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血管细丝与肾单位巢:探究高安动脉炎与膜性肾病之间的关联

Vascular Threads and Nephron Nests: Exploring the Association Between Takayasu Arteritis and Membranous Nephropathy.

作者信息

Aamer Sameen, Rajan Anand, Arora Swati

机构信息

Department of Internal Medicine, Allegheny General Hospital, Pittsburgh, USA.

Department of Radiology, Allegheny General Hospital, Pittsburgh, USA.

出版信息

Eur J Case Rep Intern Med. 2024 Jul 2;11(8):004557. doi: 10.12890/2024_004557. eCollection 2024.

Abstract

UNLABELLED

Takayasu arteritis (TA) primarily causes ischaemic nephrosclerosis but can occasionally be associated with glomerulopathy. We report a case of a female in her twenties with PLA2-negative, THSD7A-positive membranous nephropathy (MN) refractory to rituximab, who presented with neck pain and new-onset hypertension. Blood work showed elevated inflammatory markers. Imaging of the head and neck revealed focal dilation and irregularity of the vertebral arteries, consistent with TA. The patient was started on treatment with steroids, followed by mycophenolate mofetil, which led to the resolution of symptoms and nephrotic syndrome. This case highlights an uncommon sequence of events, with MN presenting before TA, underscoring the need to consider TA in differentials for patients with MN. Notably, this is the first reported case in a young female, emphasising the need for further understanding of TA-associated glomerular diseases. Additionally, the presence of THSD7A in MN, despite negative malignancy workup, is also noteworthy.

LEARNING POINTS

Membranous nephropathy (MN) and Takayasu arteritis (TA) have distinct clinical presentations; therefore, diagnosing coexisting MN and TA is challenging, which may lead to delayed diagnoses.A multidisciplinary approach with tailored treatments is essential for prompt diagnosis and optimal management.Comprehensive follow-up studies are vital to understand the pathogenesis of this rare amalgamation, refine targeted treatment strategies and potentially improve overall prognosis.

摘要

未标注

高安动脉炎(TA)主要导致缺血性肾硬化,但偶尔也可与肾小球病相关。我们报告一例二十多岁女性患者,患有对利妥昔单抗耐药的磷脂酶A2阴性、血小板反应蛋白7A阳性的膜性肾病(MN),该患者出现颈部疼痛和新发高血压。血液检查显示炎症标志物升高。头颈部影像学检查显示椎动脉局灶性扩张和不规则,符合TA表现。患者开始接受类固醇治疗,随后使用霉酚酸酯,症状和肾病综合征得以缓解。该病例突出了一种不常见的事件顺序,即MN先于TA出现,强调在MN患者的鉴别诊断中需要考虑TA。值得注意的是,这是首次报道的年轻女性病例,强调需要进一步了解TA相关的肾小球疾病。此外,尽管恶性肿瘤检查结果为阴性,但MN中存在血小板反应蛋白7A也值得关注。

学习要点

膜性肾病(MN)和高安动脉炎(TA)有不同的临床表现;因此,诊断同时存在的MN和TA具有挑战性,可能导致诊断延迟。采用量身定制治疗方案的多学科方法对于及时诊断和优化管理至关重要。全面的随访研究对于了解这种罕见合并症的发病机制、完善靶向治疗策略以及潜在改善总体预后至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eeec/11313112/d0f639f5e8ff/4557_Fig1.jpg

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