Antineutrophil cytoplasmic antibody positivity in systemic lupus erythematosus: Diagnostic dilemma and therapeutic implications.

IF 2 4区医学 Q2 PEDIATRICS

Paediatrics & child health Pub Date : 2025-07-31 eCollection Date: 2025-09-01 DOI:10.1093/pch/pxaf059

Lauren Devito, Erkan Demirkaya, Manal Gabril, Micol Romano, Guido Filler, Ajay P Sharma

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引用次数: 0

Abstract

Objectives: Antineutrophil cytoplasmic antibody (ANCA) positivity occurs in up to 25% of systemic lupus erythematosus (SLE) patients, but differentiating incidental ANCA from ANCA-associated vasculitis (AAV) in SLE nephritis remains a diagnostic and therapeutic challenge.

Case: A 14-year-old female presented with a 3-month history of undiagnosed autoimmune manifestations and chronic kidney disease. Based on autoimmune workup and kidney biopsy, she was diagnosed with SLE nephritis with incidental ANCA positivity. Despite appropriate SLE nephritis-directed treatment as per guidelines, an inflammatory flare and superimposed acute kidney injury (AKI) prompted a diagnosis revision to SLE-AAV overlap syndrome. Broadening treatment to target concomitant AAV led to autoimmune remission and AKI resolution.

Conclusions: This case underscores the importance of early suspicion of autoimmune disorders by primary care providers for a timely referral and the critical role of specialists in recognizing the limitations of conventional tools, including renal histology, when distinguishing incidental ANCA positivity from SLE-AAV overlap to guide appropriate treatment.

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系统性红斑狼疮的抗中性粒细胞细胞质抗体阳性：诊断困境和治疗意义。

目的：抗中性粒细胞胞质抗体（ANCA）阳性发生在高达25%的系统性红斑狼疮（SLE）患者中，但在SLE肾炎中区分偶发ANCA与ANCA相关血管炎（AAV）仍然是一个诊断和治疗挑战。病例：一名14岁女性，有3个月未确诊的自身免疫表现和慢性肾脏疾病病史。基于自身免疫检查和肾活检，她被诊断为SLE肾炎并附带ANCA阳性。尽管根据指南进行了适当的SLE肾炎定向治疗，但炎症发作和叠加急性肾损伤（AKI）促使诊断修改为SLE- aav重叠综合征。将治疗范围扩大到针对伴随的AAV导致自身免疫缓解和AKI的解决。结论：该病例强调了初级保健提供者早期怀疑自身免疫性疾病的重要性，以便及时转诊，以及专家在区分偶然的ANCA阳性与SLE-AAV重叠时认识到常规工具（包括肾脏组织学）局限性的关键作用，以指导适当的治疗。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Paediatrics & child health 医学-小儿科

CiteScore

2.10

自引率

5.30%

发文量

208

审稿时长

>12 weeks

期刊介绍： Paediatrics & Child Health (PCH) is the official journal of the Canadian Paediatric Society, and the only peer-reviewed paediatric journal in Canada. Its mission is to advocate for the health and well-being of all Canadian children and youth and to educate child and youth health professionals across the country. PCH reaches 8,000 paediatricians, family physicians and other child and youth health professionals, as well as ministers and officials in various levels of government who are involved with child and youth health policy in Canada.