Andersen R F, Rubak S, Jespersen B, Rittig S
Department of Paediatrics, Aarhus University Hospital, Skejby, Aarhus, Denmark.
Scand J Urol Nephrol. 2009;43(5):409-15. doi: 10.3109/00365590903164480.
Renal involvement in Henoch-Schönlein purpura (HSP) constitutes a risk of end-stage renal disease (ESRD), especially in patients presenting with nephrotic syndrome.
The clinical courses of six patients (mean age 13.2 years; four boys and two girls) admitted from 2000 to 2007 with HSP and nephrotic syndrome were reviewed. Average follow-up was 44 months (28-59). Treatment protocols included oral prednisolone and in non-responders cyclosporin A, cyclophosphamide, mycophenolate mofetil or tacrolimus. Five patients were treated immediately after presentation of nephrotic syndrome/nephrotic range proteinuria (median 277 mg/m(2)/h). The last patient was treated locally with low-dose prednisolone (0.2-0.9 mg/kg/day) and 3 months of low-dose cyclophosphamide (1 mg/kg/day).
All five patients treated promptly with high-dose immunosuppressant had normal estimated glomerular filtration rate (eGFR) (median 159 ml/min/1.73 m(2)) at follow-up. One obtained complete remission, two had positive dipstick proteinuria and two needed angiotensin-converting enzyme inhibitors to stay normotensive. The patient receiving low-dose immunosuppression at onset progressed to ESRD 44 months later. At initial presentation eGFR, blood pressure, renal biopsy grading, proteinuric range and plasma albumin were similar in all patients.
Follow-up data from the patients suggest that an early aggressive immunosuppressive approach improves long-term renal outcome in HSP patients with nephrotic syndrome.
过敏性紫癜（HSP）累及肾脏会导致终末期肾病（ESRD）风险增加，尤其是肾病综合征患者。
回顾性分析2000年至2007年间收治的6例HSP合并肾病综合征患者（平均年龄13.2岁；4例男性，2例女性）的临床病程。平均随访时间为44个月（28 - 59个月）。治疗方案包括口服泼尼松龙，对无反应者使用环孢素A、环磷酰胺、霉酚酸酯或他克莫司。5例患者在出现肾病综合征/肾病范围蛋白尿（中位数277mg/m²/h）后立即接受治疗。最后1例患者在当地接受低剂量泼尼松龙（0.2 - 0.9mg/kg/天）及3个月低剂量环磷酰胺（1mg/kg/天）治疗。
5例立即接受大剂量免疫抑制剂治疗的患者在随访时估计肾小球滤过率（eGFR）正常（中位数159ml/min/1.73m²）。1例完全缓解，2例尿试纸法蛋白尿阳性，2例需要使用血管紧张素转换酶抑制剂以维持血压正常。起病时接受低剂量免疫抑制治疗的患者44个月后进展为ESRD。所有患者初诊时的eGFR、血压、肾活检分级、蛋白尿范围及血浆白蛋白水平相似。
患者的随访数据表明，早期积极的免疫抑制方法可改善HSP肾病综合征患者的长期肾脏预后。
