Li Cui, Zhou Min-Lin, Liang Dan-Dan, Wang Jing-Jing, Yang Jing, Zeng Cai-Hong, Liu Zhi-Hong, Zhang Hai-Tao
National Clinical Research Center of Kidney Diseases, Jinling Hospital, Nanjing University School of Medicine, Nanjing, Jiangsu, China.
BMJ Open. 2017 Jul 28;7(7):e015668. doi: 10.1136/bmjopen-2016-015668.
To assess the clinical features, pathological presentations, treatments and outcomes of lupus nephritis (LN) with anti-neutrophil cytoplasmic antibody (ANCA) positivity.
A case-control study.
Patients (n=49) were retrospectively included from Jinling Hospital in China if presenting with biopsy-proven ANCA-positive LN between 1985 and 2008. Clinicopathological characteristics and outcomes were analysed and compared with those of a control group (n=1279). We further compared treatment responses and outcomes of ANCA-positive LN patients based on the treatment issued.
The study included 40 women and 9 men (median age 33 years at biopsy): 38 with myeloperoxidase (MPO)-ANCA, 7 with proteinase 3 PR3)-ANCA and 4 with double positivity. ANCA-positive LN patients exhibited higher haematuria, serum creatinine levels and systemic lupus erythematosus disease activity index scores. On pathological evaluation, class IV LN was predominant, accounting for 61.22% of cases. Light microscopy revealed significantly higher activity index and chronicity index scores, including cellular crescents, interstitial inflammation, tubular atrophy and interstitial fibrosis. ANCA-positive LN patients receiving mycophenolate mofetil as induction therapy had a higher remission rate and better renal outcomes than those receiving cyclophosphamide. During follow-up, end-stage renal disease developed in seven (14.29%) ANCA-positive LN patients, all of them were MPO-ANCA positive.
The characteristics of ANCA-positive LN were massive haematuria and advanced renal insufficiency. We observed a higher remission rate and better prognoses when using mycophenolate mofetil than when using cyclophosphamide as induction therapy.
评估抗中性粒细胞胞浆抗体（ANCA）阳性的狼疮性肾炎（LN）的临床特征、病理表现、治疗方法及预后。
病例对照研究。
回顾性纳入1985年至2008年间在中国金陵医院就诊、经活检证实为ANCA阳性LN的患者（n = 49）。分析其临床病理特征及预后，并与对照组（n = 1279）进行比较。我们还根据所采用的治疗方法，比较了ANCA阳性LN患者的治疗反应及预后。
该研究纳入40名女性和9名男性（活检时中位年龄33岁）：38例为髓过氧化物酶（MPO）-ANCA阳性，7例为蛋白酶3（PR3）-ANCA阳性，4例为双阳性。ANCA阳性LN患者血尿、血清肌酐水平及系统性红斑狼疮疾病活动指数评分更高。病理评估显示，IV型LN为主，占病例的61.22%。光镜检查显示活动指数和慢性指数评分显著更高，包括细胞性新月体、间质炎症、肾小管萎缩和间质纤维化。接受霉酚酸酯作为诱导治疗的ANCA阳性LN患者缓解率更高，肾脏预后优于接受环磷酰胺治疗的患者。随访期间，7例（14.29%）ANCA阳性LN患者发展为终末期肾病，均为MPO-ANCA阳性。
ANCA阳性LN的特征为大量血尿和晚期肾功能不全。我们观察到，诱导治疗时使用霉酚酸酯比使用环磷酰胺缓解率更高，预后更好。
