Mysterious quad of constrictive pericarditis, recurrent pleural effusions, bone involvement and interstitial lung disease

Feihong Ding, Johnny Chahine, Himanshu Deshwal, Subha Ghosh, Carmela Tan, Conrad Simpfendorfer, Paul Cremer, Christine Jellis, A. Valeria Arrossi, Allan L. Klein*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

5 Scopus citations

Abstract

Erdheim-Chester disease (ECD) is a rare multisystemic non-Langerhans cell histiocytic neoplasm. The rarity of the disease and heterogenous clinical presentations often leads to delayed diagnosis. Historically, ECD lacked effective treatment and the prognosis was poor. Following the recent discovery of frequent BRAF-V600E mutation in patients with ECD, vemurafenib, a selective BRAF V600 kinase inhibitor has been approved for BRAF-mutated ECD patients. The prognosis of ECD has dramatically improved with early recognition of the disease and available treatment. ECD affects nearly every organ system. Cardiac involvement with pericardial effusion is common but rarely with constrictive physiology or requiring pericardiectomy. We present a case of a 56-year-old woman with recurrent pericarditis with constrictive physiology along with pleural effusion and interstitial lung disease that was diagnosed with ECD 3 years after initial presentation. The patient's symptoms were relieved with pericardiectomy and targeted therapy.

Original languageEnglish (US)
Pages (from-to)127-131
Number of pages5
JournalOxford Medical Case Reports
Volume2019
Issue number3
DOIs
StatePublished - Mar 1 2019

ASJC Scopus subject areas

  • Parasitology
  • Microbiology
  • Infectious Diseases

Fingerprint

Dive into the research topics of 'Mysterious quad of constrictive pericarditis, recurrent pleural effusions, bone involvement and interstitial lung disease'. Together they form a unique fingerprint.

Cite this