Rapid progression to cardiac tamponade in Erdheim–Chester disease despite treatment with interferon alpha. Issue 4 (3rd July 2016)

• Record Type: Journal Article
• Authors: Nakhleh, Afif; Slobodin, Gleb; Elias, Nizar; Bejar, Jacob; Odeh, Majed
• Is Part Of: Modern rheumatology. Volume 26:Issue 4(2016)
• Page Start: 621
• Page End: 624
• Publication Date: 2016-07-03
• Subjects: Erdheim–Chester disease -- Pericarditis tamponade -- Interferon alpha; Rheumatology -- Periodicals; 616.723005
• DOI: 10.3109/14397595.2014.905235
• Languages: English
• ISSNs: 1439-7595

Abstract

Abstract : Erdheim–Chester disease (ECD) is a rare form of non-Langerhans histiocytosis with heterogeneous clinical manifestations. The most common presentation is bone pains typically involving the long bones. Approximately 75% of the patients develop extraskeletal involvement. Cardiac involvement is seen in up to 45% of the patients, and although, pericardial involvement is the most common cardiac pathology of this rare disease, cardiac tamponade due to ECD has been very rarely reported. We describe a case of a patient found to have ECD with multi-organ involvement and small pericardial effusion, which progressed to cardiac tamponade despite treatment with interferon alpha.