Twenty Years of Undiagnosed Eosinophilia: Diabetic Attribution Bias Delaying Diagnosis of Anca-Positive EGPA Until End-Stage Organ Damage.

Q3 Medicine
European journal of case reports in internal medicine Pub Date : 2026-08-10 eCollection Date: 2026-01-01 DOI:10.12890/2026_007267
Minha Aslam, Amira Bitar, Stephanie Ammari, Nawras Ibrahim
{"title":"Twenty Years of Undiagnosed Eosinophilia: Diabetic Attribution Bias Delaying Diagnosis of Anca-Positive EGPA Until End-Stage Organ Damage.","authors":"Minha Aslam, Amira Bitar, Stephanie Ammari, Nawras Ibrahim","doi":"10.12890/2026_007267","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>Eosinophilic granulomatosis with polyangiitis (EGPA) has the longest diagnostic delay among anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides, with a reported median of 455 days. Cardiac disease, the leading cause of EGPA-related mortality, is largely preventable with early immunosuppression.</p><p><strong>Case description: </strong>A 53-year-old Hispanic woman with type 2 diabetes, hypertension, and no documented asthma had 20 years of persistent peripheral eosinophilia (absolute eosinophil count 1-2.7 ×10<sup>3</sup>/μl) and active glomerulonephritic sediment. Eosinophilia was first documented at age 33 when no medical comorbidities existed, predating the diabetes diagnosis by years. Renal function declined from creatinine 0.6 mg/dl (2013) to end-stage renal disease requiring peritoneal dialysis (2024). Ejection fraction fell from 70% (2024) to 20-24% (2026), with left heart catheterization excluding ischemic aetiology. Peripheral neuropathy was attributed to diabetes without electrodiagnostic evaluation. Bone marrow biopsy excluded clonal eosinophilic disorders. ANCA testing, never previously ordered, revealed myeloperoxidase (MPO)-ANCA positivity (>8.0 units, reference 0-0.9) with ANCA IIF titre 1:160 in April 2026. The 2022 American College of Rheumatology / European Alliance of Associations for Rheumatology classification criteria score was ≥6 points without asthma or nasal polyps. Seven missed diagnostic opportunities were identified across multiple specialties and facilities over two decades.</p><p><strong>Conclusion: </strong>This case illustrates how \"diabetic attribution bias,\" care fragmentation, and socioeconomic barriers delayed EGPA diagnosis for 20 years despite persistent eosinophilia and progressive multi-organ damage. Systematic ANCA testing in patients with unexplained eosinophilia and organ involvement could prevent irreversible end-organ damage.</p><p><strong>Learning points: </strong>Persistent unexplained eosinophilia (absolute eosinophil count >1 ×10<sup>3</sup>/μl) with active glomerulonephritic sediment warrants (ANCA) testing regardless of asthma status, as myeloperoxidase (MPO)-ANCA-positive eosinophilic granulomatosis with polyangiitis (EGPA) may present without prominent asthma and with predominant vasculitic manifestations including glomerulonephritis and peripheral neuropathy.\"Diabetic attribution bias\": the reflexive attribution of organ damage to diabetes in patients with coexisting diabetes. can delay vasculitis diagnosis for years when renal decline and peripheral neuropathy are present alongside atypical features such as improving glycaemic control and active glomerulonephritic sediment.Corticosteroid exposure suppresses eosinophilia in peripheral blood within hours and in body fluids within days, masking the hallmark laboratory finding of EGPA; eosinophil counts and serosal fluid analyses should always be interpreted in the context of recent steroid use.</p>","PeriodicalId":11908,"journal":{"name":"European journal of case reports in internal medicine","volume":"13 8","pages":"007267"},"PeriodicalIF":0.0000,"publicationDate":"2026-08-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13479843/pdf/","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"European journal of case reports in internal medicine","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.12890/2026_007267","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2026/1/1 0:00:00","PubModel":"eCollection","JCR":"Q3","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 0

Abstract

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) has the longest diagnostic delay among anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides, with a reported median of 455 days. Cardiac disease, the leading cause of EGPA-related mortality, is largely preventable with early immunosuppression.

Case description: A 53-year-old Hispanic woman with type 2 diabetes, hypertension, and no documented asthma had 20 years of persistent peripheral eosinophilia (absolute eosinophil count 1-2.7 ×103/μl) and active glomerulonephritic sediment. Eosinophilia was first documented at age 33 when no medical comorbidities existed, predating the diabetes diagnosis by years. Renal function declined from creatinine 0.6 mg/dl (2013) to end-stage renal disease requiring peritoneal dialysis (2024). Ejection fraction fell from 70% (2024) to 20-24% (2026), with left heart catheterization excluding ischemic aetiology. Peripheral neuropathy was attributed to diabetes without electrodiagnostic evaluation. Bone marrow biopsy excluded clonal eosinophilic disorders. ANCA testing, never previously ordered, revealed myeloperoxidase (MPO)-ANCA positivity (>8.0 units, reference 0-0.9) with ANCA IIF titre 1:160 in April 2026. The 2022 American College of Rheumatology / European Alliance of Associations for Rheumatology classification criteria score was ≥6 points without asthma or nasal polyps. Seven missed diagnostic opportunities were identified across multiple specialties and facilities over two decades.

