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目的·探讨IgA肾病(IgAN)患者肾组织H因子(CFH)沉积的临床病理特征及预后的相关性。方法·纳入上海交通大学医学院附属瑞金医院肾脏科肾脏穿刺确诊随访1年以上的原发性IgAN患者。采用免疫组织化学检测患者肾组织CFH沉积,酶联免疫吸附方法检测血清CFH水平。记录患者基线临床、病理资料及预后情况。研究终点定义为eGFR下降30%或eGFR<15mL/(min·1.73 m~2)或接受肾脏替代治疗。结果·共入选283例IgAN患者,肾脏病理检査示198例(70%)患者伴有CFH沉积。CFH沉积阳性的患者尿蛋白排泄增多、血尿酸增高,肾脏病理示系膜细胞增生、节段硬化及肾小管萎缩/间质纤维化更严重。肾组织CFH沉积与血清CFH之间无显著相关性。CFH沉积阳性患者的疾病无进展时间较阴性患者明显缩短[(59.90±1.87)个月vs(65.10±1.78)个月,P=0.01]。多元COX回归分析在校正基线eGFR、收缩压、白蛋白和血红蛋白后,CFH沉积仍为IgAN疾病进展的独立危险因素(HR=2.54,95%:CI1.04~6.17)。结论·CFH沉积阳性患者临床病理表现较沉积阴性患者严重、预后差,提示补体旁路局部活化而非全身活化可加重IgAN进展。
Objective To investigate the clinicopathological features and prognosis of H-factor (CFH) deposition in renal tissue of patients with IgA nephropathy (IgAN). Methods · Primary IgAN patients were included in the Department of Nephrology, Ruijin Hospital affiliated to Shanghai Jiaotong University School of Medicine for more than one year after they were followed up for more than one year. The renal tissue CFH deposition was detected by immunohistochemistry and the level of serum CFH was detected by enzyme-linked immunosorbent assay. The patients’ baseline clinical, pathological data and prognosis were recorded. The study endpoint was defined as a 30% reduction in eGFR or eGFR <15 mL / (min · 1.73 m 2) or renal replacement therapy. Results · A total of 283 patients with IgAN were enrolled. Nephrology showed 198 patients (70%) with CFH deposition. Patients with positive CFH deposition had increased urinary protein excretion, increased serum uric acid, increased renal pathology, mesangial cell hyperplasia, segmental sclerosis and tubular atrophy / interstitial fibrosis. There was no significant correlation between renal CFH deposition and serum CFH. Patients with positive CFH deposition had significantly shorter progression-free disease progression than those with negative disease (59.90 ± 1.87 months vs 65.10 ± 1.78 months, P = 0.01). Multivariate COX regression analysis CFH deposition remained an independent risk factor for progression of IgAN (HR 2.54, 95% CI 1.04 to 6.17) after baseline eGFR, systolic blood pressure, albumin, and hemoglobin were corrected. Conclusions · The patients with positive CFH deposition are more severe than those with negative deposition, the prognosis is poor, suggesting that local activation of complement bypass rather than systemic activation may aggravate IgAN progression.