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“伟大模仿者”的诊断困境:狼疮累及心脏和大脑表现为双侧上下肢无力

The Diagnostic Dilemma of "The Great Imitator": Heart and Cerebral Involvement of Lupus Manifesting as Bilateral Upper and Lower Extremity Weakness.

作者信息

Santos Alexander, Kure Catrina, Sanchez Cesar, Gross Phillip

机构信息

Northeast Georgia Medical Center, Gainesville, USA.

出版信息

Case Rep Rheumatol. 2023 Oct 10;2023:6676357. doi: 10.1155/2023/6676357. eCollection 2023.

DOI:10.1155/2023/6676357
PMID:37854887
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10581839/
Abstract

BACKGROUND

Systemic lupus erythematous (SLE) is an autoimmune condition which can cause complex, multiorgan dysfunction. This autoimmune disease is caused by the production of antinuclear antibodies which allows this disease to target virtually any organ in the human body. When a patient experiences an unpredictable worsening of disease activity, it is generally considered a lupus flare. Organ dysfunction due to a lupus flare tends to manifest as separate events in the literature and rarely do we witness multiple compounding organ failures during a lupus flare. If we do witness organ dysfunction and failure, rarely do we see cardiac and cerebral involvement. Typically, patients take immunosuppressants for a long term to avoid the patient's disease process from worsening and to provide prophylaxis from a flare to occur. Despite the availability in preventive strategies, some patients will have increased disease activity multiple times throughout their lifetime and will need increases in their medication doses or changes to their regimen. Some flares can be managed in the clinic, but more severe ones may be life-threatening that they require intravenous medications and hospitalization to achieve remission. In the following case, we see a patient with a past medical history of SLE on multiple immunosuppressants who arrived at the hospital with acute, bilateral weakness of the upper and lower extremities. It was later determined via various imaging and laboratory testing that she was having an SLE flare that was directly causing myocarditis which progressed to global ischemia of the brain via myocardial hypoperfusion. She experienced substantial recovery from her flare with treatment with high-dose, intravenous corticosteroids. . A 27-year-old female with a 2-year history of lupus and a 1-week history of paroxysmal atrial fibrillation presented with three days of bilateral focal neurological deficits in the arms and legs. She was found to have ischemic cardiac and neurologic manifestations during her hospital stay.

CONCLUSION

Our patient presented with reversible focal neurological deficits, elevated high-sensitive troponin levels, and high lupus serum antibodies who showed significant improvement after the introduction of high-dose steroids. This case recommends keeping a large differential and to not discount patients' past comorbidities for causing atypical symptomatology.

摘要

背景

系统性红斑狼疮(SLE)是一种自身免疫性疾病,可导致复杂的多器官功能障碍。这种自身免疫性疾病是由抗核抗体的产生引起的,这使得该疾病几乎可以靶向人体的任何器官。当患者经历疾病活动不可预测的恶化时,通常被认为是狼疮发作。在文献中,狼疮发作导致的器官功能障碍往往表现为单独的事件,我们很少看到在狼疮发作期间出现多个复合器官衰竭。如果我们确实看到器官功能障碍和衰竭,很少会看到心脏和大脑受累。通常,患者长期服用免疫抑制剂以避免病情恶化,并预防发作。尽管有预防策略,但一些患者在其一生中会多次出现疾病活动增加,需要增加药物剂量或改变治疗方案。一些发作可以在门诊处理,但更严重的发作可能危及生命,需要静脉用药和住院治疗以实现缓解。在以下病例中,我们看到一名有SLE病史且正在服用多种免疫抑制剂的患者因急性双侧上下肢无力入院。后来通过各种影像学和实验室检查确定她正在经历一次SLE发作,该发作直接导致心肌炎,进而通过心肌灌注不足发展为全脑缺血。通过大剂量静脉注射皮质类固醇治疗,她的发作得到了显著恢复。一名有2年狼疮病史和1周阵发性房颤病史的27岁女性,出现双侧手臂和腿部局灶性神经功能缺损3天。在住院期间,她被发现有缺血性心脏和神经表现。

结论

我们的患者出现可逆性局灶性神经功能缺损、高敏肌钙蛋白水平升高和狼疮血清抗体升高,在引入大剂量类固醇后有显著改善。本病例建议保持广泛的鉴别诊断,不要忽视患者既往合并症导致非典型症状的可能性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/044f/10581839/c82e99bb28e6/CRIRH2023-6676357.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/044f/10581839/e39cfdb295a6/CRIRH2023-6676357.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/044f/10581839/c82e99bb28e6/CRIRH2023-6676357.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/044f/10581839/e39cfdb295a6/CRIRH2023-6676357.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/044f/10581839/c82e99bb28e6/CRIRH2023-6676357.002.jpg

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