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新诊断的系统性红斑狼疮合并灾难性抗磷脂综合征中的广泛血栓形成:一例报告

Extensive Thrombosis in Catastrophic Antiphospholipid Syndrome in a Newly Diagnosed Systemic Lupus Erythematosus: A Case Report.

作者信息

Tanariyakul Manasawee, Nebrajas Kevin, Saowapa Sakditad, Polpichai Natchaya

机构信息

Internal Medicine, University of Hawaii John A. Burns School of Medicine, Honolulu, USA.

Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, USA.

出版信息

Cureus. 2024 May 2;16(5):e59542. doi: 10.7759/cureus.59542. eCollection 2024 May.

Abstract

In this case report, we present the development of catastrophic antiphospholipid syndrome (CAPS), a rare and potentially fatal consequence of systemic lupus erythematosus (SLE), in a 33-year-old Micronesian woman. CAPS is characterized by extensive arterial thrombosis and multiorgan failure. The patient first showed signs of neuropsychiatric symptoms, brain infarctions on imaging, and severe hypoxic respiratory failure brought into the hospital by diffuse alveolar hemorrhage (DAH) along with lupus nephritis (LN). Blood urea nitrogen (BUN) and creatinine (Cr) were progressively elevated to 102/4.1 mg/dL, respectively. A urinalysis revealed microscopic hematuria and proteinuria with a urine protein/creatinine ratio of 6052 mg/g. She was also found to have had microangiopathic hemolytic anemia (MAHA) and severe venous thrombosis, both of which were indicative of CAPS. An aggressive approach, including immunosuppressive medication, therapeutic plasma exchange, and anticoagulation, had positive results, including renal recovery and the cessation of thrombotic episodes. This instance highlights how crucial it is to identify CAPS patients early and take appropriate action to improve patient outcomes for this difficult and sometimes deadly disorder.

摘要

在本病例报告中,我们呈现了一名33岁密克罗尼西亚女性发生灾难性抗磷脂综合征(CAPS)的情况,CAPS是系统性红斑狼疮(SLE)一种罕见且可能致命的后果。CAPS的特征为广泛的动脉血栓形成和多器官功能衰竭。该患者最初表现出神经精神症状、影像学检查显示脑梗死,以及因弥漫性肺泡出血(DAH)伴狼疮性肾炎(LN)导致的严重低氧性呼吸衰竭而入院。血尿素氮(BUN)和肌酐(Cr)分别逐渐升高至102/4.1mg/dL。尿液分析显示镜下血尿和蛋白尿,尿蛋白/肌酐比值为6052mg/g。还发现她患有微血管病性溶血性贫血(MAHA)和严重静脉血栓形成,这两者均提示CAPS。包括免疫抑制药物、治疗性血浆置换和抗凝在内的积极治疗方法取得了积极效果,包括肾功能恢复和血栓形成发作停止。这个病例突出了早期识别CAPS患者并采取适当行动以改善这种疑难且有时致命疾病患者预后的重要性。

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