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狼疮肾炎中蛋白酶 3-ANCA 阳性与髓过氧化物酶-ANCA 阳性的临床病理特征和结局差异。

Differences in clinico-pathological characteristics and outcomes between proteinase 3-ANCA positivity and myeloperoxidase-ANCA positivity in lupus nephritis.

机构信息

National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, Nanjing, China.

出版信息

Lupus. 2019 Aug;28(9):1111-1119. doi: 10.1177/0961203319861680. Epub 2019 Jul 10.

DOI:10.1177/0961203319861680
PMID:31291845
Abstract

BACKGROUND

Owing to the low prevalence of anti-neutrophil cytoplasmic antibodies (ANCA) in lupus nephritis (LN), there is no study about the differences between proteinase 3 (PR3)-ANCA positivity and myeloperoxidase (MPO)-ANCA positivity in LN until now.

METHODS

Here we perform a retrospective study to determine whether there are differences in clinic-pathological characteristics and renal outcomes between PR3-ANCA-positive LN patients and MPO-ANCA-positive LN patients.

RESULTS

A total of 26 (27.4%) PR3-ANCA-positive LN patients and 69 (72.6%) MPO-ANCA-positive LN patients ( < 0.001) were eligible for this study. Compared with PR3-ANCA-positive LN patients, MPO-ANCA-positive LN patients had significantly higher levels of serum creatinine (109.6 µmol/l vs. 74.3 µmol/l,  = 0.02), lower titers of antinuclear antibodies (ANA) (128 vs. 256,  = 0.01), and higher serum concentrations of C3 and C4 (0.54 g/l vs. 0.36 g/l,  = 0.002; 0.12 g/l vs. 0.06 g/l,  < 0.001; respectively). Furthermore, the MPO-ANCA-positive group had higher scores for chronicity index ( = 0.007), including interstitial fibrosis ( = 0.001) and tubular atrophy ( = 0.03) on biopsy specimens. The renal survival rates for MPO-ANCA-positive LN patients were 94.1% at 1 year, 83.2% at 5 years and 79.6% at 10 years; these values were worse when compared with those of the PR3-ANCA-positive group, which were 100%, 100% and 100%, respectively.

CONCLUSION

MPO-ANCA-positive LN patients had more severely impaired baseline renal function and less active lupus serology. More severely chronic pathological changes, including interstitial fibrosis and tubular atrophy on renal specimens, occurred in MPO-ANCA-positive LN patients. We found that MPO-ANCA-positive LN patients had worse renal outcomes.

摘要

背景

由于抗中性粒细胞胞质抗体(ANCA)在狼疮肾炎(LN)中的患病率较低,因此目前尚无关于蛋白酶 3(PR3)-ANCA 阳性与髓过氧化物酶(MPO)-ANCA 阳性在 LN 中的差异的研究。

方法

在这里,我们进行了一项回顾性研究,以确定 PR3-ANCA 阳性 LN 患者和 MPO-ANCA 阳性 LN 患者在临床病理特征和肾脏结局方面是否存在差异。

结果

共有 26 名(27.4%)PR3-ANCA 阳性 LN 患者和 69 名(72.6%)MPO-ANCA 阳性 LN 患者( < 0.001)符合本研究条件。与 PR3-ANCA 阳性 LN 患者相比,MPO-ANCA 阳性 LN 患者的血清肌酐水平明显更高(109.6 µmol/l 比 74.3 µmol/l, = 0.02),抗核抗体(ANA)滴度更低(128 比 256, = 0.01),血清 C3 和 C4 浓度更高(0.54 g/l 比 0.36 g/l,  = 0.002;0.12 g/l 比 0.06 g/l,  < 0.001;分别)。此外,MPO-ANCA 阳性组的慢性指数评分更高( = 0.007),包括活检标本中的间质纤维化( = 0.001)和肾小管萎缩( = 0.03)。MPO-ANCA 阳性 LN 患者的肾脏 1 年、5 年和 10 年的生存率分别为 94.1%、83.2%和 79.6%;与 PR3-ANCA 阳性组的 100%、100%和 100%相比,这些数值更差。

结论

MPO-ANCA 阳性 LN 患者的基线肾功能受损更严重,狼疮血清学更不活跃。MPO-ANCA 阳性 LN 患者的肾脏标本中更严重的慢性病理变化,包括间质纤维化和肾小管萎缩。我们发现 MPO-ANCA 阳性 LN 患者的肾脏结局更差。

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