Calabrese Vincenzo, Gallizzi Romina, Spagnolo Alessandra, Zicarelli Mariateresa, Sutera Diana, Farina Alessandra, Cernaro Valeria, Santoro Domenico
Department of Medicine and Surgery, University of Enna "Kore", 94100 Enna, Italy.
Unit of Pediatric Nephrology and Rheumatology, University of Messina, 98125 Messina, Italy.
Medicina (Kaunas). 2025 Mar 18;61(3):534. doi: 10.3390/medicina61030534.
Wegener's granulomatosis (WG), or granulomatosis with polyangiitis (GPA), is a rare autoimmune disease that can cause inflammation in various organs, including the kidneys. Renal involvement in GPA is a major cause of morbidity and mortality in both adults and children, and early detection and effective treatment are essential for preventing renal failure. This review aims to summarize the current evidence on the incidence, clinical features, treatment, and outcomes of renal involvement in children with Wegener's granulomatosis. The incidence of renal involvement in children with GPA ranged from 26% to 56%. Renal involvement is a common and serious complication of GPA in children, and early detection and effective treatment are crucial for preventing renal failure. The most common clinical features were proteinuria, hematuria, and reduced glomerular filtration rate. The majority of children with renal involvement in GPA required treatment with corticosteroids and immunosuppressive agents. The treatment outcomes varied among the studies, with some children achieving remission of renal involvement while others developed end-stage renal disease. Although most features are the same in children and adult patients, this review summed up some important differences between these two different populations. Further studies are needed to identify the most effective treatment strategies for renal involvement in children with GPA.
韦格纳肉芽肿（WG），即肉芽肿伴多血管炎（GPA），是一种罕见的自身免疫性疾病，可导致包括肾脏在内的多个器官发生炎症。GPA累及肾脏是成人和儿童发病及死亡的主要原因，早期发现和有效治疗对于预防肾衰竭至关重要。本综述旨在总结有关儿童韦格纳肉芽肿肾脏受累的发病率、临床特征、治疗及预后的现有证据。儿童GPA肾脏受累的发病率在26%至56%之间。肾脏受累是儿童GPA常见且严重的并发症，早期发现和有效治疗对于预防肾衰竭至关重要。最常见的临床特征是蛋白尿、血尿和肾小球滤过率降低。大多数GPA肾脏受累的儿童需要使用糖皮质激素和免疫抑制剂进行治疗。各研究的治疗结果有所不同，一些儿童肾脏受累情况得到缓解，而另一些则发展为终末期肾病。尽管儿童和成年患者的大多数特征相同，但本综述总结了这两个不同人群之间的一些重要差异。需要进一步研究以确定针对儿童GPA肾脏受累最有效的治疗策略。
