Minha Aslam, Amira Bitar, Stephanie Ammari, Nawras Ibrahim
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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.
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.