A 13-year-old boy underwent a kidney biopsy for recurrent macrohematuria and proteinuria, resulting in an IgAN diagnosis (MEST-C score M1-E1-S0-T0-C1). At admission, serum creatinine and UPCR were slightly increased. Three methylprednisolone pulses were performed, followed by prednisone and RAAS inhibitors therapy. However, after 10 months, macrohematuria became constant, and UPCR increased. A new kidney biopsy was performed, showing an increase in sclerotic lesions. Prednisone was discontinued, and a trial with IBD TRF-budesonide 9 mg/day started. One month later, macrohematuria episodes disappeared and UPCR decreased, with a stable kidney function. After 5 months, due to a reduction in morning cortisol levels and difficulty in drug provisioning, we started to wean TRF-budesonide by 3 mg every 3 months, with complete withdrawal after 1 year. During this period, episodes of macrohematuria dramatically decreased, and UPCR and kidney function were maintained stable.
Our case demonstrates that TRF-budesonide could be considered an effective second-line treatment in pediatric IgAN, particularly when a long course of steroids is necessary to control active inflammation. However, pediatric clinical trials to identify the correct dosage and tolerability of TRF-budesonide are urgently needed.
■一名13岁男孩因复发性大血尿和蛋白尿而接受肾活检,导致IgAN诊断(MEST-C评分M1-E1-S0-T0-C1)。入院时,血清肌酐和UPCR略有升高.进行了三次甲基强的松龙脉冲,其次是泼尼松和RAAS抑制剂治疗。然而,10个月后,巨大血尿变得恒定,UPCR增加。做了新的肾活检,显示硬化病变增加。泼尼松停药,并开始使用IBDTRF-布地奈德9mg/天的试验。一个月后,巨大血尿发作消失,UPCR减少,有稳定的肾功能.5个月后,由于早晨皮质醇水平降低和药物供应困难,我们开始每3个月用3毫克的TRF-布地奈德断奶,1年后完全退出。在此期间,巨大血尿的发作显著减少,UPCR和肾功能维持稳定。
结论:我们的病例表明TRF-布地奈德可被认为是小儿IgAN的有效二线治疗,特别是当需要长期服用类固醇来控制活动性炎症时。然而,迫切需要进行儿科临床试验,以确定TRF-布地奈德的正确剂量和耐受性.