Conclusion: This case illustrates how "diabetic attribution bias," care fragmentation, and socioeconomic barriers delayed EGPA diagnosis for 20 years despite persistent eosinophilia and progressive multi-organ damage. Systematic ANCA testing in patients with unexplained eosinophilia and organ involvement could prevent irreversible end-organ damage.

Learning points: Persistent unexplained eosinophilia (absolute eosinophil count >1 ×103/μl) with active glomerulonephritic sediment warrants (ANCA) testing regardless of asthma status, as myeloperoxidase (MPO)-ANCA-positive eosinophilic granulomatosis with polyangiitis (EGPA) may present without prominent asthma and with predominant vasculitic manifestations including glomerulonephritis and peripheral neuropathy."Diabetic attribution bias": the reflexive attribution of organ damage to diabetes in patients with coexisting diabetes. can delay vasculitis diagnosis for years when renal decline and peripheral neuropathy are present alongside atypical features such as improving glycaemic control and active glomerulonephritic sediment.Corticosteroid exposure suppresses eosinophilia in peripheral blood within hours and in body fluids within days, masking the hallmark laboratory finding of EGPA; eosinophil counts and serosal fluid analyses should always be interpreted in the context of recent steroid use.

二十年未确诊的嗜酸性粒细胞增多症:糖尿病归因偏倚将anca阳性EGPA的诊断延迟至终末期器官损伤。
在抗中性粒细胞细胞质抗体(ANCA)相关血管炎中,嗜酸性肉芽肿病合并多血管炎(EGPA)的诊断延迟时间最长,据报道中位延迟为455天。心脏病是egpa相关死亡的主要原因,在很大程度上可以通过早期免疫抑制来预防。病例描述:一名53岁的西班牙裔女性,患有2型糖尿病,高血压,无哮喘记录,持续20年的外周嗜酸性粒细胞增多(嗜酸性粒细胞绝对计数1-2.7 ×103/μl)和活动性肾小球肾小球沉积。嗜酸性粒细胞增多症在33岁时首次被记录,当时没有任何医学合并症存在,比糖尿病诊断早了很多年。肾功能从肌酐0.6 mg/dl(2013年)下降到需要腹膜透析的终末期肾病(2024年)。射血分数从70%(2024)下降到20-24%(2026),左心导管排除缺血性病因。周围神经病变被认为是糖尿病,没有电诊断评估。骨髓活检排除克隆性嗜酸性疾病。此前从未订购的ANCA检测于2026年4月显示髓过氧化物酶(MPO)-ANCA阳性(bbb8.0单位,参考文献0-0.9),ANCA IIF滴度为1:160。2022年美国风湿病学会/欧洲风湿病协会联盟分类标准评分≥6分,无哮喘或鼻息肉。二十年来,在多个专业和设施中发现了七次错失的诊断机会。结论:该病例说明了“糖尿病归因偏差”、护理碎片化和社会经济障碍如何在持续嗜酸性粒细胞增多和进行性多器官损伤的情况下延迟了EGPA诊断20年。在不明原因嗜酸性粒细胞增多和器官受累的患者中进行系统的ANCA检测可以预防不可逆的终末器官损害。学习要点:持续不明原因的嗜酸性粒细胞增多(嗜酸性粒细胞绝对计数>1 ×103/μl)伴活动性肾小球肾炎沉积物(ANCA)检测,无论哮喘状态如何,因为髓过氧化物酶(MPO)-ANCA阳性嗜酸性粒细胞肉芽肿病伴多血管炎(EGPA)可能没有明显的哮喘,并伴有主要的血管表现,包括肾小球肾炎和周围神经病变。“糖尿病归因偏差”:并发糖尿病患者器官损伤对糖尿病的反射性归因。当肾脏衰退和周围神经病变同时伴有非典型特征,如血糖控制改善和活跃的肾小球肾炎沉积物时,可将血管炎的诊断延迟数年。皮质类固醇暴露可在数小时内抑制外周血嗜酸性粒细胞,并在数天内抑制体液嗜酸性粒细胞,掩盖了EGPA的标志性实验室发现;嗜酸性粒细胞计数和浆液分析应该总是在最近使用类固醇的背景下解释。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
求助全文
约1分钟内获得全文 求助全文
来源期刊
CiteScore
2.10
自引率
0.00%
发文量
166
审稿时长
8 weeks
期刊介绍: The European Journal of Case Reports in Internal Medicine is an official journal of the European Federation of Internal Medicine (EFIM), representing 35 national societies from 33 European countries. The Journal''s mission is to promote the best medical practice and innovation in the field of acute and general medicine. It also provides a forum for internal medicine doctors where they can share new approaches with the aim of improving diagnostic and clinical skills in this field. EJCRIM welcomes high-quality case reports describing unusual or complex cases that an internist may encounter in everyday practice. The cases should either demonstrate the appropriateness of a diagnostic/therapeutic approach, describe a new procedure or maneuver, or show unusual manifestations of a disease or unexpected reactions. The Journal only accepts and publishes those case reports whose learning points provide new insight and/or contribute to advancing medical knowledge both in terms of diagnostics and therapeutic approaches. Case reports of medical errors, therefore, are also welcome as long as they provide innovative measures on how to prevent them in the current practice (Instructive Errors). The Journal may also consider brief and reasoned reports on issues relevant to the practice of Internal Medicine, as well as Abstracts submitted to the scientific meetings of acknowledged medical societies.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:604180095
Book学术官方微信
小红书