Highlights of CEGIR Publications

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Validation of self-reported diagnosis of eosinophilic gastrointestinal disorders patients enrolled in the CEGIR contact registry
Clin Res Hepatol Gastroenterol. Available online 5 November 2020.

In a letter to the editor published in Clinics and Research in Hepatology and Gastroenterology, researchers describe their work to validate the information of patient-reported data supplied to a contact registry established by the Consortium of Eosinophilic Gastrointestinal Disease Researchers (CEGIR), with support from the Data Management Coordinating Center of the Rare Diseases Clinical Research Network.

The purpose of the research was to validate the information that patients supplied to the registry regarding diagnoses, procedures, and symptoms of eosinophilic gastrointestinal diseases (EGID) by comparing it to what their physicians reported, and to lab reports.

Among the findings, researchers observed:

  • 100% agreement between the participant- and physician-reported diagnosis of EGID
  • For 56% of participants who had pathology reports available, there was 100% agreement between that report and reports from both physician and patient.
  • There was high agreement between patient and physician reports for the subtype of EGID, history of upper endoscopy, food impaction, esophageal dilation, and colonoscopy.

“Validation of the CEGIR Contact Registry data is important because it supports the high level of accuracy of patient-reported data in this cohort,” said lead study author Dr. Girish Hiremath, of Vanderbilt University Medical Center in Nashville, TN. “This in turn strengthens the credibility of self-reported data in this registry, which may be used for future research to help us to better understand EGIDs.”

Molecular, endoscopic, histologic, and circulating biomarker-based diagnosis of eosinophilic gastritis: Multi-site study
J Allergy Clin Immunol. Jan. 2020 Volume 145, Issue 1, Pages 255–269

This paper describes new testing platforms for blood and tissue that may help guide clinicians to a diagnosis of eosinophilic gastritis (EG) in the future. Currently, there are no consensus guidelines to standardize diagnostic criteria for EG. This is in large part due to EG being a rare condition, which makes it challenging to study, and also because it is common for people who have EG to also have another subset of eosinophilic gastrointestinal disease. The samples analyzed by the CEGIR investigators enabled them to develop a molecular profile that correlated with findings from endoscopy and histology. Levels of eotaxin-3 in the blood were strongly associated with expression of gastric cytokine CCL26. Additional studies are needed to validate these findings before these new testing platforms could be considered for clinical use.

Advancing patient care through the Consortium of Eosinophilic Gastrointestinal Disease Researchers (CEGIR)
J Allergy Clin Immunol. Jan. 2020 Volume 145, Issue 1, Pages 28-37

In this rostum, investigators from CEGIR outline the progress and direction of the consortium and the multidisciplinary relationships and engagement with patient advocacy groups like APFED, the National Institutes of Health and partners from industry and academia. The article outlines CEGIRs work with eosinophilic gastrointestinal diseases, including natural history studies to promote clinical trial readiness tools, clinical trials, investigator training program, and innovative pilot studies.

Eosinophilic gastrointestinal disease below the belt
J Allergy Clin Immunol. January 2020 Volume 145, Issue 1, Pages 87–89

This article from the Journal of Allergy and Clinical Immunology reviews the current state of eosinophilic gastrointestinal diseases (EGIDs) with a focus on “unmet needs, barriers, and future directions in patients with nonesophageal EGIDs.” While eosinophilic esophagitis (EoE) has been the subject of much focus and advancement, EGIDs that affect other parts of the gastrointestinal tract, including eosinophilic gastritis (EG), eosinophilic gastroenteritis (EGE), and eosinophilic colitis (EC), are poorly understood by comparison and there are no well-established guidelines for diagnosis or management.

The article concludes that: 1. Clear diagnostic criteria for EG, EGE, and EC must be established; 2. outcome measures, such as patient-reported outcomes and endoscopic and histologic assessments, need to be developed; 3. More work is required to understand pathogenesis; and 4. Longitudinal trials are needed to better understand disease mechanisms and long-term outcomes.

Emerging therapies for eosinophilic esophagitis
J Allergy Clin Immunol. January 2020 Volume 145, Issue 1, Pages 38–45

This article published in the Journal of Allergy and Clinical Immunology discusses recent advances in the treatment of eosinophilic esophagitis (EoE), specifically swallowed topical steroids, biological agents, dietary approaches, and novel molecular targets. It concludes that newer formulations of swallowed topical corticosteroids have appeared to be highly effective in short- and long-term EoE management of EoE. Dietary approaches have shown a similar effectiveness; however, new approaches, such as the 2-4-6-food elimination diet (step-up approach) are similarly effective to empiric food elimination but also more efficient.

For patients who do not achieve/maintain long-term remission with steroid formulations and dietary restrictions, biological agents may be an effective treatment alternative, with anti–IL-13 and anti–IL-4 receptor antibodies thus far offering promising results in phase 2 clinical trials. The article also highlights newer targets now being tested in clinical trials, including the angiotensin II receptor type 1 (losartan) or Siglec-8 (anti–Siglec-8).

Diagnosis and treatment of eosinophilic esophagitis
J Allergy Clin Immunol. January 2020 Volume 145, Issue 1, Pages 1–7

This review from the Journal of Allergy and Clinical Immunology highlights the most recent diagnostic criteria and disease management for adult and pediatric EoE patients. The review concludes that when untreated, EoE can lead to esophageal remodeling and strictures; therefore, maintenance therapy is advised. The review also concludes that both dietary and medical therapy for EoE can help “reduce histologic inflammation, improve clinical symptoms and endoscopic abnormalities, and improve remodeling in a subset of patients.”

The authors also note the need for better ways to identify food triggers in patients with EoE, the need to identify clinical or genetic predictors of response to treat or slow progression of the disease, and the importance of identifying effective maintenance therapies.


Other CEGIR Journal Publications