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

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

^*Corresponding author for this work

Medicine, Allergy Division

Research output: Contribution to journal › Article › peer-review

24 Scopus citations

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.

Original language	English (US)
Pages (from-to)	2718-2726.e2
Journal	Journal of Allergy and Clinical Immunology: In Practice
Volume	8
Issue number	8
DOIs	https://doi.org/10.1016/j.jaip.2020.04.025
State	Published - Sep 2020

Funding

This work was supported in part by the Division of Intramural Research at National Institute of Allergy and Infectious Diseases and National Institute of Diabetes and Digestive and Kidney Diseases . This project has been funded in whole or in part with federal funds from the National Cancer Institute, National Institutes of Health , under Contract No. HHSN261200800001E. The content of this publication does not necessarily reflect the views or policies of the Department of Health and Human Services, nor does mention of trade names, commercial products, or organizations imply endorsement by the US Government. This work was supported in part by the Division of Intramural Research at National Institute of Allergy and Infectious Diseases and National Institute of Diabetes and Digestive and Kidney Diseases. This project has been funded in whole or in part with federal funds from the National Cancer Institute, National Institutes of Health, under Contract No. HHSN261200800001E. The content of this publication does not necessarily reflect the views or policies of the Department of Health and Human Services, nor does mention of trade names, commercial products, or organizations imply endorsement by the US Government.

Keywords

Eosinophilia
Eosinophilic colitis
Eosinophilic gastritis
Eosinophilic gastroenteritis
Eosinophilic gastrointestinal disorders
Hypereosinophilic syndrome

ASJC Scopus subject areas

Immunology and Allergy

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10.1016/j.jaip.2020.04.025

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Kuang, F. L., Curtin, B. F., Alao, H., Piligian, B., Berry, A., Holland-Thomas, N., Powers, A., Quezado, M., Lumbard, K., Fay, M. P., Klion, A. D., Kumar, S., & Khoury, P. (2020). Single-Organ and Multisystem Hypereosinophilic Syndrome Patients with Gastrointestinal Manifestations Share Common Characteristics. Journal of Allergy and Clinical Immunology: In Practice, 8(8), 2718-2726.e2. https://doi.org/10.1016/j.jaip.2020.04.025

@article{17a3e41ae6334d34b61e8eb92fd41c17,

title = "Single-Organ and Multisystem Hypereosinophilic Syndrome Patients with Gastrointestinal Manifestations Share Common Characteristics",

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",

author = "Kuang, {Fei Li} and Curtin, {Bryan F.} and Hawwa Alao and Brent Piligian and Alexis Berry and Nicole Holland-Thomas and Astin Powers and Martha Quezado and Keith Lumbard and Fay, {Michael P.} and Klion, {Amy D.} and Sheila Kumar and Paneez Khoury",

note = "Publisher Copyright: {\textcopyright} 2020",

year = "2020",

month = sep,

doi = "10.1016/j.jaip.2020.04.025",

language = "English (US)",

volume = "8",

pages = "2718--2726.e2",

journal = "Journal of Allergy and Clinical Immunology: In Practice",

issn = "2213-2198",

publisher = "American Academy of Allergy, Asthma and Immunology",

number = "8",

}

Kuang, FL, Curtin, BF, Alao, H, Piligian, B, Berry, A, Holland-Thomas, N, Powers, A, Quezado, M, Lumbard, K, Fay, MP, Klion, AD, Kumar, S & Khoury, P 2020, 'Single-Organ and Multisystem Hypereosinophilic Syndrome Patients with Gastrointestinal Manifestations Share Common Characteristics', Journal of Allergy and Clinical Immunology: In Practice, vol. 8, no. 8, pp. 2718-2726.e2. https://doi.org/10.1016/j.jaip.2020.04.025

TY - JOUR

T1 - Single-Organ and Multisystem Hypereosinophilic Syndrome Patients with Gastrointestinal Manifestations Share Common Characteristics

AU - Kuang, Fei Li

AU - Curtin, Bryan F.

AU - Alao, Hawwa

AU - Piligian, Brent

AU - Berry, Alexis

AU - Holland-Thomas, Nicole

AU - Powers, Astin

AU - Quezado, Martha

AU - Lumbard, Keith

AU - Fay, Michael P.

AU - Klion, Amy D.

AU - Kumar, Sheila

AU - Khoury, Paneez

PY - 2020/9

Y1 - 2020/9

N2 - 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.

AB - 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.

KW - Eosinophilia

KW - Eosinophilic colitis

KW - Eosinophilic gastritis

KW - Eosinophilic gastroenteritis

KW - Eosinophilic gastrointestinal disorders

KW - Hypereosinophilic syndrome

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U2 - 10.1016/j.jaip.2020.04.025

DO - 10.1016/j.jaip.2020.04.025

M3 - Article

C2 - 32344186

AN - SCOPUS:85085104381

SN - 2213-2198

VL - 8

SP - 2718-2726.e2

JO - Journal of Allergy and Clinical Immunology: In Practice

JF - Journal of Allergy and Clinical Immunology: In Practice

IS - 8

ER -

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

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