National Referral Center for Rare Systemic Autoimmune Diseases, Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Paris, France.
Rheumatology Unit, Geneva University Hospitals and University of Geneva, Geneva, Switzerland.
Rheumatology (Oxford). 2022 Mar 2;61(3):1211-1216. doi: 10.1093/rheumatology/keab495.
To describe disease presentation and long-term outcome of granulomatosis with polyangiitis (GPA) patients according to blood eosinophils count (Eos) at vasculitis diagnosis.
Data from newly diagnosed GPA patients registered in the French Vasculitis Study Group database with available eosinophil count at diagnosis were reviewed. Disease characteristics, rate and type of relapses, and overall survival were analysed according to Eos, categorized as normal (<500/mm3), mild-to-moderate hypereosinophilia (HE) (between 500 and 1500/mm3) and severe HE (>1500/mm3).
Three hundred and fifty-four patients were included. At diagnosis, 90 (25.4%) patients had HE ≥500/mm3; they were more likely male (73% vs 56%, P = 0.006) and had more frequent cutaneous manifestations (49% vs 33%, P = 0.01), peripheral neuropathy (32% vs 17%, P = 0.004) and higher BVAS (21 vs 18, P = 0.01), compared with those with Eos <500/mm3. Patients with severe HE (n = 28; median Eos 2355, range 1500-9114) had more frequent renal function worsening at presentation (P = 0.008). After a median follow-up of 3.95 (interquartile range 1.95-6.76) years, no difference was found in overall relapse rates according to baseline Eos, but those with HE experienced more neurological (P = 0.013) and skin (P = 0.024) relapses and had more frequently peripheral neuropathy as damage at last follow-up (P = 0.02). Overall survival was not significantly different in patients with normal Eos or HE at diagnosis. (P = 0.08).
Blood HE at diagnosis, observed in about one-quarter of GPA patients, identifies a subgroup of patients with a more severe disease and higher rate of skin and neurological involvement both at presentation and during follow-up.
根据血管炎诊断时的血嗜酸性粒细胞计数（Eos）描述肉芽肿性多血管炎（GPA）患者的疾病表现和长期预后。
回顾了法国血管炎研究组数据库中登记的新诊断 GPA 患者的数据，这些患者的诊断时可获得嗜酸性粒细胞计数。根据 Eos 将疾病特征、复发率和类型以及总体生存率进行分析，Eos 分为正常（<500/mm3）、轻度至中度嗜酸性粒细胞增多症（HE）（500 至 1500/mm3）和重度 HE（>1500/mm3）。
共纳入 354 例患者。诊断时，90 例（25.4%）患者的 HE ≥500/mm3；与 Eos<500/mm3 的患者相比，他们更可能为男性（73%比 56%，P=0.006），且更常出现皮肤表现（49%比 33%，P=0.01）、周围神经病（32%比 17%，P=0.004）和更高的 BVAS（21 比 18，P=0.01）。与 Eos<500/mm3 的患者相比，重度 HE（n=28；中位 Eos 2355，范围 1500-9114）患者在就诊时肾功能恶化更为常见（P=0.008）。在中位随访 3.95 年（四分位距 1.95-6.76）后，根据基线 Eos 并未发现总体复发率存在差异，但 HE 患者发生更多的神经（P=0.013）和皮肤（P=0.024）复发，且在最后一次随访时更常出现外周神经病作为损害（P=0.02）。在诊断时 Eos 正常或存在 HE 的患者中，总生存率无显著差异（P=0.08）。
在大约四分之一的 GPA 患者中观察到血 HE，这一指标可识别出疾病更严重、皮肤和神经受累发生率更高的亚组患者，无论是在就诊时还是在随访期间。
