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混合性结缔组织病患者的继发性心脏淀粉样变性:一例报告。

Secondary cardiac amyloidosis in a patient with mixed connective tissue disease: A case report.

作者信息

Khanal Ujjwal Prakash, Ghimire Prinska, Shahi Tejash, Dhakal Tulsi Ram, Jha Saket

机构信息

Institute of Medicine, Tribhuwan University Teaching Hospital Kathmandu Nepal.

出版信息

Clin Case Rep. 2023 Jul 6;11(7):e07669. doi: 10.1002/ccr3.7669. eCollection 2023 Jul.

Abstract

We report the case of a 62-year-old man who presented with shortness of breath, cough, bilateral lower limbs' swelling, and blackish discoloration of multiple fingertips over the past 2 months. Anti-Ribonucleoprotein antibodies were found to be present, and gadolinium-based cardiac MRI showed non-vascular subendocardial enhancement with diffuse symmetrical thickening of the left ventricular wall. A diagnosis of Mixed connective tissue disease with secondary cardiac amyloidosis was thus made, and the patient was successfully managed with intravenous cyclophosphamide, corticosteroids, and other supportive measures. Although extremely rare, this case shows that secondary cardiac amyloidosis should be considered while managing patients with MCTD.

摘要

我们报告了一例62岁男性患者,在过去2个月中出现呼吸急促、咳嗽、双侧下肢肿胀以及多个指尖发黑变色的症状。发现存在抗核糖核蛋白抗体,基于钆的心脏磁共振成像显示非血管性心内膜下强化,伴有左心室壁弥漫性对称性增厚。因此诊断为混合性结缔组织病伴继发性心脏淀粉样变性,患者通过静脉注射环磷酰胺、皮质类固醇及其他支持措施得到成功治疗。尽管极为罕见,但该病例表明在治疗混合性结缔组织病患者时应考虑继发性心脏淀粉样变性。

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[Late gadolinium enhancement and T1 mapping for the diagnosis of cardiac amyloidosis].[延迟钆增强及T1成像在心脏淀粉样变性诊断中的应用]
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本文引用的文献

3
Cardiac amyloidosis.心脏淀粉样变性。
Clin Med (Lond). 2018 Apr 1;18(Suppl 2):s30-s35. doi: 10.7861/clinmedicine.18-2-s30.
7
Systemic amyloidosis.系统性淀粉样变性。
Lancet. 2016 Jun 25;387(10038):2641-2654. doi: 10.1016/S0140-6736(15)01274-X. Epub 2015 Dec 21.
8
The diagnosis and classification of mixed connective tissue disease.混合性结缔组织病的诊断与分类。
J Autoimmun. 2014 Feb-Mar;48-49:46-9. doi: 10.1016/j.jaut.2014.01.008. Epub 2014 Jan 22.

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