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
N1 - Funding Information:
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.
Funding Information:
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.
Publisher Copyright:
© 2020
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
VL - 8
SP - 2718-2726.e2
JO - Journal of Allergy and Clinical Immunology: In Practice
JF - Journal of Allergy and Clinical Immunology: In Practice
SN - 2213-2198
IS - 8
ER -