Will Research Consortium Mean Progress in Eosinophilic GI Disorders?

The first clinical descriptions of eosinophilic esophagitis (EoE) emerged over two decades ago. Subsequently, adults with food impaction or dysphagia or change to children with non-descriptive gastroesophageal reflux disease (GERD)–like symptoms are increasingly recognized as having something different than GERD. Soon thereafter, a whole set of distinct eosinophilic GI diseases (EGID) involving various segments of the GI tract were described, with EoE receiving the most attention.

Despite the fact that these patient phenotypes were increasingly recognized, no diagnostic criteria existed and no metrics were developed to assess disease activity. Both of these factors led to myriad ways that patients received the diagnosis and how remission was assessed, thus limiting the development of novel therapeutic drugs. Although swallowing steroids provided by metered-dose inhalers and limiting food triggers brought relief of symptoms and reduced esophageal eosinophilia, the need existed to determine the dosing of medications, duration of treatment and identification of allergenic foods.

During the last decade, significant advances have been made in the care of patients with EoE, the most prevalent of these disorders, due in many ways to teams of pediatric and adult colleagues from different specialties working together. The first consensus recommendations that set initial suggestions for making the diagnosis of EoE were published in 2007, thus providing a starting place for research studies and clinical care. These recommendations were based primarily on clinical expertise since the literature was only just emerging and identified EoE as a clinicopathologic, chronic, allergendriven disease different than GERD. Although useful, these early recommendations contained one major concern — the use of a therapeutic trial of proton pump inhibitors (PPIs) to make the diagnosis. Few, if any, other diseases required a trial of a medication to establish a diagnosis. However, this practice was included because of the lack of alternative ways to rule out reflux as a cause of underlying problems.

Since 2007, recommendations have been modified more on the basis of published literature than clinical experiences, and a number of important advances have been made. These advances include discovering key pathogenic genes and pathways, developing validated metrics to measure disease activity, identifying novel ways to assess disease activity and conducting double-blind, placebo-controlled trials. In addition, the entity of PPI-responsive esophageal eosinophilia (PPIREE) was described and is close relationship with EoE has been established. Despite these advances, there is still no U.S. Food and Drug Administration (FDA)–approved treatment specifically for any EGID. In order to facilitate progress in this field, we have developed strong relationships with key EGID stakeholders, including the patients themselves, primarily through the support of patient advocacy groups, the National Institutes of Health (NIH), FDA, industry, physicians and investigators.

As a result of these efforts, NIH has helped us to establish the Consortium of Eosinophilic Gastrointestinal Disease Researchers (CEGIR), which is part of the Rare Diseases Clinical Research Network and supported by the National Center for Advancing Translational Sciences (NCATS). CEGIR is directly funded by NCATS and two other NIH institutes (the National Institute of Allergy and Infectious Diseases and the National Institute of Diabetes and Digestive and Kidney Diseases), and two patient advocacy groups (the American Partnership for Eosinophilic Disorders and the Campaign Urging Research for Eosinophilic Disease).


CEGIR aims to improve the lives of individuals with EGID through innovative research, clinical expertise and education via collaboration between scientists, health-care providers, patients and professional organizations.


CEGIR aims to improve the lives of individuals with EGID through innovative research, clinical expertise and education via collaboration between scientists, health-care providers, patients and professional organizations. CEGIR provides a framework for clinicians, researchers, patients, NIH and other stakeholders to partner around EGID and strengthens momentum for better outcomes for EGID in several ways: establishing frequent, structured communication between stakeholders, jumpstarting new research, training physicians in these rare disorders and conducting multicenter clinical trials. CEGIR’s pilot program supports a series of preliminary investigations testing new hypotheses concerning EGID and is a kick starter for promising research. CEGIR’s training program is designed to equip the next generation of physicians with expertise in EGID; educational material and a full description are available through the CEGIR website (https:// www.rarediseasesnetwork.org/cms/cegir/).

CEGIR is now conducting several clinical studies, including an observational study investigating outcome measures for EGID across ages, also known as OMEGA. It is a prospective, multicenter study to compare and validate endoscopic, histologic, molecular and patient-reported outcomes in pediatric and adult patients with EoE, eosinophilic gastritis and eosinophilic colitis. The OMEGA study will be a critical step forward for eosinophilic gastritis and eosinophilic colitis, which still lack consensus for diagnostic criteria. In addition, CEGIR is conducting an EoE intervention trial comparing six-food versus one-food EoE elimination diets. This study was based on priorities conveyed by patients, who indicated the need for less restrictive diet therapy.

CEGIR is already making major advances concerning understanding, treating and educating patients and the public about these diseases. The formation of CEGIR is also particularly timely, as the first generation of anti-eosinophil therapy (humanized anti– interleukin 5 monoclonal antibody therapy) has been recently approved by FDA for eosinophilic asthma. CEGIR, due to its national collaborative nature, is poised to apply these and other developing anti-eosinophil therapy advances to treating EGID.

However, key to the completion of these studies is recruitment of eligible subjects. Interested colleagues can learn more about present studies and goals at https://www. rarediseasesnetwork.org/cms/cegir. We anticipate that the collaborative efforts of CEGIR will provide important advances in the care of patients with EGID.

Dr. Rothenberg is program director of the Consortium for Gastrointestinal Eosinophilic Researchers (CEGIR). CEGIR (U54 AI117804) is part of the Rare Diseases Clinical Research Network (RDCRN), an initiative of the Office of Rare Diseases Research (ORDR), NCATS, and is funded through collaboration between NIAID, NIDDK and NCATS. Dr. Furuta is the Program Director of the Consortium for Gastrointestinal Eosinophilic Researchers (CEGIR). CEGIR (U54 AI117804) is part of the Rare Diseases Clinical Research Network (RDCRN), an initiative of the Office of Rare Diseases Research (ORDR), NCATS, and is funded through collaboration between NIAID, NIDDK and NCATS. Division of Allergy and Immunology, Department of Pediatrics, Cincinnati Children’s Hospital Medical Center.

Dr. Rothenberg is the principle investigator of the Consortium for Gastrointestinal Eosinophilic Researchers (CEGIR). CEGIR (U54 AI117804) is part of the Rare Diseases Clinical Research Network (RDCRN), an initiative of the Office of Rare Diseases Research (ORDR), NCATS, and is funded through collaboration between NIAID, NIDDK and NCATS. Dr. Furuta is the Program Director of the Consortium for Gastrointestinal Eosinophilic Researchers (CEGIR). CEGIR (U54 AI117804) is part of the Rare Diseases Clinical Research Network (RDCRN), an initiative of the Office of Rare Diseases Research (ORDR), NCATS, and is funded through collaboration between NIAID, NIDDK and NCATS. Division of Allergy and Immunology, Department of Pediatrics, Cincinnati Children’s Hospital Medical Center

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