Department of Internal Medicine and Clinical Immunology, University Hospital Centre Dijon Bourgogne, Dijon, France
INSERM, CIC 1432, Clinical Epidemiology Unit, University of Burgundy, Dijon, France.
RMD Open. 2023 Mar;9(1). doi: 10.1136/rmdopen-2022-002953.
To develop a score assessing the probability of relapse in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).
Long-term follow-up data from GPA and MPA patients included in five consecutive randomised controlled trials were pooled. Patient characteristics at diagnosis were entered into a competing-risks model, with relapse as the event of interest and death the competing event. Univariate and multivariate analyses were computed to identify variables associated with relapse and build a score, which was then validated in an independent cohort of GPA or MPA patients.
Data collected from 427 patients (203 GPA, 224 MPA) at diagnosis were included. Mean±SD follow-up was 80.6±51.3 months; 207 (48.5%) patients experienced ≥1 relapse. Relapse risk was associated with proteinase 3 (PR3) positivity (HR=1.81 (95% CI 1.28 to 2.57); p<0.001), age ≤75 years (HR=1.89 (95% CI 1.15 to 3.13); p=0.012) and estimated glomerular filtration rate (eGFR) ≥30 mL/min/1.73 m² (HR=1.67 (95% CI 1.18 to 2.33); p=0.004) at diagnosis. A score, the French Vasculitis Study Group Relapse Score (FRS), from 0 to 3 points was modelised: 1 point each for PR3-antineutrophil cytoplasmic antibody positivity, eGFR ≥30 mL/min/1.73 m² and age ≤75 years. In the validation cohort of 209 patients, the 5-year relapse risk was 8% for a FRS of 0, 30% for 1, 48% for 2 and 76% for 3.
The FRS can be used at diagnosis to assess the relapse risk in patients with GPA or MPA. Its value for tailoring the duration of maintenance therapy should be evaluated in future prospective trials.
开发一种评估肉芽肿性多血管炎（GPA）和显微镜下多血管炎（MPA）复发概率的评分系统。
将纳入的五个连续随机对照试验中 GPA 和 MPA 患者的长期随访数据进行汇总。将患者诊断时的特征纳入竞争风险模型，以复发为事件，死亡为竞争事件。进行单变量和多变量分析，以确定与复发相关的变量，并构建评分系统，然后在 GPA 或 MPA 患者的独立队列中进行验证。
共纳入 427 例患者（203 例 GPA，224 例 MPA）的诊断数据。平均随访时间为 80.6±51.3 个月；207 例（48.5%）患者发生≥1 次复发。复发风险与蛋白酶 3（PR3）阳性（HR=1.81（95%CI 1.28 至 2.57）；p<0.001）、年龄≤75 岁（HR=1.89（95%CI 1.15 至 3.13）；p=0.012）和诊断时估算肾小球滤过率（eGFR）≥30 mL/min/1.73 m²（HR=1.67（95%CI 1.18 至 2.33）；p=0.004）相关。建立了一个 0 至 3 分的评分系统，即法国血管炎研究组复发评分（FRS）：PR3-抗中性粒细胞胞质抗体阳性、eGFR≥30 mL/min/1.73 m²和年龄≤75 岁各记 1 分。在 209 例验证队列患者中，FRS 为 0 时，5 年复发风险为 8%；FRS 为 1 时，复发风险为 30%；FRS 为 2 时，复发风险为 48%；FRS 为 3 时，复发风险为 76%。
FRS 可用于诊断时评估 GPA 或 MPA 患者的复发风险。其在未来前瞻性试验中评估维持治疗持续时间的价值有待进一步评估。
