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The Idiopathic Hypereosinophilic Syndrome: Clinical, Pathophysiologic, and Therapeutic Considerations
Abstract
The idiopathic hypereosinophilic syndrome (HES) represents a heterogeneous group of disorders with the common features of prolonged eosinophilia of an undetectable cause and organ system dysfunction. Fifty patients with the idiopathic HES were studied over 11 years of the National Institutes of Health. Multiple organ systems were involved; bone marrow hypereosinophilia was common to all patients, but the most severe clinicopathologic involvement was of the heart and nervous system. Postmortem gross pathologic examination of the hearts of patients with idiopathic and nonidiopathic HES suggested that the common mechanism of cardiac disease is the eosinophilia. Endomyocardial biopsy findings showed that the endothelial cells in the endocardium and of the microvasculature were the primary targets of the tissue damage. This damage initiates thrombosis; endocardial fibrosis and restrictive endomyocardopathy may follow. In-vitro culture of circulating eosinophil colony-forming units showed some normal studies, some studies showing increased progenitor cells committed to eosinophil development, and others showing an excess production of eosinophil colony-stimulating factor. Chemotherapy to lower the eosinophil counts has resulted in marked improvement of HES prognosis, as have agressive medical and surgical approaches to cardiovascular complications.
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Eosinophils likely play a pathogenic role in eosinophilic myocarditis, contributing to inflammation and subsequent fibrosis and heart failure.
“Eosinophils likely play a pathogenic role in eosinophilic myocarditis, contributing to inflammation and its subsequent consequences such as fibrosis and heart failure conditions.”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [204]
Cytokines and granule contents released by eosinophils including major basic protein are highly proinflammatory and cytotoxic in the heart.
“The cytokines and granule contents released by eosinophils including major MBP (major basic protein) are known to be highly proinflammatory and cytotoxic in the heart.”
From Autoimmune Myocarditis, Old Dogs and New Tricks. (2024) · cites this paper as [204]
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Cited by
Sorted by citations
Autoimmune Myocarditis, Old Dogs and New Tricks.
Won, T., Song, E. J., Kalinoski, H. M., Moslehi, J. J., & Čiháková, D. (2024) · Circulation Research · doi:10.1161/circresaha.124.323816 · Cited by 51
Anti-IL-5 and hypereosinophilic syndromes
SUTTON, S., ASSAAD, A., & ROTHENBERG, M. (2005) · Clinical Immunology · doi:10.1016/j.clim.2005.02.006 · Cited by 40
Metadata
- Authors
- FAUCI, A. S., HARLEY, J. B., ROBERTS, W. C., FERRANS, V. J., GRALNICK, H. R., & BJORNSON, B. H.
- Affiliations
- Bethesda, Maryland
- Corresponding author
- FAUCI, ANTHONY S.
- ORCID
- FAUCI, ANTHONY S.
- Venue
- Annals of Internal Medicine
- Publisher
- American College of Physicians
- Published
- 1982-07-01
- Volume
- 97
- Issue
- 1
- Pages
- 78-92
- ISSN
- 0003-4819 (ISSN-L)
- 1539-3704
- OpenAlex
- W1972031015
- Cited by
- 823
- References
- 1
- Citations by year
- 2026: 1 · 2025: 4 · 2024: 19 · 2023: 12 · 2022: 17 · 2021: 11 · 2020: 8 · 2019: 13 · 2018: 7 · 2017: 9 · 2016: 16 · 2015: 11 · 2014: 21 · 2013: 15 · 2012: 23
- FWCI
- 7.17
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Annals of Internal Medicine (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Rheumatology › Eosinophilic Disorders and Syndromes
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Asthma and respiratory diseases
- Life Sciences › Immunology and Microbiology › Immunology › Immunodeficiency and Autoimmune Disorders
- Keywords
- idiopathic hypereosinophilic syndrome, eosinophilia, cardiac involvement, endomyocardial biopsy, endothelial injury, thrombosis, endocardial fibrosis, chemotherapy, cardiovascular complications, prognosis
- MeSH terms
- Cells, Cultured, Endocardium, Eosinophilia, Eosinophils, Humans, Cardiomyopathies, Myocardium, National Institutes of Health (U.S.), Prognosis, Syndrome, United States
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