Bibliographic Details
| Title: |
Erdheim-Chester Disease presenting with constrictive pericarditis: A case report and review of the literature |
| Authors: |
Ge Guo, MD, Danfeng Zheng, MD, Xiaohua Wang, MD, Xinyu Wang, MD |
| Source: |
Radiology Case Reports, Vol 19, Iss 7, Pp 2590-2595 (2024) |
| Publisher Information: |
Elsevier, 2024. |
| Publication Year: |
2024 |
| Collection: |
LCC:Medical physics. Medical radiology. Nuclear medicine |
| Subject Terms: |
Erdheim-Chester Disease, Constrictive pericarditis, Histiocytosis, Multisystemic involvement, Imaging modalities, Interferon-α2b, Medical physics. Medical radiology. Nuclear medicine, R895-920 |
| More Details: |
Erdheim-Chester Disease (ECD) is a rare form of histiocytosis characterized by xanthomatous infiltration of affected organs. We present a case of a 62-year-old man with ECD initially presenting with constrictive pericarditis. Comprehensive imaging revealed systemic involvement, including the skeleton, orbit, pituitary, lung, kidney, and retroperitoneum, despite the absence of related symptoms. The diagnosis of ECD was eventually confirmed through histopathological evidence from a CT-guided biopsy. The patient responded well to interferon-α2b treatment, with gradual symptom amelioration and improvement in imaging and laboratory findings over a 5-month follow-up period. This case highlights the importance of considering ECD in the differential diagnosis of constrictive pericarditis and the utility of multimodal imaging for accurate diagnosis and management of this rare disease. The patient's positive response to treatment also highlights the potential for effective management of ECD, particularly with early diagnosis and intervention. |
| Document Type: |
article |
| File Description: |
electronic resource |
| Language: |
English |
| ISSN: |
1930-0433 |
| Relation: |
http://www.sciencedirect.com/science/article/pii/S1930043324001845; https://doaj.org/toc/1930-0433 |
| DOI: |
10.1016/j.radcr.2024.02.087 |
| Access URL: |
https://doaj.org/article/0c4724d67b724a339b841fd28db710fa |
| Accession Number: |
edsdoj.0c4724d67b724a339b841fd28db710fa |
| Database: |
Directory of Open Access Journals |
