The European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) and the North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) released a joint guideline on childhood Eosinophilic Gastrointestinal Disorders (EGIDs) beyond Eosinophilic Oesophagitis (EoE).

What Are EGIDS

EGIDs, short for Eosinophilic Gastrointestinal Disorders, are rare chronic inflammatory conditions affecting the gastrointestinal (GI) tract beyond Eosinophilic Oesophagitis (non-EoE EGIDs). 

People with non-EoE EGIDs experience a range of upper and lower GI symptoms, including vomiting, abdominal pain, and diarrhoea. They may also develop anaemia and hypoalbuminemia. While the natural progression of non-EoE EGIDs is not well-defined, several studies suggest that these diseases are chronic but typically not life-threatening. However, the long-term consequences of non-EoE EGIDs are still unknown, and more research is required. 

Read People Stories to learn how these diseases can affect people daily.

What Is Eosinophilic Oesophagitis (EoE)

Eosinophilic Oesophagitis (or Esophagitis in American English) or EoE is a chronic immune system disease characterised by the buildup of eosinophils, a type of white blood cell, in the lining of the oesophagus.

Allergic reactions to certain foods or allergens often trigger this condition, leading to difficulty swallowing food impaction and oesophageal inflammation. Diagnosis typically involves endoscopy (gastroscopy) and biopsy to confirm the presence of eosinophils. Treatment may include dietary management, medication, and, in some cases, procedures to dilate the oesophagus.

What Are Non-EoE EGIDS

Non-EoE EGIDs are a group of diseases categorised based on the location of inflammation.

Please note that Non-EoE EGIDs may also be called "Lower gut" or "beyond the oesophagus" EGIDS.

They include:

  • Eosinophilic Gastritis (EoG),
  • Eosinophilic Duodenitis (EoD),
  • Eosinophilic Colitis (EoC),
  • Eosinophilic Enteritis (further subdivided into Eosinophilic Duodenitis (EoD), Eosinophilic Jejunitis (EoJ), and Eosinophilic Ileitis (EoI)).

The clinical symptoms of non-EoE EGIDs vary based on which part of the gastrointestinal (GI) tract is affected and the severity of eosinophilic infiltration. 

Learn more about Non-EoE EGIDS. 

No biomarker has been found for a definitive diagnosis, including blood testing.

The condition is identified based on symptoms and histologic evidence of eosinophilic inflammation after ruling out other causes of inflammation or systemic disease.

Treatment methods are determined based on individual medical and social factors.

Living with Eosinophilic Gastritis - Carla's Story

Challenges In Developing Medical Guidelines For Non-EoE EGIDs

Developing guidelines for non-EoE EGIDs presents several challenges.

  1. These conditions are rare, so there is limited clinical experience and a lack of extensive literature, especially in paediatric cases where much of the current literature is based on adult experiences.
  2. Unlike the oesophagus, the GI tract distal to the oesophagus contains a resident population of eosinophils, likely involved in various forms of innate immunity. Their numbers may vary depending on which part of the GI tract is being examined. As a result, establishing a diagnostic threshold for eosinophil counts in non-EoE EGIDs remains a challenging task.
  3. The underlying causes of non-EoE EGIDs are poorly understood and may vary depending on the part of the GI tract being examined. As a result, treatment options are limited and have not been thoroughly studied.
a portrait of a  MD, Division of Gastroenterology and Hepatology, First Department of Pediatrics, University of Athens, Childrens Hospital Agia Sofia, Athens, Greece

Alexandra Papadopoulou MD, Division of Gastroenterology and Hepatology, First Department of Paediatrics, University of Athens, Children's Hospital Agia Sofia, Athens, Greece:

"After publishing guidelines on EoE, we saw a few thousand publications and increased disease recognition. We now have tools for recognising and classifying endoscopy and histological findings. 

With EGIDs beyond EoE, we started from zero. Clinicians and pathologists need a common language to name these diseases, and pathologists need guidance in evaluating and reporting eosinophilic inflammation."


ESPGHAN Podcast

Listen to ESPGHAN's podcast episode with Alexandra Papadopoulo, "Pitfalls in the diagnosis and management of children with non-EoE EGIDs", to learn more about the development of the Guidelines:


Methods

The joint working group included paediatric gastroenterologists, adult gastroenterologists, allergists/immunologists, and pathologists. 

