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揭开无声风暴:系统性红斑狼疮中的狼疮性肾炎和脑血管炎

Silent Storm Unveiled: Lupus Nephritis and Cerebral Vasculitis in Systemic Lupus Erythematosus.

作者信息

Sawant Rucha, Gaidhane Shilpa A, Mahajan Vrushali, Chaudhari Pranav, Bedi Gautam N

机构信息

Internal Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education & Research, Wardha, IND.

School of Epidemiology and Public Health, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education & Research, Wardha, IND.

出版信息

Cureus. 2024 Apr 7;16(4):e57751. doi: 10.7759/cureus.57751. eCollection 2024 Apr.

DOI:10.7759/cureus.57751
PMID:38716007
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11074806/
Abstract

Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder known for its intricate clinical manifestations, spanning a spectrum of symptoms, including neuropsychiatric SLE (NPSLE) and lupus nephritis (LN). This condition predominantly affects young women of childbearing age, presenting a diverse array of symptoms that pose challenges in both diagnosis and treatment. Diagnosing central nervous system (CNS) involvement in SLE remains notably difficult despite being rooted in an autoimmune inflammatory response driven by cytokine surges. There exists no single definitive test for diagnosis, necessitating a thorough evaluation of clinical presentations, neurological indicators, and specific antibody detection. LN typically evades diagnosis until the patient progresses to a state of advanced renal insufficiency, demanding aggressive therapeutic interventions. In this discourse, we examine a case marked by generalized tonic-clonic seizures. While epilepsy might be initially suspected, in this instance, the underlying cause lay deeper, concealed within the complexities of autoimmune dysregulation. Additional symptoms included generalized edema, sun-exposed rash, oral ulcers, and recurrent fever over the past six months. The puzzle pieces eventually coalesced through meticulous examination of each clinical manifestation, coupled with laboratory analyses, neuroimaging studies, and renal biopsy, revealing a complex scenario of cerebral vasculitis concurrent with LN in a case of SLE.

摘要

系统性红斑狼疮(SLE)是一种慢性自身免疫性疾病,以其复杂的临床表现而闻名,涵盖一系列症状,包括神经精神性狼疮(NPSLE)和狼疮性肾炎(LN)。这种疾病主要影响育龄期年轻女性,呈现出各种症状,给诊断和治疗带来挑战。尽管中枢神经系统(CNS)受累是由细胞因子激增驱动的自身免疫性炎症反应所致,但诊断SLE中的CNS受累仍然非常困难。目前尚无单一的确诊检查,需要对临床表现、神经学指标和特定抗体检测进行全面评估。LN通常在患者发展到晚期肾功能不全状态之前难以诊断,需要积极的治疗干预。在本文中,我们研究了一例以全身性强直阵挛性癫痫发作为特征的病例。虽然最初可能怀疑是癫痫,但在这个病例中,潜在原因更深层次,隐藏在自身免疫失调的复杂性之中。其他症状包括全身性水肿、日晒后皮疹、口腔溃疡以及过去六个月内反复发热。通过对每个临床表现进行细致检查,结合实验室分析、神经影像学研究和肾活检,这些拼图最终拼凑在一起,揭示了一例SLE患者中并发脑血管炎和LN的复杂情况。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c61/11074806/4359f40c66bd/cureus-0016-00000057751-i07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c61/11074806/2d1f5249325b/cureus-0016-00000057751-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c61/11074806/1f4177a30f9c/cureus-0016-00000057751-i02.jpg
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https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c61/11074806/e7412ed81273/cureus-0016-00000057751-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c61/11074806/5293251236b9/cureus-0016-00000057751-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c61/11074806/4359f40c66bd/cureus-0016-00000057751-i07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c61/11074806/2d1f5249325b/cureus-0016-00000057751-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c61/11074806/1f4177a30f9c/cureus-0016-00000057751-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c61/11074806/6f6a516e8f7e/cureus-0016-00000057751-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c61/11074806/1ff0155e05fe/cureus-0016-00000057751-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c61/11074806/e7412ed81273/cureus-0016-00000057751-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c61/11074806/5293251236b9/cureus-0016-00000057751-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2c61/11074806/4359f40c66bd/cureus-0016-00000057751-i07.jpg

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Neurological Manifestations of Connective Tissue Disorders.结缔组织病的神经表现
Cureus. 2023 Oct 16;15(10):e47108. doi: 10.7759/cureus.47108. eCollection 2023 Oct.
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