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Abstract：
IgA vasculitis nephritis (IgAVN) and IgA nephropathy (IgAN) share several clinical and pathological characteristics, though distinctions also exist. Their interrelation, however, remains undefined. This study investigates the clinicopathological divergences and prognostic disparities in pediatric patients with IgAVN and IgAN.
Our study encompasses 809 pediatric patients with IgAVN and 236 with IgAN, all of whom underwent kidney biopsy. We utilized the Semiquantitative Classification (SQC) scoring system to juxtapose the pathologies of the two conditions, and performed a COX regression analysis to examine factors influencing their prognoses.
Both patient groups demonstrated a predominance of males. A seasonality was observed, with a higher incidence of IgAN in the summer, and IgAVN in the fall (P < 0.0001). Patients with IgAN exhibited more severe tubulointerstitial injury, higher chronicity index, and total biopsy scores compared to those with IgAVN (P < 0.0001). Mesangial deposition intensity of complement C3, and the rate of pure IgA deposition, were found to be greater in patients with IgAVN compared to those with IgAN (P < 0.0001). The intensity of IgA deposition was also significantly higher in IgAVN patients (P = 0.003). IgAVN demonstrated a superior prognosis, with a higher rate of kidney remission (P < 0.0001). COX regression analysis indicated that interstitial fibrosis, as identified in the SQC pathology system, was associated with the prognosis of both conditions. Furthermore, the findings suggest that IgA deposition levels (IgA + + and IgA + + +) could potentially influence the prognosis of IgAVN.
Compared to IgAVN, IgAN manifests more severely with regard to renal impairment, interstitial damage, and prognosis. The disparities in immune complex deposition levels and locations within the kidneys support the hypothesis of IgAVN and IgAN as distinct diseases. Interstitial fibrosis may serve as a key pathological indicator within the SQC system associated with kidney prognosis in children with IgAVN and IgAN. The degree of IgA deposition could also be linked with the prognosis of IgAVN.
Our study encompasses 809 pediatric patients with IgAVN and 236 with IgAN, all of whom underwent kidney biopsy. We utilized the Semiquantitative Classification (SQC) scoring system to juxtapose the pathologies of the two conditions, and performed a COX regression analysis to examine factors influencing their prognoses.
Both patient groups demonstrated a predominance of males. A seasonality was observed, with a higher incidence of IgAN in the summer, and IgAVN in the fall (P < 0.0001). Patients with IgAN exhibited more severe tubulointerstitial injury, higher chronicity index, and total biopsy scores compared to those with IgAVN (P < 0.0001). Mesangial deposition intensity of complement C3, and the rate of pure IgA deposition, were found to be greater in patients with IgAVN compared to those with IgAN (P < 0.0001). The intensity of IgA deposition was also significantly higher in IgAVN patients (P = 0.003). IgAVN demonstrated a superior prognosis, with a higher rate of kidney remission (P < 0.0001). COX regression analysis indicated that interstitial fibrosis, as identified in the SQC pathology system, was associated with the prognosis of both conditions. Furthermore, the findings suggest that IgA deposition levels (IgA + + and IgA + + +) could potentially influence the prognosis of IgAVN.
Compared to IgAVN, IgAN manifests more severely with regard to renal impairment, interstitial damage, and prognosis. The disparities in immune complex deposition levels and locations within the kidneys support the hypothesis of IgAVN and IgAN as distinct diseases. Interstitial fibrosis may serve as a key pathological indicator within the SQC system associated with kidney prognosis in children with IgAVN and IgAN. The degree of IgA deposition could also be linked with the prognosis of IgAVN.
摘要：
背景：IgA血管炎肾炎（IgAVN）和IgA肾病（IgAN）共有几个临床和病理特征，虽然区别也存在。它们的相互关系,然而,仍然未定义。这项研究调查了小儿IgAVN和IgAN患者的临床病理差异和预后差异。
方法：我们的研究包括809例IgAVN患儿和236例IgAN患儿，所有这些人都接受了肾活检。我们利用半定量分类（SQC）评分系统将两种情况的病理并列，并进行了COX回归分析，以检查影响其预后的因素。
结果：两组患者均以男性为主。观察到季节性，夏季IgAN的发病率较高，和IgAVN在秋季（P<0.0001）。IgAN患者表现出更严重的肾小管间质损伤，较高的慢性指数，和总活检评分与IgAVN相比（P<0.0001）。补体C3的系膜沉积强度和纯IgA沉积率，与IgAN患者相比，IgAVN患者的比例更高（P<0.0001）。IgAVN患者的IgA沉积强度也明显更高(P=0.003)。IgAVN显示出优越的预后，肾脏缓解率较高(P<0.0001)。COX回归分析显示间质纤维化,如在SQC病理学系统中确定的，与这两种情况的预后有关。此外，研究结果表明,IgA沉积水平(IgA++和IgA+++)可能潜在地影响IgAVN的预后.
结论：与IgAVN相比，IgAN在肾功能损害方面表现更严重，间隙损伤,和预后。肾脏内免疫复合物沉积水平和位置的差异支持IgAVN和IgAN作为不同疾病的假设。间质纤维化可能是与IgAVN和IgAN患儿肾脏预后相关的SQC系统内的关键病理指标。IgA沉积的程度也可能与IgAVN的预后有关。
方法：我们的研究包括809例IgAVN患儿和236例IgAN患儿，所有这些人都接受了肾活检。我们利用半定量分类（SQC）评分系统将两种情况的病理并列，并进行了COX回归分析，以检查影响其预后的因素。
结果：两组患者均以男性为主。观察到季节性，夏季IgAN的发病率较高，和IgAVN在秋季（P<0.0001）。IgAN患者表现出更严重的肾小管间质损伤，较高的慢性指数，和总活检评分与IgAVN相比（P<0.0001）。补体C3的系膜沉积强度和纯IgA沉积率，与IgAN患者相比，IgAVN患者的比例更高（P<0.0001）。IgAVN患者的IgA沉积强度也明显更高(P=0.003)。IgAVN显示出优越的预后，肾脏缓解率较高(P<0.0001)。COX回归分析显示间质纤维化,如在SQC病理学系统中确定的，与这两种情况的预后有关。此外，研究结果表明,IgA沉积水平(IgA++和IgA+++)可能潜在地影响IgAVN的预后.
结论：与IgAVN相比，IgAN在肾功能损害方面表现更严重，间隙损伤,和预后。肾脏内免疫复合物沉积水平和位置的差异支持IgAVN和IgAN作为不同疾病的假设。间质纤维化可能是与IgAVN和IgAN患儿肾脏预后相关的SQC系统内的关键病理指标。IgA沉积的程度也可能与IgAVN的预后有关。
