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

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 effu...

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Detalles Bibliográficos
Autores principales: Schneider, Artur, Jhawar, Nikita, Balistreri, Lisa, Chirila, Razvan, Berianu, Florentina
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10239203/
https://www.ncbi.nlm.nih.gov/pubmed/37273403
http://dx.doi.org/10.7759/cureus.38533
Descripción
Sumario: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.