Single-Organ and Multisystem Hypereosinophilic Syndrome Patients with Gastrointestinal Manifestations Share Common Characteristics

Fei Li Kuang; Bryan F Curtin; Hawwa Alao; Brent Piligian; Alexis Berry; Nicole Holland-Thomas; Astin Powers; Martha Quezado; Keith Lumbard; Michael P Fay; Amy D Klion; Sheila Kumar; Paneez Khoury

doi:10.1016/j.jaip.2020.04.025

Single-Organ and Multisystem Hypereosinophilic Syndrome Patients with Gastrointestinal Manifestations Share Common Characteristics

J Allergy Clin Immunol Pract. 2020 Sep;8(8):2718-2726.e2. doi: 10.1016/j.jaip.2020.04.025. Epub 2020 Apr 25.

Authors

Affiliations

¹ Laboratory of Parasitic Diseases, NIAID, Bethesda, Md; Division of Allergy and Immunology, Northwestern University Feinberg School of Medicine, Chicago, Ill. Electronic address: feili.kuang@northwestern.edu.
² Digestive Diseases Branch, NIDDK, National Institutes of Health, Bethesda, Md.
³ Laboratory of Parasitic Diseases, NIAID, Bethesda, Md.
⁴ Laboratory of Pathology, NCI, National Institutes of Health, Bethesda, Md.
⁵ Clinical Monitoring Research Program Directorate, Frederick National Laboratory for Cancer Research sponsored by the National Cancer Institute, Bethesda, Md.
⁶ Biostatistical Research Branch, NIAID, National Institutes of Health, Bethesda, Md.

Abstract

Background: Eosinophilic gastrointestinal diseases (EGIDs) are defined by marked eosinophilia in the gastrointestinal (GI) tract resulting in a wide variety of GI symptoms. When accompanied by blood hypereosinophilia (HE; absolute eosinophil count ≥1500/mm³), EGID can occur as an isolated GI disorder (hypereosinophilic syndrome [HES]/EGID overlap) or as part of a multisystem hypereosinophilic syndrome (Multisystem HES).

Objective: To describe the GI disease of patients categorized as those with HES/EGID overlap versus those with Multisystem HES.

Methods: Consecutively enrolled patients on a natural history protocol to study eosinophilia with biopsy-proven EGID involving the esophagus, stomach, small-bowel, and/or colon were evaluated for clinical, histopathologic, and endoscopic features by retrospective chart review.

Results: Among the 56 patients with EGID and HE, 34 were categorized as HES/EGID overlap and 22 as Multisystem HES. Demographics, GI symptoms, and associated comorbidities were similar between the 2 groups. Multisegment GI eosinophilia was present in 20 of 30 (67%) patients who underwent tissue sampling of all 4 GI segments. Tissue eosinophilia in all 4 GI segments was found in 5 of 30 (17%) patients. Dietary therapy was more common in patients with HES/EGID overlap (65% vs 23%, P = .0028). Patients with Multisystem HES were more likely to receive glucocorticoids (100% vs 79%, P = .0349) and nonglucocorticoid systemic therapies (77% vs 38%, P = .0061). One-third (8 of 22) of patients with Multisystem HES presented with isolated GI symptoms before developing extraintestinal manifestations at a median of 1 year (range, 0.25-15 years).

Conclusion: There are striking clinical similarities between patients with Multisystem HES and those with HES/EGID overlap, despite differing treatment approaches. Moreover, Multisystem HES can present with isolated GI involvement. Larger prospective studies are needed to confirm these findings.

Keywords: Eosinophilia; Eosinophilic colitis; Eosinophilic gastritis; Eosinophilic gastroenteritis; Eosinophilic gastrointestinal disorders; Hypereosinophilic syndrome.

Published by Elsevier Inc.

Publication types

Research Support, N.I.H., Extramural
Research Support, N.I.H., Intramural

MeSH terms

Enteritis* / diagnosis
Gastritis* / diagnosis
Gastritis* / epidemiology
Humans
Hypereosinophilic Syndrome* / diagnosis
Hypereosinophilic Syndrome* / drug therapy
Prospective Studies
Retrospective Studies

Abstract

Publication types

MeSH terms

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