40.0 mg/day, respectively. Immunosuppressants were used in 20.0% of mild cPAN, 90.5% of severe cPAN, and 80.0% of systemic PAN patients. Although the mortality rates were indistinguishable, the relapse rates of severe cPAN (ulcer type) were significantly higher than those of other types (88.9%).Conclusion: The clinical characteristics of mild cPAN, severe cPAN (ulcer type), severe cPAN (neuritis type), and systemic PAN were distinct from each other. In particular, patients with severe cPAN (ulcer type) had higher relapse rates, indicating the importance of combination therapy.