Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, APHP, Université Paris Descartes, Paris, France.
Division of Rheumatology, Mount Sinai Hospital, Toronto, Canada.
J Autoimmun. 2020 Aug;112:102467. doi: 10.1016/j.jaut.2020.102467. Epub 2020 Apr 25.
To describe characteristics and long-term outcomes of patients with microscopic polyangiitis (MPA), an antineutrophil cytoplasm antibody (ANCA)-associated small-vessel necrotizing vasculitis.
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.
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.
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.
描述抗中性粒细胞胞浆抗体（ANCA）相关性小血管坏死性血管炎——显微镜下多血管炎（MPA）患者的特征和长期结局。
回顾性分析了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。分析了诊断时的特征、治疗方法、复发和死亡情况，以确定预测死亡或复发的因素。
在 1966 年至 2017 年间，诊断出 378 名 MPA 患者（中位年龄 63.7 岁），平均随访 5.5 年。在诊断时，主要的临床表现包括肾受累（74%）、关节痛（45%）、皮肤（41%）、肺（40%）和单神经炎多发性（32%），较少出现肺泡出血（16%）、心肌病（5%）和严重胃肠道症状（4%）；平均血清肌酐为 217μmol/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 293 名可获得 ELISA 特异性的患者中，272 名（92.8%）识别髓过氧化物酶，13 名（4.4%）识别蛋白酶-3。在随访期间，131 名（34.7%）患者复发，78 名（20.6%）死亡，主要死于感染。相应的 5 年总生存率和无复发生存率分别为 84.2%和 60.4%。多变量分析保留了年龄>65 岁、肌酐>130μmol/L、严重胃肠道受累和单神经炎多发性作为死亡的独立危险因素。肾功能不全与较低的复发风险相关。
在这个全国性队列中，非肾脏表现和几个死亡或复发的危险因素很常见。虽然死亡率较低，主要是由于治疗相关的并发症，但复发仍然很常见，这表明 MPA 的管理可以进一步改善。
