Department of Pediatrics, Unit of Pediatric Nephrology, Faculty of Medicine, Federal University of Minas Gerais (UFMG), Belo Horizonte, MG, Brazil.
Interdisciplinary Laboratory of Medical Investigation, Faculty of Medicine, UFMG, Belo Horizonte, MG, Brazil.
Curr Med Chem. 2022;29(34):5543-5559. doi: 10.2174/0929867329666220613103316.
Acute post-streptococcal glomerulonephritis (APSGN) is an immune- complex (ICs) mediated glomerular disease triggered by group A β-hemolytic streptococcus (GAS) or Streptococcus pyogenes infections. APSGN represents a major cause of acquired kidney injury in children.
This non-systematic review summarizes recent evidence on APSGN. We discuss the epidemiology, pathogenesis, clinical and laboratory findings, histopathology, treatment and prognosis of the disease.
The median APSGN incidence in children in developing countries is estimated at 24.3/100,000 per year, compared with 6.2/100,000 per year in developed countries. Nephritis-associated plasmin receptor, identified as glyceraldehyde-3-phosphate dehydrogenase, and the cationic cysteine proteinase streptococcal pyrogenic exotoxin B are thought to be two leading streptococcal antigens involved in the pathogenesis of APSGN, which activate the complement system, mainly via the alternative but also the lectin pathway. This process is critical for the generation of inflammation by the ICs deposited in the glomerulus. The classic phenotype is an acute diffuse proliferative glomerulonephritis leading to features of the nephritic syndrome, including hematuria, oliguria, hypertension and edema. The histopathology shows that the glomeruli are diffusely affected, mostly presenting enlarged glomerular tuffs due to hypercellularity. Proliferative endothelial and mesangial cells and inflammation have also been observed. APSGN frequently has spontaneous recovery. There is no specific therapy, but its morbidity and mortality are drastically reduced by the prevention and/or treatment of complications.
Despite recent advances, the pathogenesis of APSGN is not fully understood. There is no specific treatment for APSGN. The prognosis is generally good. However, some cases may evolve into chronic kidney disease.
急性链球菌后肾小球肾炎（APSGN）是一种由 A 组β溶血性链球菌（GAS）或化脓性链球菌感染引起的免疫复合物（ICs）介导的肾小球疾病。APSGN 是儿童获得性肾损伤的主要原因。
本非系统性综述总结了 APSGN 的最新证据。我们讨论了疾病的流行病学、发病机制、临床和实验室表现、组织病理学、治疗和预后。
发展中国家儿童 APSGN 的中位发病率估计为每年 24.3/10 万，而发达国家为每年 6.2/10 万。肾炎相关纤溶酶受体，鉴定为甘油醛-3-磷酸脱氢酶，以及阳离子半胱氨酸蛋白酶化脓性链球菌外毒素 B，被认为是参与 APSGN 发病机制的两种主要链球菌抗原，它们激活补体系统，主要通过替代途径，但也通过凝集素途径。这一过程对于 IC 在肾小球中沉积引发炎症至关重要。经典表型是急性弥漫性增生性肾小球肾炎，导致肾炎综合征的特征，包括血尿、少尿、高血压和水肿。组织病理学显示肾小球弥漫性受累，主要表现为肾小球毛细胞增多导致肾小球增大。也观察到增生性内皮细胞和系膜细胞和炎症。APSGN 常可自行恢复。目前尚无特异性治疗方法，但通过预防和/或治疗并发症可大大降低发病率和死亡率。
尽管最近取得了进展，但 APSGN 的发病机制尚未完全了解。目前尚无特异性治疗 APSGN 的方法。预后一般良好。然而，一些病例可能会发展为慢性肾脏病。
