英国肾脏协会指南审查:儿童和青少年 IgA 血管炎(Henoch-Schönlein 紫癜)的初始管理 "以及 "儿童和青少年并发症相关 IgA 血管炎(Henoch-Schönlein 紫癜)的管理"。

Charlotte Day, Rachel Shute
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引用次数: 0

摘要

IgA 血管炎(IgAV)以前被称为 "过敏性紫癜"(HSP),是一种由 IgA 介导的自身免疫性血管炎,通常累及皮肤、关节、胃肠道和肾脏。在 IgAV 中,IgA 复合物沉积在这些器官的小血管内皮上,引起炎症。IgAV 的发病率约为五千分之一,主要影响 2 至 10 岁的儿童,高峰期为 4 至 7 岁1 ,也可影响青少年和成人,是英国儿科医生、全科医生和急诊科医生常见的儿童疾病。尽管如此,该领域的研究仍然有限,因此目前在 IgAV 的初始治疗和并发症治疗方面都存在差异。虽然大多数 IgAV 病例可通过保守治疗或初级和二级医疗机构的治疗自行缓解,但有些患儿会出现严重的、改变生活或危及生命的并发症,如急性消化道出血和终末期肾病,需要三级医疗中心介入治疗。2 对这些患儿进行仔细的专科转诊、筛查和早期治疗非常重要,以确保及时预防或治疗这些并发症。### 当前指南的相关信息 当前指南由英国肾脏协会(UKKA)于 2022 年 12 月发布,是首个针对儿童和青少年 IgAV 初期管理以及 IgAV 相关并发症管理的国家指南。2019 年,SHARE(欧洲儿科风湿病学单一枢纽和接入点)发布了第一份国际指南,为诊断和治疗提供建议,其目的同样是提供循证建议,并使各国和各中心的治疗标准化。...
本文章由计算机程序翻译,如有差异,请以英文原文为准。
UK Kidney Association guideline review: ‘The initial management of IgA vasculitis (Henoch-Schönlein purpura) in children and young people’ in conjunction with ‘The management of complications-associated IgA vasculitis (Henoch-Schönlein purpura) in children and young people’
IgA vasculitis (IgAV), previously known as Henoch-Schönlein purpura (HSP), is an IgA-mediated autoimmune vasculitis typically affecting the skin, joints, gastrointestinal (GI) tract and kidneys. In IgAV, IgA complexes are deposited on the endothelium of the small vessels in these organs, causing inflammation. IgAV has an incidence of approximately 1 in 5000, predominantly affects children between the ages of 2 and 10 years and peaks between 4 and 7 years,1 can affect teenagers and adults, and is a common childhood condition seen by paediatricians, general practitioners (GPs) and emergency department (ED) doctors across the UK. Despite this, the research in this field is still limited, so currently, there are discrepancies in both the initial management and management of complications related to IgAV. While most cases of IgAV self-resolve with conservative management or management in primary and secondary care, some children will develop severe, life-changing or life-threatening complications such as acute GI bleeding and end-stage renal disease requiring tertiary centre involvement.2 It is important that careful specialist referral, screening and early management of these children occur to ensure that these complications are prevented or treated in a timely manner. ### Information about the current guideline The current guidelines were published in December 2022 by the UK Kidney Association (UKKA), and are the first national guidelines for the initial management of IgAV and the management of complications associated with IgAV in children and young people. In 2019, the first international guidelines providing recommendations for diagnosis and treatment were published by SHARE (Single Hub and Access Point for paediatric Rheumatology in Europe) with the same aim to provide evidence-based recommendations and to standardise treatments throughout countries and centres.3 Until now, due to lack of best practice recommendations in the UK, experts in different National Health Service trusts have been formulating guidelines based on the most recent research. …
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