Rezidivierender Perikarderguss als Erstmanifestation eines Morbus Erdheim-Chester

Translated title of the contribution: Recurrent pericardial effusion as first manifestation of Erdheim-Chester disease

S. Z. Lutz, M. Schmalzing, J. Vogel-Claussen, P. Adam, A. E. May

Research output: Contribution to journalArticlepeer-review

Abstract

History and admission findings: 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. Investigations: 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. Diagnosis, treatment and course: 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.

Translated title of the contributionRecurrent pericardial effusion as first manifestation of Erdheim-Chester disease
Original languageGerman
Pages (from-to)1952-1956
Number of pages5
JournalDeutsche Medizinische Wochenschrift
Volume136
Issue number39
DOIs
StatePublished - Sep 28 2011

Keywords

  • Erdheim-Chester disease
  • cardiac involvement
  • histiocytosis
  • non-Langerhanscell-histiocytosis

ASJC Scopus subject areas

  • Medicine(all)

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