Erdheim-Chester's Disease of the Heart: A Diagnostic Conundrum and Collision with the Same Mass in the Orbit

Authors

  • Gábor Bogáts
  • Györgyi Piros
  • László Tiszlavicz
  • Béla Iványi
  • Viktor Sasi
  • Viktor Sasi
  • Judit Simon
  • Barna Babik
  • Andrea Csillik
  • Lilla Kardos
  • András Palkó
  • Keyvan Matin
  • Zoltán Hanzély
  • Katalin Korányi
  • István Nyáry
  • Mihály Végh
  • Lajos Kolozsvári
  • Zsuzsanna Kahán
  • András Bajcsay
  • Attila Tóth
  • György Balázs
  • Tamás Simor
  • László Pávics
  • András Palotás

DOI:

https://doi.org/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.

References

Chester W. 1930. Über lipoidgranulomatose. Virchows Arch Pathol Anat 279:561-602.nCline MJ. 1994. Histiocytes and histiocytosis. Blood 84:2840-53.nDevouassoux G, Lantuejoul S, Chatelain P, Brambilla E, Brambilla C. 1998. Erdheim-Chester disease: a primary macrophage cell disorder. Am J Respir Crit Care Med 157:650-3.nKenn W, Stäbler A, Zachoval R, Zietz C, Raum W, Wittenberg G. 1999. Erdheim-Chester disease: a case report and a literature overview. Eur Radiol 9:153-8.nPetrikowski CG, McGaw WT. 2000. Erdheim-Chester disease of the jaws: literature review and case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 90:389-98.nSerratrice J, Granel B, De Roux C, et al. "Coated aorta": a new sign of Erdheim-Chester disease. J Rheumatol 27:1550-3.nHaroche J, Amoura Z, Dion E, et al. 2004. Cardiovascular involvement, an overlooked feature of Erdheim-Chester disease: report of 6 new cases and a literature review. Medicine (Baltimore) 83:371-92.nVeyssier-Belot C, Cacoub P, Caparros-Lefebvre D, et al. 1996. Erdheim-Chester disease: clinical and radiologic characteristics of 59 cases. Medicine (Baltimore) 75:157-69.nShiff SJ, Rigas B. 1999. The role of cyclooxygenase inhibition in the anti-neoplastic effects of nonsteroidal anti-inflammatory drugs (NSAIDs). J Exp Med 190:445-50.n

Published

2006-01-09

How to Cite

Bogáts, G., Piros, G., Tiszlavicz, L., Iványi, B., Sasi, V., Sasi, V., Simon, J., Babik, B., Csillik, A., Kardos, L., Palkó, A., Matin, K., Hanzély, Z., Korányi, K., Nyáry, I., Végh, M., Kolozsvári, L., Kahán, Z., Bajcsay, A., Tóth, A., Balázs, G., Simor, T., Pávics, L., & Palotás, A. (2006). Erdheim-Chester’s Disease of the Heart: A Diagnostic Conundrum and Collision with the Same Mass in the Orbit. The Heart Surgery Forum, 9(1), E549-E554. https://doi.org/10.1532/HSF98.20051109

Issue

Section

Article