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心力衰竭作为严重非心脏疾病的首发表现。

Heart failure as the first manifestation of severe non-cardiac disease.

作者信息

Matuszna Anna, Meryn Jakob, Bachorski Witold

机构信息

Student Scientific Circle, 1st Department of Cardiology, Medical University of Gdansk, Poland.

出版信息

Pol Merkur Lekarski. 2019 Aug 30;47(278):67-69.

Abstract

UNLABELLED

Heart failure in patients with rheumatoid arthritis (RA) caused by secondary amyloidosis is now extremely rare.

A CASE REPORT

A 42 year old female patient with rheumatoid arthritis was admitted to our cardiology unit to diagnose and find the cause of her heart failure. Echocardiography showed marked diastolic dysfunction, hypertrophic cardiomyopathy and global longitudinal strain characteristic for cardiac amyloidosis. However, the suspicion of secondary amyloidosis related to her RA was excluded based on negative results of anti-SAA test. Cardiac MRI showed typical changes for cardiac amyloidosis in agreement with the primary echocardiography. Measurement of serum free light chain ratio revealed pattern typical for light chain amyloidosis secondary to multiple myoloma, confirmed by plasmocytosis on bone marrow biopsy and histopathology of salivary gland.

CONCLUSIONS

In patients with cardiac amyloidosis, despite strong clinical suggestions, the definite diagnosis should be always established because it may allow to implement effective treatment.

摘要

未标注

类风湿关节炎(RA)患者因继发性淀粉样变性导致的心力衰竭现在极为罕见。

病例报告

一名42岁的类风湿关节炎女性患者入住我们的心脏病科,以诊断并找出其心力衰竭的病因。超声心动图显示出明显的舒张功能障碍、肥厚型心肌病以及心脏淀粉样变性的整体纵向应变特征。然而,基于抗SAA检测结果为阴性,排除了与她的类风湿关节炎相关的继发性淀粉样变性的怀疑。心脏磁共振成像显示出与初步超声心动图一致的心脏淀粉样变性的典型变化。血清游离轻链比值测定显示出多发性骨髓瘤继发的轻链淀粉样变性的典型模式,骨髓活检中的浆细胞增多症和唾液腺组织病理学证实了这一点。

结论

对于心脏淀粉样变性患者,尽管有强烈的临床提示,但仍应始终确立明确诊断,因为这可能有助于实施有效治疗。

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