AI Article Synopsis
- Erdheim-Chester's disease is a rare condition characterized by xanthogranulomatosis that primarily affects the skeletal system but can also involve soft tissues.
- A unique case is presented involving a cardiac variant that includes pericardial effusion and simultaneous orbital manifestations.
- The study outlines a diagnostic approach and suggests using nonsteroidal anti-inflammatory drugs (NSAIDs) as a potential treatment, exploring the role of cyclo-oxygenase in the disease process.
Article Abstract
Erdheim-Chester's disease is a rare multisystem xanthogranulomatosis, afflicting the skeletal system with the occasional involvement of soft tissues. We delineate an unusual case of a cardiac variant of Erdheim-Chester's disease presenting with pericardial effusion and as a collision with a synchronous orbital manifestation. We describe our diagnostic pathway and propose a novel treatment option involving nonsteroidal anti-inflammatory drugs. The role of cyclo-oxygenase in the disease process and inhibition thereof by NSAIDs is hypothesized and discussed.
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| http://dx.doi.org/10.1532/HSF98.20051109 | DOI Listing |
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Article Synopsis
- - Erdheim-Chester Disease is a rare inflammatory myeloid neoplasm primarily affecting men over 50 years old, though its exact incidence is unknown.
- - It often presents as a multisystemic condition, impacting the skeletal system in 90% of patients, with genitourinary issues in 60% and central nervous system involvement in 25%.
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