Oni Louise, Sampath Sunil
Department of Paediatric Nephrology, Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, United Kingdom.
Department of Women's and Children's Health, Institute of Translational Medicine, University of Liverpool, Liverpool, United Kingdom.
Front Pediatr. 2019 Jun 27;7:257. doi: 10.3389/fped.2019.00257. eCollection 2019.
Immunoglobulin A vasculitis (IgAV; formerly Henoch Schonlein Purpura) is the most common form of childhood vasculitis. It can occur in any age and peaks around 4-6 years old. It demonstrates seasonal variation implicating a role for environmental triggers and geographical variation. The diagnosis is made clinically and 95% of patients will present with a rash, together with any from a triad of other systems-gastrointestinal, musculoskeletal, and renal. Most cases of IgAV in children have an excellent outcome. Treatment may be required during the acute phase for gastrointestinal involvement and renal involvement, termed IgAV nephritis (previously HSP nephritis), is the most serious long-term manifestation accounting for ~1-2% of all childhood end stage kidney disease (ESKD). It therefore requires a period of renal monitoring conducted for 6-12 months. Patients presenting with nephrotic and/or nephritic syndrome or whom develop significant persistent proteinuria should undergo a renal biopsy to evaluate the extent of renal inflammation and there are now international consensus guidelines that outline the indications for when to do this. At present there is no evidence to support the use of medications at the outset in all patients to prevent subsequent renal inflammation. Consensus management guidelines suggest using oral corticosteroids for milder disease, oral, or intravenous corticosteroids plus azathioprine or mycophenolate mofetil or intravenous cyclophosphamide for moderate disease and intravenous corticosteroids with cyclophosphamide for severe disease. Angiotensin system inhibitors act as adjunctive treatment for persisting proteinuria and frequently relapsing disease may necessitate the use of immunosuppressant agents. Renal outcomes in this disease have remained static over time and progress may be hindered due to many reasons, including the lack of reliable disease biomarkers and an absence of core outcome measures allowing for accurate comparison between studies. This review article summarizes the current evidence supporting the management of this condition highlighting recent findings and areas of unmet need. In order to improve the long term outcomes in this condition international research collaboration is urgently required.
免疫球蛋白A血管炎（IgAV；原称过敏性紫癜）是儿童血管炎最常见的形式。它可发生于任何年龄，发病高峰在4至6岁左右。该病呈现季节性变化，提示环境触发因素起作用，同时也存在地理差异。临床即可做出诊断，95%的患者会出现皮疹，同时伴有胃肠道、肌肉骨骼和肾脏这三个其他系统中的任何一个系统的症状。儿童IgAV的大多数病例预后良好。急性期可能需要针对胃肠道受累情况进行治疗，而肾脏受累情况，即IgAV肾炎（以前称为过敏性紫癜肾炎），是最严重的长期表现，占所有儿童终末期肾病（ESKD）的1%至2%左右。因此需要进行6至12个月的肾脏监测。出现肾病和/或肾炎综合征或出现显著持续性蛋白尿的患者应进行肾活检，以评估肾脏炎症的程度，目前有国际共识指南概述了进行肾活检的指征。目前没有证据支持一开始就对所有患者使用药物来预防后续的肾脏炎症。共识管理指南建议，对于病情较轻的患者使用口服糖皮质激素，对于中度病情使用口服或静脉注射糖皮质激素加硫唑嘌呤或霉酚酸酯或静脉注射环磷酰胺，对于重度病情使用静脉注射糖皮质激素加环磷酰胺。血管紧张素系统抑制剂可作为持续性蛋白尿的辅助治疗，频繁复发的疾病可能需要使用免疫抑制剂。随着时间的推移，这种疾病的肾脏预后一直没有变化，由于多种原因，包括缺乏可靠的疾病生物标志物以及缺乏能够在研究之间进行准确比较的核心结局指标，进展可能会受到阻碍。这篇综述文章总结了支持这种疾病管理的当前证据，突出了近期的发现和未满足需求的领域。为了改善这种疾病的长期预后，迫切需要开展国际研究合作。
