Istituto di Ricovero e Cura a Carattere Scientifico Policlinico S. Matteo Foundation, University of Pavia, Pavia, Italy.
Ospedale Civile Sant'Andrea, La Spezia, Italy.
Arthritis Care Res (Hoboken). 2023 May;75(5):1158-1165. doi: 10.1002/acr.24958. Epub 2022 Dec 21.
To assess the prevalence and impact on damage accrual of different levels of disease activity in patients with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).
Patients with GPA and MPA followed for ≥5 years in 2 different centers were included. Disease activity and damage were assessed using the Birmingham Vasculitis Activity Score (BVAS) and Vasculitis Damage Index (VDI), respectively. Three levels of remission were defined: complete remission (BVAS = 0, negative for antineutrophil cytoplasmic antibody [ANCA], off treatment), clinical remission off therapy (CROffT; BVAS = 0, positive for ANCA), and clinical remission on therapy (CROnT; BVAS = 0, negative or positive for ANCA, glucocorticoids ≤5 mg/day and/or immunosuppressant). A low disease activity state (LDAS) was defined as 0 < BVAS ≤3, low-dose glucocorticoids (≤7.5 mg/day), and/or immunosuppressant. Remission or LDAS were defined as prolonged when lasting ≥2 consecutive years.
A total of 167 patients were included: 128 (76.6%) with GPA, 39 (23.4%) with MPA, mean ± SD age 51.0 ± 16.7 years. During a 5-year follow-up, 10 patients (6.0%) achieved prolonged complete remission, 6 (3.6%) prolonged CROffT, 89 (53.3%) prolonged CROnT, 42 (25.1%) prolonged LDAS, and 20 (12.0%) never achieved LDAS. The VDI score at 5 years progressively worsened according to increasing levels of disease activity targets (complete remission, CROffT, CROnT, and LDAS). The mean ± SD 5-year VDI score was higher in patients not achieving prolonged remission compared to those who did (3.7 ± 2.0 versus 2.2 ± 1.9; P < 0.0001). By multivariate analysis, baseline ear, nose, and throat (P = 0.006), and lung involvement (P = 0.047) were negative predictors of prolonged remission.
More than 60% of patients with GPA/MPA achieved prolonged remission, which was associated with better long-term outcomes. In contrast, prolonged LDAS correlated with increased damage accrual and was not a sufficient treatment target.
评估肉芽肿性多血管炎（GPA）和显微镜下多血管炎（MPA）患者不同疾病活动水平的患病率及其对累积损害的影响。
纳入在 2 个不同中心接受至少 5 年随访的 GPA 和 MPA 患者。使用伯明翰血管炎活动评分（BVAS）和血管炎损伤指数（VDI）分别评估疾病活动度和损伤。定义了 3 种缓解水平：完全缓解（BVAS=0，抗中性粒细胞胞质抗体[ANCA]阴性，停药）、治疗后临床缓解（CROffT；BVAS=0，ANCA 阳性）和治疗中临床缓解（CROnT；BVAS=0，ANCA 阴性或阳性，糖皮质激素≤5mg/天和/或免疫抑制剂）。低疾病活动状态（LDAS）定义为 0<BVAS≤3、低剂量糖皮质激素（≤7.5mg/天）和/或免疫抑制剂。当持续≥2 年时，缓解或 LDAS 被定义为延长。
共纳入 167 例患者：128 例（76.6%）为 GPA，39 例（23.4%）为 MPA，平均年龄 51.0±16.7 岁。在 5 年的随访期间，10 例（6.0%）患者达到延长的完全缓解，6 例（3.6%）患者达到延长的 CROffT，89 例（53.3%）患者达到延长的 CROnT，42 例（25.1%）患者达到延长的 LDAS，20 例（12.0%）患者从未达到 LDAS。根据疾病活动目标（完全缓解、CROffT、CROnT 和 LDAS）的不同，5 年后 VDI 评分逐渐恶化。与达到延长缓解的患者相比，未达到延长缓解的患者 5 年平均 VDI 评分更高（3.7±2.0 与 2.2±1.9；P<0.0001）。多变量分析显示，基线耳部、鼻部和喉部（P=0.006）和肺部受累（P=0.047）是延长缓解的负预测因子。
超过 60%的 GPA/MPA 患者达到延长缓解，这与更好的长期预后相关。相比之下，延长 LDAS 与累积损害增加相关，且不是一个充分的治疗目标。
