Department of Pediatric Nephrology, Dr. Sami Ulus Maternity and Child Health and Diseases Training and Research Hospital, Ankara, Turkey.
Department of Pathology, Dr. Sami Ulus Maternity and Child Health and Diseases Training and Research Hospital, Ankara, Turkey.
Clin Exp Nephrol. 2019 Jul;23(7):939-947. doi: 10.1007/s10157-019-01726-5. Epub 2019 Mar 20.
Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood. The long-term prognosis is variable and depends on renal involvement. The aims of this study were to investigate the clinical and laboratory characteristics of our HSP patients, to identify the risk factors for the development of Henoch-Schönlein purpura nephritis (HSPN) and to assess the efficacy of the Oxford Classification system for predicting renal outcomes.
We performed a retrospective review of HSP patients who admitted to our center between 2001 and 2016, and were < 18 years on admission.
A total of 1120 children with HSP were analyzed. Their mean age was 7.4 ± 3.4 years. At onset, purpura was present in all cases, arthritis/arthralgia in 42.4%, abdominal involvement in 39% and renal involvement in 37%. Risk factors for the development of nephritis were age ≥ 8 years, atypical distribution of purpura, ESR > 20 mm/h and abdominal pain. Renal biopsy was performed on 75 patients before immunosuppressive treatment. The mesangial score was strongly associated with proteinuria. Segmental glomerulosclerosis, tubular atrophy/interstitial fibrosis, and crescent formation of ≥ 50% were associated with reduced eGFR at the time of biopsy. A Kaplan-Meier plot showed that segmental glomerulosclerosis and tubular atrophy/interstitial fibrosis significantly predict poor renal outcome.
The long-term morbidity of HSP is predominantly attributed to renal involvement. Patients with HSP, who have a high risk to develop nephritis, could be followed for longer periods of time. The Oxford classification is useful in predicting long-term outcomes of HSPN.
过敏性紫癜（HSP）是儿童中最常见的血管炎。长期预后存在差异，取决于肾脏受累情况。本研究旨在调查 HSP 患者的临床和实验室特征，确定发生过敏性紫癜肾炎（HSPN）的危险因素，并评估牛津分类系统预测肾脏结局的有效性。
我们对 2001 年至 2016 年期间在我院住院的 HSP 患者进行回顾性研究，纳入标准为入院时年龄<18 岁。
共分析了 1120 例 HSP 患儿。平均年龄为 7.4±3.4 岁。发病时所有患者均有紫癜，42.4%有关节炎/关节痛，39%有腹部受累，37%有肾脏受累。发生肾炎的危险因素为年龄≥8 岁、紫癜分布不典型、ESR>20mm/h 和腹痛。在免疫抑制治疗前对 75 例患者进行了肾活检。系膜评分与蛋白尿密切相关。节段性肾小球硬化、肾小管萎缩/间质纤维化和新月体形成≥50%与活检时 eGFR 降低相关。Kaplan-Meier 图显示节段性肾小球硬化和肾小管萎缩/间质纤维化显著预测肾脏不良结局。
HSP 的长期发病率主要归因于肾脏受累。有发生肾炎高风险的 HSP 患者需要进行更长时间的随访。牛津分类系统有助于预测 HSPN 的长期结局。
