Service de Médecine Interne, Centre Hospitalier Universitaire, Hôpital Gabriel-Montpied, 58 rue Montalembert, 63003, Clermont-Ferrand, France.
Service de Santé Publique, Centre Hospitalier Universitaire, Hôpital Gabriel-Montpied, Clermont-Ferrand, France.
Clin Rheumatol. 2020 May;39(5):1601-1608. doi: 10.1007/s10067-019-04816-7. Epub 2020 Jan 6.
To investigate the activity of relapsing events (RE) and their mode of presentation in patients with anti-neutrophil cytoplasmic (ANCA)-associated vasculitis (AAV).
Patients diagnosed with AAV between 1990 and 2015 experiencing at least one RE were investigated. The different organ involvements were registered during each RE. Presentation at initial onset (IO) and RE were compared. The Birmingham Vasculitis Activity Score was used to assess the activity.
Ninety-nine patients were followed: 54 patients with 96 RE and 45 patients with none. The rate of RE was 53% with a median time of follow-up of 6.8 years. The mean time to first RE was 2.8 years. Thirty patients experienced one single RE, 15 had 2, 5 had 3, 2 had 4, and 2 had, respectively, 7 and 8. Fifty-five percent of RE had the same features as IO. Compared to IO, some clinical manifestations were less present: constitutional symptoms (29% vs 69%), ear-nose-throat (50% vs 76%), lung involvement (59% vs 76%), peripheral neuropathies (14% vs 24%), arthritis (7% vs 27%), kidney (25% vs 41%), and heart (4% vs 20%) (p < 0.001). Skin, eye, and bowel manifestations were not significantly less involved during RE. The mean Birmingham Vasculitis Activity Score at IO was 9.02 and 5.11 at relapse (p < 0.0001). Among the 96 RE, 46% had a new organ involvement compared to IO: none were life-threatening.
Global activity of RE in AAV patients is lower than that of IO. Fewer organs are involved in relapses. RE turned out to begin with the same manifestations as IO in most cases.Key Points•First study looking into clinical characteristics of relapses including mostly granulomatosis with polyangiitis.•Around half of patients with AAV seemed to relapse in a similar way compared to the initial diagnosis.•The activity score during relapsing events is less important.
研究抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）患者复发事件（RE）的活动情况及其表现方式。
调查了 1990 年至 2015 年间诊断为 AAV 且至少经历过一次 RE 的患者。在每次 RE 期间，登记了不同的器官受累情况。比较初始发作（IO）和 RE 时的表现。使用伯明翰血管炎活动评分来评估活动度。
共随访 99 例患者：54 例患者有 96 次 RE，45 例患者无 RE。RE 发生率为 53%，中位随访时间为 6.8 年。首次 RE 的平均时间为 2.8 年。30 例患者仅经历了 1 次 RE，15 例患者经历了 2 次，5 例患者经历了 3 次，2 例患者经历了 4 次，2 例患者经历了 7 次和 8 次。55%的 RE 与 IO 具有相同的特征。与 IO 相比，一些临床表现较少出现：全身症状（29%对 69%）、耳鼻喉（50%对 76%）、肺部受累（59%对 76%）、周围神经病（14%对 24%）、关节炎（7%对 27%）、肾脏（25%对 41%）和心脏（4%对 20%）（p < 0.001）。皮肤、眼睛和肠道表现在 RE 期间并未明显减少。IO 时的平均伯明翰血管炎活动评分（Birmingham Vasculitis Activity Score）为 9.02，RE 时为 5.11（p < 0.0001）。在 96 次 RE 中，与 IO 相比，有 46%出现了新的器官受累：没有危及生命的情况。
AAV 患者的 RE 整体活动度低于 IO。复发时受累器官较少。RE 在大多数情况下似乎与 IO 以相同的表现开始。
首次研究观察了包括肉芽肿性多血管炎在内的复发的临床特征。
大约一半的 AAV 患者似乎以与初始诊断相似的方式复发。
复发时的活动评分并不重要。
