Erdheim-Chester Disease With Extensive Pericardial Involvement: A Case Report and Systematic Review

Area of Science:

• Cardiovascular Medicine
• Oncology
• Rare Diseases

Background:

• Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis.
• It involves systemic xanthogranulomatous infiltration.
• Cardiac involvement is a known, though less common, manifestation.

Observation:

• This article details a case of a female adult with Erdheim-Chester disease presenting with pericardial effusion.
• Pericardial effusion represents the most frequent cardiac manifestation of ECD.
• The case highlights the clinical presentation of cardiac involvement in ECD.

Findings:

• The study observed that pericardial infiltration is the most common cardiac manifestation in Erdheim-Chester disease.
• Analysis revealed that the majority of patients diagnosed with pericardial infiltration associated with ECD required a cardiovascular procedure.
• This indicates a significant need for cardiac intervention in affected individuals.

Implications:

• Early recognition of cardiac manifestations, particularly pericardial effusion, is crucial for managing Erdheim-Chester disease.
• The high rate of required cardiovascular procedures underscores the importance of cardiac monitoring and intervention strategies.
• Further research into the pathogenesis and optimal treatment of cardiac ECD is warranted.