ArticleBMJ case reports2017
An unusual case of interstitial lung disease in a patient with cardiopulmonary syndrome as the initial presentation of Erdheim-Chester disease.
Article in BMJ case reports, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed, 5 citations in OpenAlex.
- An Autopsied Case of Erdheim-Chester Disease with Severe Cardiovascular Involvement.Internal medicine (Tokyo, Japan) · 2025Article
- PIRO-CIC model can predict mortality and futility of care in critically ill cirrhosis patients in the intensive care unit.Hepatology international · 2023Article
- Malignant Otitis Externa in a 20-Year-Old Male Patient With Erdheim-Chester Disease: A Case Report.Cureus · 2021Article
- Erdheim-Chester Disease With Extensive Pericardial Involvement: A Case Report and Systematic Review.Cardiology research · 2020Article
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Authors and funding
4 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Erdheim-Chester disease (ECD) is a very rare disorder with only approximately 600 cases reported in the literature. ECD has been recently reclassified as a histiocytic dendritic cell neoplasm. The clinical spectrum ranges from asymptomatic tissue accumulation of histiocytes to invasive tissue infiltration, which can cause fulminant multisystem failure. It typically presents with bone pain and constitutional symptoms. Extraosseous manifestations are not uncommon. ECD-associated interstitial lung disease has been described in 20%-35% of patients. Diagnosis is primarily by tissue biopsy and immunohistochemistry showing xanthogranulomas composed of foamy histiocytes that stain positive for CD68, CD14 and CD163 and negative for CD1á and langerin. We report a case of ECD in a young man with cardiopulmonary involvement who presented with haemoptysis and dyspnoea.
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