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

Research output: Contribution to journalArticlepeer-review

16 Scopus citations


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 languageEnglish (US)
Pages (from-to)2718-2726.e2
JournalJournal of Allergy and Clinical Immunology: In Practice
Issue number8
StatePublished - Sep 2020


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

ASJC Scopus subject areas

  • Immunology and Allergy


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