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Volume 15
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Review

Cardiovascular Involvement in Erdheim–Chester Disease Presenting as Pericardial Tamponade: A Case Report and Multimodality Imaging Review

by
Francesco Di Spigno
1,†,
Andrea Faggiano
2,†,
Alfredo Ricchiuto
3,
Andrea Tedeschi
1,
Federico Breviario
1,
Luigi Gerra
1,
Piergiorgio Turchio
3,
Daniela Aschieri
1 and
Marco Sebastiani
2,4,*
1
Cardiology Department, Azienda USL Piacenza, Via Giuseppe Taverna 49, 29121 Piacenza, Italy
2
Rheumatology Unit, Azienda USL Piacenza, Via Giuseppe Taverna 49, 29121 Piacenza, Italy
3
Radiology Department, Azienda USL Piacenza, Via Giuseppe Taverna 49, 29121 Piacenza, Italy
4
Department of Medicine and Surgery, University of Parma, 43125 Parma, Italy
*
Author to whom correspondence should be addressed.
†
These authors contributed equally to this work.
J. Clin. Med. 2026, 15(12), 4650; https://doi.org/10.3390/jcm15124650 (registering DOI)
Submission received: 4 May 2026 / Revised: 30 May 2026 / Accepted: 9 June 2026 / Published: 15 June 2026
(This article belongs to the Section Immunology & Rheumatology)
Versions Notes

Abstract

Erdheim–Chester Disease (ECD) constitutes a rare and clinically heterogeneous non-Langerhans cell histiocytosis, characterized by the systemic infiltration of tissues by foamy, lipid-laden histiocytes. These cells typically exhibit an immunophenotypic profile positive for CD68 and negative for CD1a. The disease’s multifaceted presentation, which can span from isolated bone lesions to fulminant multi-organ failure, frequently results in considerable diagnostic delay. In this case-based review, we describe the case of a 58-year-old who presented with a primary complaint of exertional dyspnoea and fatigue. The initial diagnostic evaluation revealed a hemodynamically significant circumferential pericardial effusion and imaging findings suggestive of aortitis. Clinical presentation of ECD depends on the organs and tissues involved, and may range from bone pain to neurological symptoms, endocrine dysfunction, and cardiac involvement. Cardiovascular involvement occurs in at least 40% of ECD patients, although it is frequently underdiagnosed. Cardiac ECD is heterogeneous and may mimic many alternative aetiologies. The infiltration of the right atrioventricular sulcus, right atrial walls, or interatrial septum is one of the most typical cardiac manifestations of ECD. Recognition of pseudo-tumour intra-atrial mass, pericardial involvement, as well as the circumferential encasement of the entire aorta, the so-called coated aorta, are other frequent findings. Diagnosis often requires a multimodal approach, in particular when cardiac symptoms represent the onset of clinical manifestation of ECD. The combined use of computed tomography, fluorodeoxyglucose positron emission tomography, dedicated cardiac and abdominal magnetic resonance imaging, and X-ray of long bones can collectively reveal a constellation of findings diagnostic of ECD.
Keywords: Erdheim–Chester disease; histiocytosis; pericardial effusion; cardiac tamponade; coated aorta; multimodality imaging Erdheim–Chester disease; histiocytosis; pericardial effusion; cardiac tamponade; coated aorta; multimodality imaging

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MDPI and ACS Style

Di Spigno, F.; Faggiano, A.; Ricchiuto, A.; Tedeschi, A.; Breviario, F.; Gerra, L.; Turchio, P.; Aschieri, D.; Sebastiani, M. Cardiovascular Involvement in Erdheim–Chester Disease Presenting as Pericardial Tamponade: A Case Report and Multimodality Imaging Review. J. Clin. Med. 2026, 15, 4650. https://doi.org/10.3390/jcm15124650

AMA Style

Di Spigno F, Faggiano A, Ricchiuto A, Tedeschi A, Breviario F, Gerra L, Turchio P, Aschieri D, Sebastiani M. Cardiovascular Involvement in Erdheim–Chester Disease Presenting as Pericardial Tamponade: A Case Report and Multimodality Imaging Review. Journal of Clinical Medicine. 2026; 15(12):4650. https://doi.org/10.3390/jcm15124650

Chicago/Turabian Style

Di Spigno, Francesco, Andrea Faggiano, Alfredo Ricchiuto, Andrea Tedeschi, Federico Breviario, Luigi Gerra, Piergiorgio Turchio, Daniela Aschieri, and Marco Sebastiani. 2026. "Cardiovascular Involvement in Erdheim–Chester Disease Presenting as Pericardial Tamponade: A Case Report and Multimodality Imaging Review" Journal of Clinical Medicine 15, no. 12: 4650. https://doi.org/10.3390/jcm15124650

APA Style

Di Spigno, F., Faggiano, A., Ricchiuto, A., Tedeschi, A., Breviario, F., Gerra, L., Turchio, P., Aschieri, D., & Sebastiani, M. (2026). Cardiovascular Involvement in Erdheim–Chester Disease Presenting as Pericardial Tamponade: A Case Report and Multimodality Imaging Review. Journal of Clinical Medicine, 15(12), 4650. https://doi.org/10.3390/jcm15124650

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