■很少描述在系膜区域具有IgA沉积的抗GBM疾病。抗GBM病合并系膜IgA沉积患者肾脏预后的影响因素未知。
■检索郑州大学第一附属医院2015-2023年的病理报告,共发现72例抗GBM病患者,25例合并系膜IgA沉积。我们研究了临床和病理特征,肾脏预后,抗GBM病合并系膜IgA沉积患者肾脏预后的影响因素。
■他们的年龄中位数是44岁,他们的年龄分布是单峰的。抗GBM病患者合并系膜IgA沉积的少尿或无尿比例明显低于经典抗GBM病患者(13.04vs.42.31%,p=0.030)。他们的24小时尿蛋白排泄量明显较高[中位数:3.25vs.1.12g/24h,四分位数间距(IQR):1.032〜3.945vs.0.63~1.79g/24h,p=0.020],初始诊断时血清肌酐(SCr)水平较低(中位数:456.0vs.825.5μmol/L,IQR:270.0~702.0vs.515.8~1231.2μmol/L,p=0.002),峰值SCr水平较低(中位数:601.0vs.907.2μmol/L,IQR:376.5~937.0vs.607.0~1361.2μmol/L,p=0.007),他们的血清补体3(C3)水平更高(中位数:1.275vs.1.015g/L,IQR:1.097~1.462vs.0.850~1.220g/L,p=0.027)。随访期间他们的肾脏结局更好(p<0.001)。调整高血压后,少尿或无尿,和新月%,在抗GBM患者中,肾小球系膜区域的IgA沉积仍然是ESRD的独立保护因素(p=0.003)。初次诊断时的高血压(p=0.026)和SCr水平(p=0.004)是抗GBM疾病合并系膜IgA沉积患者肾脏预后的危险因素。
■与经典抗GBM疾病患者相比,抗GBM疾病合并系膜IgA沉积的患者肾脏损害较轻,肾脏预后较好。
UNASSIGNED: Anti-GBM diseases with IgA deposition in the mesangial region are rarely described.The factors influencing renal prognosis in patients with anti-GBM disease combined with mesangial IgA deposition are unknown.
UNASSIGNED: We searched the pathological reports of the First Affiliated Hospital of Zhengzhou University from 2015 to 2023 and found that a total of 72 patients with the anti-GBM disease and 25 patients combined with mesangial IgA deposition. We studied the clinical and pathological features, renal prognosis, and the factors affecting renal prognosis in patients with anti-GBM disease combined with mesangial IgA deposition.
UNASSIGNED: Their median age was 44 years, and their age distribution was unimodal. The proportion of oliguria or anuria in patients with anti-GBM disease combined with mesangial IgA deposition was significantly lower than that in patients with classic anti-GBM disease (13.04 vs. 42.31%, p=0.030). Their 24-hour urinary protein excretion was significantly higher [median:3.25 vs. 1.12g/24h, Interquartile range(IQR):1.032~3.945 vs. 0.63~1.79g/24h, p=0.020], serum creatinine (SCr) level at the initial diagnosis was lower(median:456.0 vs. 825.5μmol/L, IQR:270.0~702.0 vs. 515.8~1231.2μmol/L, p=0.002), peak SCr level was lower (median: 601.0 vs. 907.2μmol/L, IQR: 376.5~937.0 vs. 607.0~1361.2μmol/L, p=0.007), and their serum complement 3(C3) level was higher(median: 1.275 vs. 1.015g/L, IQR:1.097~1.462 vs. 0.850~1.220g/L, p=0.027). They had better renal outcomes during follow-up (p<0.001). After adjustment for hypertension, oliguria or anuria, and crescents%, IgA deposition in the mesangial region was still an independent protective factor (p=0.003) for ESRD in anti-GBM patients. Hypertension (p=0.026) and SCr levels at initial diagnosis (p=0.004) were risk factors for renal prognosis in patients with anti-GBM disease combined with mesangial IgA deposition.
UNASSIGNED: Patients with anti-GBM disease combined with mesangial IgA deposition have less severe renal impairment and better renal prognosis than patients with classic anti-GBM disease.