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一名诊断为嗜酸性肉芽肿性多血管炎患者抗中性粒细胞胞浆抗体谱的变化

A Changing Anti-Neutrophil Cytoplasmic Antibody Profile in a Patient With a Diagnosis of Eosinophilic Granulomatosis With Polyangiitis.

作者信息

Jinno Yusuke, Kozu Yutaka, Hiranuma Hisato, Maruoka Shuichiro, Gon Yasuhiro

机构信息

Division of Respiratory Medicine, Department of Internal Medicine, Nihon University School of Medicine, Tokyo, Japan.

出版信息

J Med Cases. 2023 Aug;14(8):299-306. doi: 10.14740/jmc4088. Epub 2023 Aug 28.

Abstract

This report describes a hitherto unique case of eosinophilic granulomatosis with polyangiitis (EGPA), a subtype of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. The patient was an 81-year-old man whose clinical course involved notable changes in the ANCA profile, specifically a transition from positive proteinase 3 (PR3)-ANCA to myeloperoxidase (MPO)-ANCA, followed by simultaneous positivity for both. The patient's medical history included bronchial asthma, allergic rhinitis, sinusitis, and multiple comorbidities. Despite being initially PR3-ANCA-positive, subsequent admissions demonstrated MPO-ANCA positivity along with eosinophilic manifestations, highlighting the complexity of diagnosis of EGPA. Diagnostic evaluation included imaging, serological markers, and clinical symptoms, which collectively supported the classification of EGPA. Notably, this case challenges the conventional diagnostic paradigms and emphasizes the evolving nature of ANCA profiles in vasculitis. The shift in ANCA profile prompted a reevaluation of the patient's diagnosis and treatment strategy. This case underscores the importance of considering fluctuations in ANCA in patients with a diagnosis of EGPA, management decisions, and potential implications for disease progression. Further research is warranted to elucidate the mechanisms underlying changes in ANCA and their clinical significance in vasculitis.

摘要

本报告描述了一例迄今为止独一无二的嗜酸性肉芽肿性多血管炎(EGPA)病例,它是抗中性粒细胞胞浆抗体(ANCA)相关血管炎的一种亚型。患者为一名81岁男性,其临床病程中ANCA谱有显著变化,具体表现为从蛋白酶3(PR3)-ANCA阳性转变为髓过氧化物酶(MPO)-ANCA阳性,随后两者同时呈阳性。患者的病史包括支气管哮喘、过敏性鼻炎、鼻窦炎以及多种合并症。尽管最初PR3-ANCA呈阳性,但随后的住院显示MPO-ANCA阳性以及嗜酸性表现,凸显了EGPA诊断的复杂性。诊断评估包括影像学检查、血清学标志物和临床症状,这些共同支持了EGPA的分类。值得注意的是,该病例挑战了传统诊断模式,并强调了血管炎中ANCA谱的演变性质。ANCA谱的变化促使对患者的诊断和治疗策略进行重新评估。该病例强调了在诊断为EGPA的患者中考虑ANCA波动的重要性、管理决策以及对疾病进展的潜在影响。有必要进行进一步研究以阐明ANCA变化的潜在机制及其在血管炎中的临床意义。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4ea6/10482595/a41ae781b2b2/jmc-14-299-g001.jpg

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