IgA nephropathy with anti-neutrophil cytoplasmic antibody seropositivity
IgA nephropathy with anti-neutrophil cytoplasmic antibody seropositivity
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DOI：
10.5414/cn108571
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发表时间：
2015-09-01
影响因子：
1.1
通讯作者：
Zhang, Jingbo
中科院分区：
文献类型：
--
作者：
Huang, Xin;Wang, Yuan;Zhang, Jingbo
背景：伊加肾病（IgAN）伴抗中性粒细胞胞浆抗体（ANCA）相关性肾小球肾炎的报道较少。方法：报告14例IgAN和ANCA血清阳性患者的临床和病理资料。结果：这些回顾性病例包括4名男性和10名女性，平均年龄为44.4 ± 12.7岁。ANCA阳性记录EUROBlot试剂盒和间接免疫荧光在所有患者。EUROBlot试剂盒检测14例阳性，其中MPO-ANCA 12例，PR 3-ANCA 2例。间接免疫荧光阳性14例（P-ANCA 12例，C-ANCA 2例）。14例ANCA阳性IgAN患者中有3例表现出严重的临床表现，新月体平均累及56%的肾小球，包括大量蛋白尿（平均24小时尿蛋白：3.8 g/d）、血尿和急性肾功能衰竭（平均肌酐：4.5 +/- 3.7 mg/dL）。其余11例无新月体的患者显示不同程度的蛋白尿（平均24小时尿蛋白：2.4 ± 2.4 g/d）、血尿和血清肌酐水平（肌酐中位数：0.9（IQR，0.5 - 1.4）mg/dL）。10例患者的随访期平均为14. 0 +/-11. 2个月。在接受类固醇和环磷酰胺治疗的3例新月体患者中，1例患者在活检时成为透析依赖性，并在治疗后继续透析，另1例患者死于急性心力衰竭，最后1例患者在治疗后肾功能改善，并在肾活检后26个月未发生终末期肾病（ESRD）。其余7例无新月体的患者接受类固醇、环磷酰胺、肾素-血管紧张素系统抑制剂和/或中药治疗; 6例肾功能稳定或改善，1例进展为ESRD伴肾功能恶化。结论：这些发现提示并非所有ANCA都参与IgAN的病理过程。在IgAN和ANCA患者中，建议鉴别致病性ANCA与非致病性ANCA。
Background: There are few reports of IgA nephropathy (IgAN) with anti-neutrophil cytoplasmic antibody (ANCA)associated glomerulonephritis. Methods: The authors report the clinical and pathological findings in 14 patients with IgAN and ANCA seropositivity. Results: These retrospective cases consisted of 4 men and 10 women with a mean age of 44.4 +/- 12.7 years. ANCA-positivity was documented by EUROBlot kits and indirect immunofluorescence in all patients. The results of EUROBlot kits were positive in 14 patients (12 MPO-ANCA, 2 PR3-ANCA). Indirect immunofluorescence was positive in 14 patients (12 P-ANCA, 2 C-ANCA). Three of 14 IgAN with ANCA-positive patients showed severe clinical manifestations with crescents involving a mean of 56% glomeruli, including heavy proteinuria (mean 24-hour urine protein: 3.8 g/d), hematuria and acute renal failure (mean creatinine: 4.5 +/- 3.7 mg/dL). The remaining 11 patients with no crescents showed various degrees of proteinuria (mean 24-hour urine protein: 2.4 +/- 2.4 g/d), hematuria and serum creatinine levels (median creatinine: 0.9 (IQR, 0.5 - 1.4) mg/dL). The follow- up period for 10 patients had an average length of 14.0 +/- 11.2 months. Among the three patients with crescents who had been treated with steroids and cyclophosphamide, one patient became dialysis dependent at the time of biopsy and remained on dialysis after treatment, another died of acute heart failure, and the last one showed improvement in renal function after treatment and did not develop end-stage renal disease (ESRD) 26 months after renal biopsy. The remaining 7 patients with no crescents were treated with steroids, cyclophosphamide, renin-angiotensin system inhibitors, and/or Traditional Chinese Medicine; 6 had stabilized or improved renal function and one progressed to ESRD with worsening renal function. Conclusions: These findings suggest not all ANCAs are involved in the pathology of IgAN. In patients with IgAN and ANCAs, identification of pathogenic vs. non-pathogenic ANCAs is recommended.