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探索IgA血管炎的诊断历程

Exploring the Diagnostic Odyssey of IgA Vasculitis.

作者信息

Rosmaninho Irene, Simão-Parreira Beatriz, Leal Carolina, Cardoso Leila, Almeida Jorge

机构信息

Internal Medicine Department, Unidade Local de Saúde de São João, Porto, PRT.

Anatomopathology Department, Unidade Local de Saúde de São João, Porto, PRT.

出版信息

Cureus. 2024 Aug 30;16(8):e68170. doi: 10.7759/cureus.68170. eCollection 2024 Aug.

DOI:10.7759/cureus.68170
PMID:39347365
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11439127/
Abstract

IgA vasculitis is a form of small vessel vasculitis characterized by IgA immune complex deposition. Although primarily affecting children, it can also occur in adults, often with more severe manifestations and a higher risk of chronic kidney disease (CKD). The authors present the case of a 77-year-old man with significant cardiovascular risk and atrial fibrillation who was admitted to the emergency department (ED) with pruritic and painful palpable purpuric rash, weight loss, and asthenia persisting for approximately one month prior to presentation. Laboratory findings revealed normocytic normochromic anemia, worsening renal function, elevated inflammatory markers, and leukoerythrocyturia. Initially diagnosed as infectious purpura associated with urinary tract infection, the patient was discharged with a prescription for antibiotics (cefixime). Subsequent worsening of the skin lesions, constitutional symptoms, and gross hematuria prompted a second visit to the ED, and the persistent deterioration of kidney function and inflammatory parameters led to admission for further investigation and consideration of a kidney biopsy. This case report describes the etiological investigation, providing a brief review of the typical characteristics of this disease and highlighting the importance of certain factors in establishing the diagnosis, notably the need and timing of a biopsy of the affected organ for a definitive diagnosis. Additionally, the clinical case underscores the diagnostic challenge, particularly when histological confirmation is elusive.

摘要

IgA血管炎是一种以IgA免疫复合物沉积为特征的小血管炎。虽然主要影响儿童,但也可发生于成人,通常表现更为严重,慢性肾脏病(CKD)风险更高。作者报告了一例77岁男性病例,该患者有显著心血管风险且患有心房颤动,因瘙痒性和疼痛性可触及紫癜性皮疹、体重减轻和乏力在就诊前持续约1个月而入住急诊科(ED)。实验室检查发现正细胞正色素性贫血、肾功能恶化、炎症标志物升高以及白细胞红细胞尿。最初诊断为与尿路感染相关的感染性紫癜,患者出院时开具了抗生素(头孢克肟)处方。随后皮肤病变、全身症状和肉眼血尿恶化促使患者再次前往急诊科就诊,肾功能和炎症参数持续恶化导致其住院进一步检查并考虑进行肾活检。本病例报告描述了病因学调查,简要回顾了该疾病的典型特征,并强调了某些因素在确立诊断中的重要性,特别是对受累器官进行活检以明确诊断的必要性和时机。此外,该临床病例凸显了诊断挑战,尤其是在难以获得组织学确诊时。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2fc8/11439127/7665bfc705f3/cureus-0016-00000068170-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2fc8/11439127/f79cebda650f/cureus-0016-00000068170-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2fc8/11439127/905868f2eef6/cureus-0016-00000068170-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2fc8/11439127/4ca855795a6c/cureus-0016-00000068170-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2fc8/11439127/7665bfc705f3/cureus-0016-00000068170-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2fc8/11439127/f79cebda650f/cureus-0016-00000068170-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2fc8/11439127/905868f2eef6/cureus-0016-00000068170-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2fc8/11439127/4ca855795a6c/cureus-0016-00000068170-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2fc8/11439127/7665bfc705f3/cureus-0016-00000068170-i04.jpg

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本文引用的文献

1
IgA Nephropathy: An Interesting Autoimmune Kidney Disease.IgA 肾病:一种有趣的自身免疫性肾脏疾病。
Am J Med Sci. 2021 Feb;361(2):176-194. doi: 10.1016/j.amjms.2020.10.003. Epub 2020 Oct 8.
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Henoch-Schönlein Purpura (IgA Vasculitis): Rapid Evidence Review.过敏性紫癜(IgA 血管炎):快速证据回顾。
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Henoch-Schönlein Purpura: A Literature Review.过敏性紫癜:文献综述。
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IgA vasculitis in adults: the performance of the EULAR/PRINTO/PRES classification criteria in adults.成人IgA血管炎:欧洲抗风湿病联盟/儿童风湿病国际试验组织/儿科风湿病学会成人分类标准的性能
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IgA vasculitis (formerly Henoch-Schönlein purpura) in an adult with systemic lupus erythematosus.一名患有系统性红斑狼疮的成年人的IgA血管炎(原称过敏性紫癜)。
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