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

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

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嗜酸性肉芽肿合并多血管炎患者抗中性粒细胞细胞质抗体谱的变化
本报告描述了一个迄今为止独特的嗜酸性肉芽肿病合并多血管炎(EGPA)的病例,多血管炎是抗中性粒细胞细胞质抗体(ANCA)相关血管炎的一个亚型。患者为81岁男性,其临床过程涉及ANCA谱的显著变化,特别是从蛋白酶3 (PR3)-ANCA阳性转变为髓过氧化物酶(MPO)-ANCA,随后两者同时呈阳性。病史包括支气管哮喘、变应性鼻炎、鼻窦炎及多种合并症。尽管最初为pr3 - anca阳性,但随后入院时显示MPO-ANCA阳性并伴有嗜酸性粒细胞的表现,这突出了EGPA诊断的复杂性。诊断评价包括影像学、血清学指标和临床症状,这些指标共同支持EGPA的分类。值得注意的是,该病例挑战了传统的诊断范式,并强调了血管炎中ANCA谱的演变性质。ANCA概况的转变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、管理决策和疾病进展的潜在影响的重要性。需要进一步的研究来阐明ANCA变化的机制及其在血管炎中的临床意义。
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