Department of Internal Medicine, Caen University Hospital, Basse Normandie University, Caen, France.
National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Centre, Université Paris Cité, Paris, France.
Autoimmun Rev. 2023 May;22(5):103293. doi: 10.1016/j.autrev.2023.103293. Epub 2023 Feb 12.
ANCA-associated vasculitis (AAV) is an exceptional cause of small and large vascular aneurysms. Here, we present the phenotypic characteristics of patients with AAV associated with the presence of aneurysms.
We conducted a retrospective multicenter study and a systematic review of the literature. Only AAV patients with positive ANCA results and > 1 aneurysm(s) were enrolled. Patients were recruited through a call of observations among the French Vasculitis Study Group (FVSG) and the French Internal Medicine Network. Patients with aneurysm rupture were compared to those without.
We enrolled 51 patients in the cohort, including 31 (67%) with granulomatosis with polyangiitis. The median Birmingham Vasculitis Activity Score was 18 [6-41]. A total of 92 aneurysms were noted, 74% of which involved medium-sized arteries, particularly the renal artery. During a follow-up of 24 [6-56] months, 22 (43%) patients experienced aneurysmal rupture, 91% of which involved medium-sized vessels. Patients with aneurysmal rupture showed significantly more pulmonary infiltrates and higher creatinine levels at baseline than patients without rupture. Initial treatments did not differ between the two groups. Ten (20%) patients died during the follow-up, including three from an aneurysmal rupture.
Aneurysms were more frequently observed in GPA patients and predominantly affected medium-sized vessels, especially the renal arteries. The risk of rupture was high and occurred in >40% of patients. Because of their increased mortality, further studies are required to better manage this subset of patients.
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）是小血管和大血管动脉瘤的罕见病因。在此，我们介绍了伴有动脉瘤的 AAV 患者的表型特征。
我们进行了一项回顾性多中心研究和系统文献回顾。仅纳入了 ANCA 阳性结果且>1 个动脉瘤的 AAV 患者。患者是通过法国血管炎研究组（FVSG）和法国内科网络的观察性研究招募的。将有动脉瘤破裂的患者与无动脉瘤破裂的患者进行比较。
我们共纳入了 51 例患者，其中 31 例（67%）患有肉芽肿性多血管炎。Birmingham 血管炎活动评分中位数为 18 [6-41]。共发现 92 个动脉瘤，其中 74%涉及中等大小的动脉，特别是肾动脉。在 24 [6-56]个月的随访期间，22 例（43%）患者发生了动脉瘤破裂，其中 91%涉及中等大小的血管。与未破裂的患者相比，有动脉瘤破裂的患者在基线时肺部浸润和肌酐水平明显更高。两组初始治疗无差异。10 例（20%）患者在随访期间死亡，其中 3 例死于动脉瘤破裂。
GPA 患者中更常观察到动脉瘤，且主要影响中等大小的血管，特别是肾动脉。破裂的风险很高，超过 40%的患者发生破裂。由于死亡率增加，需要进一步研究以更好地管理这组患者。