Researchers have taken a two-pronged approach to address current issues in caring for paediatric patients with non-EoE EGIDs. This method includes a comprehensive literature review and a series of electronic and virtual discussions. The group thoroughly searched the MEDLINE, EMBASE, and Cochrane databases until February 2022. 

Researchers developed statements and recommendations to help improve care for children with non-EoE EGIDs. They are based in part on the more extensive body of literature available for adults.

To ensure current standards of evidence assessment were met, researchers formulated recommendations using the Appraisal of Guidelines for Research and Evaluation (AGREE) II and the Grading of Recommendations Assessment, Development and Evaluation (GRADE) system.

Dr. Glenn Furuta, Specialist in Eosinophilic Diseases, Wearing White Lab Coat

Glenn T. Furuta, MD, a professor of paediatrics at the University of Colorado School of Medicine and the section chief of pediatric gastroenterology at Children’s Hospital Colorado, in Aurora, in the interview with Gastroenterology&Endoscopy News

"Currently EGIDs are rare, are characterized by common GI symptoms, and a diagnostic biopsy. Treatments are in development. For pediatricians, the goals of treatment are making sure that patients are able to grow and develop, improving symptoms and quality of life, and then helping that tissue appear more normal by the eye as well as histologically."

Photo: rarediseasesnetwork.org

Results

The guidelines provide information on the current concept of non-EoE EGIDs, disease pathogenesis, epidemiology, clinical manifestations, diagnostic and disease surveillance procedures, and current treatment options.

The joint working group developed 34 statements based on available evidence and 41 recommendations based on expert opinion and best clinical practices.

Diagnostic Algorithm for Eosinophilic Gastrointestinal Disorders (EGIDs) in Children

Graphical Abstract from ESPGHAN/NASPGHAN Guidelines illustrating the diagnostic algorithm and criteria for eosinophilic gastrointestinal disorders in children, Journal of Pediatric Gastroenterology and Nutrition (JPGN).

Conclusion

The existing literature on Non-EoE EGIDs is limited, which makes it challenging to provide clear recommendations. These consensus-based clinical practice guidelines aid clinicians in caring for children with Non-EoE EGIDs and facilitate high-quality randomised controlled trials of different treatment approaches using standardised, consistent disease definitions.

Dr. Glenn Furuta, Specialist in Eosinophilic Diseases, Wearing White Lab Coat

Glenn T. Furuta, MD, in the interview with Gastroenterology&Endoscopy News:

"The guideline helps standardize clinical and research practice. It's a benchmark for pediatric patients. We are putting a stake in the ground to say, "Here's where things are today with the knowledge base we have, and this is what it's going to look like for X period of time." As we learn more and do more research, then we'll know whether that was right or wrong or needs to be adjusted.

The second part of that relates to clinical features and guides us in caring for patients. For example, when we institute a new treatment, how do we know that the patient is better? We've been able to say, "Here's what normal is, here's what abnormal is from symptoms, physical examination, laboratory, radiology, endoscopy, and histology standpoints.

After we invoke a treatment, are they better? And what do those measures look like? Standardizing care and research and knowing what natural history looks like are a big part of what we're doing."

Photo: rarediseasesnetwork.org

What does this mean to our EOS community? 

As an organisation, since the publication became available, we have advocated for clarification in areas of concern to our community.

We have asked the experts:

  • As the histology reporting and number of biopsies taken have changed over time, how will this affect previous diagnoses or missed diagnoses?
  • Given the published data of elemental diets on reducing eosinophil counts in Eosinophilic Gastritis and Gastroenteritis, how will someone's restricted diet or other treatments affect their meeting these strict biopsy eosinophilic count criteria?
  • What happens if you are symptomatic but do not qualify for new strict criteria? Is there a grey area between normal biopsies and strict research criteria that will require further evaluation and expert opinion?
Amanda Cordell at paediatric gastroenterology conference

Amanda Cordell, CEO and Founder of EOS Network:

After over two decades of advocacy, seeing the first published guideline in non-EoE EGIDs is fantastic.

Having participated in other guidelines, I acknowledge and appreciate the extensive personal time the authors gave to develop them. 

It's important to note this is now a fast-moving area of research, with experts worldwide working tirelessly to collect robust data that better clarifies the questions that caused many challenges in this first line in the sand. Many of the questions we asked are now being researched.

EOS Network is proud to be participating in developing a global joint paediatric and adult guideline, and we will keep you posted.

Read The Full Guidelines on JPGN 

Learn About EOS Network Advocacy Within ESPGHAN

Advocacy Within CEGIR