Objective

To describe characteristics and long-term outcomes of patients with microscopic polyangiitis (MPA), an antineutrophil cytoplasm antibody (ANCA)-associated small-vessel necrotizing vasculitis.

Methods

MPA patients from the French Vasculitis Study Group Registry satisfying the European Medicines Agency algorithm were analyzed retrospectively. Characteristics at diagnosis, treatments, relapses and deaths were analyzed to identify factors predictive of death or relapse.

Results

Between 1966 and 2017, 378 MPA patients (median age 63.7 years) were diagnosed and followed for a mean of 5.5 years. At diagnosis, the main clinical manifestations included renal involvement (74%), arthralgias (45%), skin (41%), lung (40%) and mononeuritis multiplex (32%), with less frequent alveolar hemorrhage (16%), cardiomyopathy (5%) and severe gastrointestinal signs (4%); mean serum creatinine was 217 μmol/L. ANCA were detected in 298/347 (86%) patients by immunofluorescence and/or enzyme-linked immunosorbent assay (ELISA). Among the 293 patients with available ELISA specificities, 272 (92.8%) recognized myeloperoxidase and 13 (4.4%) proteinase-3. During follow-up, 131 (34.7%) patients relapsed and 78 (20.6%) died, mainly from infections. Respective 5-year overall and relapse-free survival rates were 84.2% and 60.4%. Multivariable analyses retained age >65 years, creatinine >130 μmol/L, severe gastrointestinal involvement and mononeuritis multiplex as independent risk factors for death. Renal impairment was associated with a lower risk of relapse.

Conclusion

Non-renal manifestations and several risk factors for death or relapse were frequent in this nationwide cohort. While mortality was low, and mainly due to treatment-related complications, relapses remained frequent, suggesting that MPA management can be further improved.

中文翻译：

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显微镜下多血管炎：来自法国血管炎研究组登记处的 378 名患者的临床特征和长期结果。

客观的

描述显微镜下多血管炎 (MPA)、抗中性粒细胞胞浆抗体 (ANCA) 相关小血管坏死性血管炎患者的特征和长期结果。

方法

对满足欧洲药品管理局算法的法国血管炎研究组登记处的 MPA 患者进行回顾性分析。分析诊断、治疗、复发和死亡时的特征以确定预测死亡或复发的因素。

结果

1966 年至 2017 年间，378 名 MPA 患者（中位年龄 63.7 岁）被诊断出并平均随访 5.5 年。诊断时，主要临床表现包括肾脏受累（74%）、关节痛（45%）、皮肤（41%）、肺（40%）和多发性单神经炎（32%），以及较少见的肺泡出血（16%），心肌病 (5%) 和严重的胃肠道症状 (4%); 平均血清肌酐为 217 μmol/L。通过免疫荧光和/或酶联免疫吸附试验 (ELISA) 在 298/347 (86%) 名患者中检测到 ANCA。在具有可用 ELISA 特异性的 293 名患者中，272 名 (92.8%) 识别髓过氧化物酶，13 名 (4.4%) 蛋白酶 3。随访期间，131 名（34.7%）患者复发，78 名（20.6%）患者死亡，主要死于感染。5 年总生存率和无复发生存率分别为 84.2% 和 60.4%。多变量分析将年龄>65 岁、肌酐>130 μmol/L、严重胃肠道受累和多发性单神经炎作为死亡的独立危险因素。肾功能损害与较低的复发风险相关。

结论

在这个全国性队列中，非肾脏表现和死亡或复发的几个危险因素很常见。虽然死亡率低，主要是由于治疗相关并发症，但复发仍然频繁，表明 MPA 管理可以进一步改善。