Department of Child Health, Faculty of Medicine, Universitas Indonesia - Cipto Mangunkusumo Hospital, Central Jakarta, Special Capital Region of Jakarta, Indonesia.
Department of Pathology Anatomy, Faculty of Medicine, Universitas Indonesia - Cipto Mangunkusumo Hospital, Central Jakarta, Special Capital Region of Jakarta, Indonesia.
Saudi J Kidney Dis Transpl. 2020 Sep-Oct;31(5):1117-1124. doi: 10.4103/1319-2442.301180.
IgA vasculitis with nephritis (IgAVN), which was formerly known as Henoch-Schonlein purpura nephritis, commonly manifests with mild symptoms. However, in rare circumstances, IgAVN in children can progress to kidney failure. Despite the successful treatment of severe IgAVN with a combination of immunosuppressive medications including corticosteroids, no consensus has been established for IgAVN treatment. Here, we present a case of severe IgAVN in an eight-year-old Indonesian boy who was treated with simultaneous methylprednisolone, cyclophosphamide, and mycophenolic acid. He experienced recovery of kidney function within one month, while proteinuria resolved in five months, and hematuria resolved within a year after treatment initiation. No recurrences were noted during the two-year follow-up. Although our immunosuppressive regimen may seem very potent, it was shown to have tolerable side effects and could be beneficial for kidney recovery. Importantly, they have also been shown to prevent progression to chronic kidney disease in children with severe IgAVN.
IgA 血管炎伴肾炎（IgAVN），以前称为过敏性紫癜肾炎，常表现为轻度症状。然而，在极少数情况下，儿童的 IgAVN 可进展为肾衰竭。尽管联合使用包括皮质类固醇在内的免疫抑制剂治疗严重的 IgAVN 取得了成功，但 IgAVN 的治疗仍未达成共识。在这里，我们报告了一例 8 岁印度尼西亚男孩的严重 IgAVN 病例，他接受了甲基强的松龙、环磷酰胺和霉酚酸酯的联合治疗。他在一个月内恢复了肾功能，五个月后蛋白尿消失，治疗一年后血尿消失。在两年的随访中没有复发。虽然我们的免疫抑制方案看起来非常有效，但它显示出可耐受的副作用，可能有益于肾脏恢复。重要的是，它们还被证明可以预防严重 IgAVN 儿童进展为慢性肾脏病。
