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一例隐匿性快速进展性肾小球肾炎继发于寡免疫性新月体性肾小球肾炎

An Insidious Case of Rapidly Progressive Glomerulonephritis Secondary to Pauci-Immune Crescentic Glomerulonephritis.

作者信息

Mavratsas Vasilis C, Vu Lan, Yeh Owen L

机构信息

Internal Medicine, University of Texas Medical Branch, Galveston, USA.

出版信息

Cureus. 2024 Nov 7;16(11):e73233. doi: 10.7759/cureus.73233. eCollection 2024 Nov.

DOI:10.7759/cureus.73233
PMID:39650915
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11624910/
Abstract

Rapidly progressive glomerulonephritis (RPGN) is a severe type of nephritic syndrome that involves progressive loss of kidney function and can lead to significant morbidity and mortality. RPGN has many etiologies, of which pauci-immune crescenting glomerulonephritis (PICGN) is the most common; however, patients often present with nonspecific symptoms, which can lead to a delay in diagnosis and treatment. We describe one such case that was correctly identified only after multiple clinic and hospital encounters. Following the correct diagnosis, the patient was started on induction therapy with corticosteroids and rituximab with plans for outpatient maintenance therapy. We have included a discussion of the pathogenesis, epidemiology, diagnosis and treatment of RPGN, highlighting the importance of early recognition of the disease in achieving better patient outcomes.

摘要

快速进展性肾小球肾炎(RPGN)是一种严重的肾病综合征类型,涉及肾功能的进行性丧失,并可导致显著的发病率和死亡率。RPGN有多种病因,其中寡免疫性新月体性肾小球肾炎(PICGN)最为常见;然而,患者常表现为非特异性症状,这可能导致诊断和治疗延迟。我们描述了这样一个病例,该病例在多次门诊和住院就诊后才得到正确诊断。确诊后,患者开始接受糖皮质激素和利妥昔单抗诱导治疗,并计划进行门诊维持治疗。我们还讨论了RPGN的发病机制、流行病学、诊断和治疗,强调了早期识别该疾病对改善患者预后的重要性。

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An Insidious Case of Rapidly Progressive Glomerulonephritis Secondary to Pauci-Immune Crescentic Glomerulonephritis.一例隐匿性快速进展性肾小球肾炎继发于寡免疫性新月体性肾小球肾炎
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本文引用的文献

1
Classification Criteria for ANCA Associated Vasculitis - Ready for Prime Time?抗中性粒细胞胞浆抗体相关血管炎的分类标准——准备好投入实际应用了吗?
Curr Rheumatol Rep. 2024 Sep;26(9):332-342. doi: 10.1007/s11926-024-01154-9. Epub 2024 Jun 24.
2
Diagnosis and management of ANCA-associated vasculitis.抗中性粒细胞胞质抗体相关性血管炎的诊断与治疗。
Lancet. 2024 Feb 17;403(10427):683-698. doi: 10.1016/S0140-6736(23)01736-1.
3
Glomerulonephritis: immunopathogenesis and immunotherapy.肾小球肾炎:免疫发病机制与免疫治疗。
Nat Rev Immunol. 2023 Jul;23(7):453-471. doi: 10.1038/s41577-022-00816-y. Epub 2023 Jan 12.
4
Acute glomerulonephritis.急性肾小球肾炎
Lancet. 2022 Apr 23;399(10335):1646-1663. doi: 10.1016/S0140-6736(22)00461-5.
5
Early Renal Recovery after the First Flare of Pauci-Immune Glomerulonephritis.
Am J Nephrol. 2022;53(1):59-68. doi: 10.1159/000520285. Epub 2022 Jan 17.
6
Avacopan for the Treatment of ANCA-Associated Vasculitis. Reply.阿伐可泮用于治疗抗中性粒细胞胞浆抗体相关性血管炎。回复。
N Engl J Med. 2021 May 27;384(21):e81. doi: 10.1056/NEJMc2104672.
7
ANCA-Associated Vasculitis: An Update.抗中性粒细胞胞浆抗体相关血管炎:最新进展
J Clin Med. 2021 Apr 1;10(7):1446. doi: 10.3390/jcm10071446.
8
Diagnostic delays in vasculitis and factors associated with time to diagnosis.血管炎的诊断延误及与诊断时间相关的因素。
Orphanet J Rare Dis. 2021 Apr 21;16(1):184. doi: 10.1186/s13023-021-01794-5.
9
Glomerulonephritis in AKI: From Pathogenesis to Therapeutic Intervention.急性肾损伤中的肾小球肾炎:从发病机制到治疗干预
Front Med (Lausanne). 2021 Mar 2;7:582272. doi: 10.3389/fmed.2020.582272. eCollection 2020.
10
Nephritic Syndrome.肾炎综合征
Prim Care. 2020 Dec;47(4):615-629. doi: 10.1016/j.pop.2020.08.003. Epub 2020 Sep 25.