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

Heart Surg Forum. 2006;9(1):E549-54. doi: 10.1532/HSF98.20051109.

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.

Publication types

  • Case Reports

MeSH terms

  • 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*

Substances

  • Cyclooxygenase 2 Inhibitors
  • Pyrazoles
  • Sulfonamides
  • Celecoxib