title: [Recurrent pericardial effusion as first manifestation of Erdheim-Chester disease].
abstract: METHODS
  A 65-year-old woman presented with reduced general condition and dyspnoea that was progressive over the last months. Clinical findings revealed an exophthalmus on the right, xanthelasm and mild peripheral oedema. Previously, a pericardiocentesis had been performed due to a large pericardial effusion. A previous CT scan showed a mass attached to the pericardium extending through the atrio-ventricular groove and a thickened aorta. In addition, a retroperitoneal fibrosis and an occlusion of both Aa. iliacae internae were found.
  
  
  METHODS
  The ECG showed sinus rhythm. Laboratory findings demonstrated a microcytic anemia and a renal failure. Chest radiography showed a large cardiac silhouette, while the transthoracic echocardiography revealed a recurrent large pericardial effusion. A PET/CT scan of the chest and abdomen showed a tissue infiltration of the retroperitoneal structures, a mass surrounding the right coronary artery and the right orbita. Finally, a femur biopsy confirmed the diagnosis of Erdheim-Chester disease.
  
  
  METHODS
  With the diagnosis Erdheim-Chester disease we started a high dose immunsuppressive therapy using glucocorticoids and interferon-a. Tumour size slightly decreased during the following 2 months, however the patient developed a severe urosepsis and died from multiorgan failure.
  
  
  CONCLUSIONS
  We report a case of an Erdheim-Chester disease with cardiovascular involvement primarily diagnosed due to a recurrent large pericardial effusion. In case of cardial tumors with interatrial septum or coronary artery involvement together with cerebral manifestations, an Erdheim-Chester disease should be taken into account.
authors.affiliation: Abteilung für Endokrinologie, Diabetes, Nephrologie, Angiologie und Klinische Chemie, Medizinische Universitätsklinik, Eberhard-Karls-Universität, Tübingen. Stefan.Lutz@med.uni-tuebingen.de
authors.forename: S Z
authors.initials: SZ
authors.lastname: Lutz
authors.forename: M
authors.initials: M
authors.lastname: Schmalzing
authors.forename: J
authors.initials: J
authors.lastname: Vogel-Claussen
authors.forename: P
authors.initials: P
authors.lastname: Adam
authors.forename: A E
authors.initials: AE
authors.lastname: May
mesh_terms: D003937:Diagnosis, Differential; D031249:Erdheim-Chester Disease / Q000150:complications* / Q000175:diagnosis* / Q000188:drug therapy; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008875:Middle Aged; D010490:Pericardial Effusion / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology*; D055502:Secondary Prevention
chemical_list: D007166:Immunosuppressive Agents