Altugan Fatma Semsa, Ozen Seza, Aktay-Ayaz Nuray, Güçer Safak, Topaloğlu Rezan, Düzova Ali, Ozaltin Fatih, Beşbaş Nesrin
Unit of Pediatric Nephrology and Rheumatology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Turk J Pediatr. 2009 Nov-Dec;51(6):551-5.
The prognosis of Henoch-Schönlein purpura (HSP) nephritis is more severe than originally thought, with a significant portion progressing to deterioration of renal function in adulthood. Proteinuria adversely affects the outcome. The aim of this study was to evaluate the initial single-center results of a treatment protocol for severe HSP nephritis based on the Heaton classification. Age, gender, clinical features and duration of disease follow-up were assessed. Glomerular filtration rate (GFR), urinalysis and 24-hour urinary protein excretion were analyzed. All patients with severe renal involvement were biopsied and a treatment plan was assigned: Class II received oral steroids, Class III (with crescentic nephritis) received additional oral cyclophosphamide 2 mg/kg/d for 12 weeks, and Classes IV and V received azathioprine for 9 months subsequent to the treatment for Class III. All patients received angiotensin converting enzyme (ACE) inhibitors regardless of their blood pressure values. Eighteen patients presenting with severe HSP nephritis, defined as heavy proteinuria and/or decreased renal function, were evaluated. Based on the renal histology, 5, 10, 1 and 2 of the patients were classified as Classes II, III, IV and V, respectively. At presentation, 7 of the patients had impaired renal function with GFR below 75 ml/min/1.73 m2. With the presented treatment schema, all GFR returned to normal at the end of four years of follow-up. There was no proteinuria in any of the patients; only 8 had microscopic hematuria. This preliminary study suggests a stepwise treatment according to the renal histology. The excellent results with complete disappearance of proteinuria and normal renal function justify the use of the aforementioned immunosuppressive protocol with ACE inhibition. Long-term, multicenter controlled studies are needed to verify our results.
过敏性紫癜（HSP）肾炎的预后比最初认为的更为严重，相当一部分患者在成年后会进展为肾功能恶化。蛋白尿对预后有不利影响。本研究的目的是评估基于希顿分类的重症HSP肾炎治疗方案的单中心初始结果。评估了年龄、性别、临床特征和疾病随访时间。分析了肾小球滤过率（GFR）、尿液分析和24小时尿蛋白排泄情况。所有重症肾脏受累患者均接受了活检，并制定了治疗方案：II级患者接受口服类固醇治疗，III级（伴有新月体性肾炎）患者额外口服环磷酰胺2mg/kg/d，持续12周，IV级和V级患者在III级治疗后接受硫唑嘌呤治疗9个月。所有患者无论血压值如何均接受血管紧张素转换酶（ACE）抑制剂治疗。对18例表现为重症HSP肾炎（定义为大量蛋白尿和/或肾功能下降）的患者进行了评估。根据肾脏组织学检查，分别有5例、10例、1例和2例患者被分类为II级、III级、IV级和V级。就诊时，7例患者肾功能受损，GFR低于75ml/min/1.73m²。采用所提出的治疗方案，在随访四年结束时，所有患者的GFR均恢复正常。所有患者均无蛋白尿；只有8例有镜下血尿。这项初步研究表明应根据肾脏组织学进行逐步治疗。蛋白尿完全消失且肾功能正常的优异结果证明了使用上述免疫抑制方案联合ACE抑制治疗的合理性。需要进行长期、多中心对照研究来验证我们的结果。
