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抗RNA聚合酶III型硬皮病患者发生心脏压塞的罕见病例。

A Rare Case of Cardiac Tamponade in a Patient With Anti-RNA Polymerase III Scleroderma.

作者信息

Schneider Artur, Jhawar Nikita, Balistreri Lisa, Chirila Razvan, Berianu Florentina

机构信息

Internal Medicine, Mayo Clinic, Jacksonville, USA.

Rheumatology, Mayo Clinic, Jacksonville, USA.

出版信息

Cureus. 2023 May 4;15(5):e38533. doi: 10.7759/cureus.38533. eCollection 2023 May.

Abstract

Scleroderma is a chronic, progressive autoimmune disease that often presents with multiorgan involvement. Cardiac manifestations are common and include microvascular coronary artery disease, conduction abnormalities, autonomic insufficiency, and pericardial effusions. Although rare, pericardial effusions may progress and lead to cardiac tamponade. Patients diagnosed with scleroderma can be further prognosticated based on the presence of serologic scleroderma-specific antibodies. The anti-RNA polymerase III autoantibody (anti-RNAP3) is associated with an aggressive subtype of scleroderma. Looking at the current literature, no association has been reported between anti-RNAP3 and the development of cardiac tamponade in patients with underlying scleroderma. We discuss a unique case of a patient with scleroderma who was found to be anti-RNAP3 positive and signs of cardiac tamponade. This case illustrates the importance of an expeditious diagnosis and timely interventions to treat cardiac tamponade. Additionally, we share a rare but important association between anti-RNAP3 and the formation of tamponade physiology in scleroderma.

摘要

硬皮病是一种慢性、进行性自身免疫性疾病,常累及多个器官。心脏表现较为常见,包括微血管性冠状动脉疾病、传导异常、自主神经功能不全和心包积液。心包积液虽罕见,但可能进展并导致心脏压塞。根据血清学硬皮病特异性抗体的存在情况,可对诊断为硬皮病的患者进行进一步预后评估。抗RNA聚合酶III自身抗体(抗RNAP3)与硬皮病的一种侵袭性亚型相关。查阅当前文献,尚未有关于抗RNAP3与潜在硬皮病患者心脏压塞发生之间关联的报道。我们讨论了一例硬皮病患者的独特病例,该患者抗RNAP3呈阳性且出现了心脏压塞的体征。该病例说明了快速诊断和及时干预治疗心脏压塞的重要性。此外,我们分享了抗RNAP3与硬皮病中压塞生理形成之间罕见但重要的关联。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b1c5/10239203/0a14b2cbe45c/cureus-0015-00000038533-i01.jpg

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