Recurrent pericardial effusion and tamponade in a patient with Erdheim-Chester disease (ECD)

BMJ Case Rep. 2015 Nov 24:2015:bcr2015212483. doi: 10.1136/bcr-2015-212483.

Abstract

Erdheim-Chester disease (ECD) is a rare xanthogranulomatous disorder characterised by the proliferation of lipid laden histiocytes along with infiltration of various organs of the body. Although commonly presenting with bone pains secondary to bony infiltration, cardiac involvement in the form of periaortic fibrosis and pericardial involvement may be seen in a subgroup of patients. We report a case of ECD presenting as recurrent pericardial effusion along with pericardial tamponade.

Publication types

  • Case Reports

MeSH terms

  • Brain / pathology
  • Cardiac Tamponade / complications
  • Cardiac Tamponade / diagnostic imaging*
  • Cardiac Tamponade / drug therapy
  • Diagnosis, Differential
  • Erdheim-Chester Disease / complications
  • Erdheim-Chester Disease / diagnosis*
  • Erdheim-Chester Disease / drug therapy
  • Fatal Outcome
  • Femur / pathology
  • Glucocorticoids / therapeutic use
  • Humans
  • Interferons / therapeutic use
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Pericardial Effusion / complications
  • Pericardial Effusion / diagnostic imaging*
  • Pericardial Effusion / drug therapy
  • Recurrence
  • Ribs / pathology
  • Tomography, X-Ray Computed

Substances

  • Glucocorticoids
  • Interferons