Médecine Interne, Centre Hospitalier Universitaire Gabriel-Montpied, 63000 Clermont-Ferrand, France.
National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP). Centre, Université Paris Cité, Paris, France.
Semin Arthritis Rheum. 2024 Aug;67:152475. doi: 10.1016/j.semarthrit.2024.152475. Epub 2024 May 21.
Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) primarily affects small vessels. Large-vessel involvement (LVI) is rare. We aimed to describe the characteristics of LVI, to identify associated risk factors, and to describe its therapeutic management.
This multicenter case-control (1:2) study included patients with AAV according to the ACR/EULAR classification and LVI as defined by the Chapel Hill nomenclature, together with controls matched for age, sex, and AAV type.
We included 26 patients, 15 (58 %) of whom were men, with a mean age of 56.0 ± 17.1 years. The patients had granulomatosis with polyangiitis (n = 20), or microscopic polyangiitis (n = 6). The affected vessels included the aorta (n = 18; 69 %) supra-aortic trunks (n = 9; 35 %), lower-limb arteries (n = 5; 19 %), mesenteric arteries (n = 5; 19 %), renal arteries (n = 4; 15 %), and upper-limb arteries (n = 2; 8 %). Imaging showed wall thickening (n = 10; 38 %), perivascular inflammation (n = 8; 31 %), aneurysms (n = 5; 19 %), and stenosis (n = 4; 15 %). Comparisons with the control group revealed that LVI was significantly associated with neurological manifestations (OR=3.23 [95 % CI: 1.11-10.01, p = 0.03]), but not with cardiovascular risk factors (OR=0.70 [95 % CI: 0.23-2.21, p = 0.60]), or AAV relapse (OR=2.01 [95 % CI: 0.70-5.88, p = 0.16]). All patients received corticosteroids, in combination with an immunosuppressant in 24 (92 %), mostly cyclophosphamide (n = 10, 38 %) or rituximab (n = 9, 35 %).
Regardless of distinctions based on vessel size, clinicians should consider LVI as a potential manifestation of AAV, with the aorta commonly affected. The risk of developing LVI appears to be greater for clinical phenotypes of AAV with neurological involvement. Standard AAV treatment can be used to manage LVI.
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）主要影响小血管。大血管受累（LVI）较为罕见。本研究旨在描述 LVI 的特征，确定相关的危险因素，并描述其治疗管理。
这项多中心病例对照（1:2）研究纳入了符合 ACR/EULAR 分类的 AAV 患者和按照 Chapel Hill 命名法定义的 LVI 患者，并与年龄、性别和 AAV 类型相匹配的对照组进行了比较。
我们纳入了 26 例患者，其中 15 例（58%）为男性，平均年龄为 56.0±17.1 岁。患者的疾病包括肉芽肿性多血管炎（n=20）或显微镜下多血管炎（n=6）。受累的血管包括主动脉（n=18；69%）、主动脉干（n=9；35%）、下肢动脉（n=5；19%）、肠系膜动脉（n=5；19%）、肾动脉（n=4；15%）和上肢动脉（n=2；8%）。影像学检查显示壁增厚（n=10；38%）、血管周围炎症（n=8；31%）、动脉瘤（n=5；19%）和狭窄（n=4；15%）。与对照组比较发现，LVI 与神经系统表现显著相关（OR=3.23[95%CI：1.11-10.01，p=0.03]），但与心血管危险因素（OR=0.70[95%CI：0.23-2.21，p=0.60]）或 AAV 复发（OR=2.01[95%CI：0.70-5.88，p=0.16]）无关。所有患者均接受了皮质类固醇治疗，24 例（92%）患者联合使用了免疫抑制剂，主要为环磷酰胺（n=10，38%）或利妥昔单抗（n=9，35%）。
无论基于血管大小的区别如何，临床医生都应考虑 LVI 是 AAV 的一种潜在表现，其中主动脉最常受累。具有神经受累临床表型的 AAV 发生 LVI 的风险似乎更高。标准的 AAV 治疗可用于治疗 LVI。
