ANCA阴性ANCA相关性血管炎："血管炎筛查 "的陷阱。

IF 3.6 4区医学 Q1 MEDICINE, GENERAL & INTERNAL

Clinical Medicine Pub Date : 2023-11-01 DOI:10.7861/clinmed.2023-0398

Hannah Cooke, Matthew Wells, Keith Miller, Andrew R L Medford, Sam Patel

{"title":"ANCA阴性ANCA相关性血管炎：\"血管炎筛查 \"的陷阱。","authors":"Hannah Cooke, Matthew Wells, Keith Miller, Andrew R L Medford, Sam Patel","doi":"10.7861/clinmed.2023-0398","DOIUrl":null,"url":null,"abstract":"Despite its recognition as an 'ANCA-associated vasculitis' (AAV), eosinophilic granulomatosis with polyangiitis (EGPA) is ANCA negative in up to 60% of cases. Herein, we report the case of a young man with a clinical syndrome highly suggestive of EGPA but with repeated negative ANCA serology, ultimately presenting with cardiac arrest before recognition of the primary systemic vasculitis, whereupon he received successful induction therapy with high dose glucocorticoids and cyclophosphamide. The case illustrates the importance of awareness of ANCA negative AAV among general physicians in order to minimise morbidity and mortality.","PeriodicalId":10492,"journal":{"name":"Clinical Medicine","volume":"23 6","pages":"630-632"},"PeriodicalIF":3.6000,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"ANCA-negative ANCA-associated vasculitis: pitfalls of the 'vasculitis screen'.\",\"authors\":\"Hannah Cooke, Matthew Wells, Keith Miller, Andrew R L Medford, Sam Patel\",\"doi\":\"10.7861/clinmed.2023-0398\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Despite its recognition as an 'ANCA-associated vasculitis' (AAV), eosinophilic granulomatosis with polyangiitis (EGPA) is ANCA negative in up to 60% of cases. Herein, we report the case of a young man with a clinical syndrome highly suggestive of EGPA but with repeated negative ANCA serology, ultimately presenting with cardiac arrest before recognition of the primary systemic vasculitis, whereupon he received successful induction therapy with high dose glucocorticoids and cyclophosphamide. The case illustrates the importance of awareness of ANCA negative AAV among general physicians in order to minimise morbidity and mortality.\",\"PeriodicalId\":10492,\"journal\":{\"name\":\"Clinical Medicine\",\"volume\":\"23 6\",\"pages\":\"630-632\"},\"PeriodicalIF\":3.6000,\"publicationDate\":\"2023-11-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Clinical Medicine\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.7861/clinmed.2023-0398\",\"RegionNum\":4,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"MEDICINE, GENERAL & INTERNAL\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Clinical Medicine","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.7861/clinmed.2023-0398","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"MEDICINE, GENERAL & INTERNAL","Score":null,"Total":0}

引用次数: 0

摘要

尽管嗜酸性粒细胞肉芽肿伴多血管炎（EGPA）被认为是一种 "ANCA相关性血管炎"（AAV），但高达60%的病例ANCA呈阴性。在此，我们报告了一例临床综合征高度提示 EGPA 但 ANCA 血清学反复阴性的年轻男性病例，他最终在被确认为原发性系统性血管炎之前出现心脏骤停，随后接受了大剂量糖皮质激素和环磷酰胺的诱导治疗，并获得成功。该病例说明了普通医生对ANCA阴性AAV认识的重要性，以便将发病率和死亡率降至最低。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

查看原文本刊更多论文

ANCA-negative ANCA-associated vasculitis: pitfalls of the 'vasculitis screen'.

Despite its recognition as an 'ANCA-associated vasculitis' (AAV), eosinophilic granulomatosis with polyangiitis (EGPA) is ANCA negative in up to 60% of cases. Herein, we report the case of a young man with a clinical syndrome highly suggestive of EGPA but with repeated negative ANCA serology, ultimately presenting with cardiac arrest before recognition of the primary systemic vasculitis, whereupon he received successful induction therapy with high dose glucocorticoids and cyclophosphamide. The case illustrates the importance of awareness of ANCA negative AAV among general physicians in order to minimise morbidity and mortality.

求助全文

通过发布文献求助，成功后即可免费获取论文全文。去求助

来源期刊

Clinical Medicine 医学-医学：内科

CiteScore

7.20

自引率

0.00%

发文量

审稿时长

6-12 weeks

期刊介绍： Clinical Medicine is aimed at practising physicians in the UK and overseas and has relevance to all those managing or working within the healthcare sector. Available in print and online, the journal seeks to encourage high standards of medical care by promoting good clinical practice through original research, review and comment. The journal also includes a dedicated continuing medical education (CME) section in each issue. This presents the latest advances in a chosen specialty, with self-assessment questions at the end of each topic enabling CPD accreditation to be acquired. ISSN: 1470-2118 E-ISSN: 1473-4893 Frequency: 6 issues per year