PDF(Pubmed)
Abstract：
UNASSIGNED: IgG4-associated kidney disease (IgG4-RKD) encompasses a spectrum of disorders, predominantly featuring tubulointerstitial nephritis (TIN) and membranous glomerulonephropathy (MGN). The limited understanding of the co-occurrence of IgG4-RD-TIN with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) poses a diagnostic and therapeutic challenge.
UNASSIGNED: We examined 49 cases, comprising 21 cases of IgG4-RD-TIN (group A), 10 cases of IgG4-RD-TIN accompanied with MGN (group B), and 18 cases of IgG4-RD-TIN concurrent with AAV (group C), at the First Affiliated Hospital of Zhejiang University, China, from June 2015 to December 2022.
UNASSIGNED: The mean age and gender of the three IgG4-RKD subtypes were not statistically significant. IgG4-RD-TIN exhibited higher serum creatinine and a higher incidence of hypocomplementemia (group A 47.6%, group B 30%, group C 16.7%). IgG4-RD-TIN-MGN was characterized by proteinuria (group A 0.3 g/d, group B 4.0 g/d, group C 0.8 g/d, P < 0.001) and hypoalbuminemia. IgG4-RD-TIN-AAV exhibited hypohemoglobinemia (group A 103.45 g/l, group B 119.60 g/l, group C 87.94 g/l, P < 0.001) and a high level of urine erythrocytes. The primary treatment for IgG4-RD-TIN was steroids alone, whereas IgG4-RD-TIN-MGN and IgG4-RD-TIN-AAV necessitated combination therapy. Group A experienced two relapses, whereas groups B and C had no relapses. There was no significant difference in patient survival among the three groups, and only two cases in group C suffered sudden death.
UNASSIGNED: This study provides valuable insights into clinical manifestations, auxiliary examination features, pathological characteristics, and prognosis of IgG4-RD-TIN, IgG4-RD-TIN-MGN, and IgG4-RD-TIN concurrent AAV. Large-scale studies are required to validate these findings.
UNASSIGNED: We examined 49 cases, comprising 21 cases of IgG4-RD-TIN (group A), 10 cases of IgG4-RD-TIN accompanied with MGN (group B), and 18 cases of IgG4-RD-TIN concurrent with AAV (group C), at the First Affiliated Hospital of Zhejiang University, China, from June 2015 to December 2022.
UNASSIGNED: The mean age and gender of the three IgG4-RKD subtypes were not statistically significant. IgG4-RD-TIN exhibited higher serum creatinine and a higher incidence of hypocomplementemia (group A 47.6%, group B 30%, group C 16.7%). IgG4-RD-TIN-MGN was characterized by proteinuria (group A 0.3 g/d, group B 4.0 g/d, group C 0.8 g/d, P < 0.001) and hypoalbuminemia. IgG4-RD-TIN-AAV exhibited hypohemoglobinemia (group A 103.45 g/l, group B 119.60 g/l, group C 87.94 g/l, P < 0.001) and a high level of urine erythrocytes. The primary treatment for IgG4-RD-TIN was steroids alone, whereas IgG4-RD-TIN-MGN and IgG4-RD-TIN-AAV necessitated combination therapy. Group A experienced two relapses, whereas groups B and C had no relapses. There was no significant difference in patient survival among the three groups, and only two cases in group C suffered sudden death.
UNASSIGNED: This study provides valuable insights into clinical manifestations, auxiliary examination features, pathological characteristics, and prognosis of IgG4-RD-TIN, IgG4-RD-TIN-MGN, and IgG4-RD-TIN concurrent AAV. Large-scale studies are required to validate these findings.
摘要：
■IgG4相关肾脏疾病（IgG4-RKD）包括一系列疾病，主要表现为肾小管间质性肾炎（TIN）和膜性肾小球肾病（MGN）。对IgG4-RD-TIN与抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）共同出现的了解有限，提出了诊断和治疗挑战。
■我们检查了49例，包括21例IgG4-RD-TIN（A组），10例IgG4-RD-TIN伴MGN（B组），18例IgG4-RD-TIN合并AAV（C组），浙江大学附属第一医院，中国,从2015年6月到2022年12月。
■三种IgG4-RKD亚型的平均年龄和性别无统计学意义。IgG4-RD-TIN表现出更高的血清肌酐和更高的低补体血症发生率（A组47.6％，B组30%,C组16.7%)。IgG4-RD-TIN-MGN以蛋白尿为特征（A组0.3g/d，B组4.0g/d，C组0.8g/d，P<0.001）和低蛋白血症。IgG4-RD-TIN-AAV表现出低血红蛋白血症（A组103.45g/l，B组119.60g/l，C组87.94g/l，P<0.001）和高水平的尿红细胞。IgG4-RD-TIN的主要治疗是单独使用类固醇，而IgG4-RD-TIN-MGN和IgG4-RD-TIN-AAV需要联合治疗。A组经历了两次复发，而B组和C组无复发。三组患者生存率无显著差异,C组仅2例猝死。
■这项研究为临床表现提供了有价值的见解，辅助检查功能，病理特征，和IgG4-RD-TIN的预后，IgG4-RD-TIN-MGN,和IgG4-RD-TIN并发AAV。需要大规模的研究来验证这些发现。
■我们检查了49例，包括21例IgG4-RD-TIN（A组），10例IgG4-RD-TIN伴MGN（B组），18例IgG4-RD-TIN合并AAV（C组），浙江大学附属第一医院，中国,从2015年6月到2022年12月。
■三种IgG4-RKD亚型的平均年龄和性别无统计学意义。IgG4-RD-TIN表现出更高的血清肌酐和更高的低补体血症发生率（A组47.6％，B组30%,C组16.7%)。IgG4-RD-TIN-MGN以蛋白尿为特征（A组0.3g/d，B组4.0g/d，C组0.8g/d，P<0.001）和低蛋白血症。IgG4-RD-TIN-AAV表现出低血红蛋白血症（A组103.45g/l，B组119.60g/l，C组87.94g/l，P<0.001）和高水平的尿红细胞。IgG4-RD-TIN的主要治疗是单独使用类固醇，而IgG4-RD-TIN-MGN和IgG4-RD-TIN-AAV需要联合治疗。A组经历了两次复发，而B组和C组无复发。三组患者生存率无显著差异,C组仅2例猝死。
■这项研究为临床表现提供了有价值的见解，辅助检查功能，病理特征，和IgG4-RD-TIN的预后，IgG4-RD-TIN-MGN,和IgG4-RD-TIN并发AAV。需要大规模的研究来验证这些发现。
