Erdheim-Chester's disease of the heart: a diagnostic conundrum and collision with the same mass in the orbit

• Published in: The Heart surgery forum Vol. 9; no. 1; p. E549
• Main Authors: Bogáts, Gábor, Piros, Györgyi, Tiszlavicz, László, Iványi, Béla, Sasi, Viktor, Csepregil, László, Simon, Judit, Babik, Barna, Csillik, Andrea, Kardos, Lilla, Palkó, András, Matin, Keyvan, Hanzély, Zoltán, Korányi, Katalin, Nyáry, István, Végh, Mihály, Kolozsvári, Lajos, Kahán, Zsuzsanna, Bajcsay, András, Tóth, Attila, Balázs, György, Simor, Tamás, Pávics, László, Palotás, András
• Format: Journal Article
• Language: English
• Published: United States 01-01-2006
• Subjects: Celecoxib; Cyclooxygenase 2 Inhibitors - therapeutic use; Erdheim-Chester Disease - diagnosis; Erdheim-Chester Disease - drug therapy; Heart Diseases - diagnosis; Heart Diseases - drug therapy; Humans; Male; Middle Aged; Orbital Diseases - diagnosis; Orbital Diseases - drug therapy; Pyrazoles - therapeutic use; Sulfonamides - therapeutic use
• ISSN: 1522-6662
• DOI: 10.1532/HSF98.20051109

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.