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Erdheim-Chester's disease of the heart: a diagnostic conundrum and collision with the same mass in the orbit
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
Affiliation:Division of Cardiac Surgery, Center for Cardiology, Faculty of Medicine, University of Szeged, Szeged, Hungary.
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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