血清学特征（抗中性粒细胞胞浆抗体类型）与抗中性粒细胞胞浆抗体相关血管炎肾受累临床特征之间的关系。
Relationship between serologic profile (ANCA type) and clinical features of renal involvement in ANCA-associated vasculitides.
作者信息
Bulanov N M, Makarov E A, Shchegoleva E M, Zykova A S, Vinogradova E S, Novikov P I, Lysenko Kozlovskaya L V, Moiseev S V
机构信息
I.M. Sechenov First Moscow State Medical University, Ministry of Health of Russia (Sechenov University), Moscow, Russia.
M.V. Lomonosov Moscow State University, Moscow, Russia.
出版信息
Ter Arkh. 2018 Jun 20;90(6):15-21. doi: 10.26442/terarkh201890615-21.
AIM
To compare the frequency, clinical features and outcomes of renal involvement in ANCA-associated vasculitides (AAV) in patients with antibodies against proteinase-3 (pr3-ANCA) and myeloperoxidase (MPO-ANCA).
MATERIALS AND METHODS
In our retrospective study we enrolled 264 patients, 94 males and 170 females, median age 53 [36; 62] years. Among them 157 were pr3-ANCA positive and 107 were MPO-ANCA positive. AAV was diagnosed according to ACR criteria and Chapel Hill consensus conference definition (2012). Median follow up was 44 [18; 93] months. We assessed baseline BVAS and VDI by the end of the follow up. Serum creatinine (sCr), estimated glomerular filtration rate (eGFR), hematuria and daily proteinuria were estimated. Diagnosis and stage of chronic kidney disease (CKD) and acute kidney injury (AKI) were established according to KDIGO guidelines (2012) and Scientific Society of Russian Nephrologists (2016).
RESULTS
Renal involvement was present in 181 (68.6%) patients, and its frequency was similar in pr3-ANCA and MPO-ANCA subgroups. Patients with MPO-ANCA developed rapidly progressive glomerulonephritis and hypertension significantly more often than patients with pr3-ANCA: 50.7% vs 35.6% (p=0.049) and 46.1% vs 29.8% (p=0.029) respectively. At disease onset, median sCr was significantly higher and eGFR was significantly lower in patients with MPO-ANCA (p<0.05). 1-year and 5-year renal survival rates were similar in pr3-ANCA-positive (93.9% and 87.4% respectively) and MPO-ANCA positive patients (87.4% and 83.1% respectively). Median BVAS and VDI scores were significantly higher in pr3-ANCA subgroup. The number of patients who developed AAV relapse during 1-year follow up was also significantly higher in pr3-ANCA subgroup. The frequency of eye and ENT involvement was significantly higher in pr3-ANCA positive patients than in MPO-ANCA-positive patients.
CONCLUSION
The frequency of extrarenal manifestations, clinical features of renal involvement and relapse rate are associated with AAV serotype.
目的
比较抗蛋白酶-3抗体（pr3-ANCA）和抗髓过氧化物酶抗体（MPO-ANCA）阳性的抗中性粒细胞胞浆抗体相关性血管炎（AAV）患者肾脏受累的频率、临床特征及预后。
材料与方法
在我们的回顾性研究中，纳入了264例患者，其中男性94例，女性170例，中位年龄53[36；62]岁。其中157例pr3-ANCA阳性，107例MPO-ANCA阳性。AAV根据美国风湿病学会（ACR）标准和2012年查珀尔希尔共识会议定义进行诊断。中位随访时间为44[18；93]个月。随访结束时评估基线BVAS和VDI。估算血清肌酐（sCr）、估算肾小球滤过率（eGFR）、血尿和每日蛋白尿。根据KDIGO指南（2012年）和俄罗斯肾脏病学家科学协会（2016年）确定慢性肾脏病（CKD）和急性肾损伤（AKI）的诊断及分期。
结果
181例（68.6%）患者存在肾脏受累，其在pr3-ANCA和MPO-ANCA亚组中的频率相似。MPO-ANCA阳性患者发生快速进展性肾小球肾炎和高血压的频率显著高于pr3-ANCA阳性患者：分别为50.7%对35.6%（p=0.049）和46.1%对29.8%（p=0.029）。在疾病发作时，MPO-ANCA阳性患者的中位sCr显著更高，eGFR显著更低（p<0.05）。pr3-ANCA阳性患者（分别为93.9%和87.4%）和MPO-ANCA阳性患者（分别为87.4%和83.1%）的1年和5年肾脏生存率相似。pr3-ANCA亚组的中位BVAS和VDI评分显著更高。在1年随访期间发生AAV复发的患者数量在pr3-ANCA亚组中也显著更高。pr3-ANCA阳性患者眼部和耳鼻喉受累的频率显著高于MPO-ANCA阳性患者。
结论
肾外表现的频率、肾脏受累的临床特征及复发率与AAV血清型相关。
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