Eosinophilic Oesophagitis (EoE) is the most well-known type of eosinophilic gastrointestinal disease (EGID).¹ It is an inflammatory condition in which large numbers of eosinophils (a type of white blood cell that is part of the body’s immune system) infiltrate and accumulate in the lining of the food pipe (oesophagus) that connects the mouth to the stomach.^1,2 This may be due to an allergic response to food, environment, or other unknown triggers.

EoE can cause difficulties in feeding or swallowing food, food getting stuck in the oesophagus (food impaction or food bolus obstruction/FOB), nausea, persistent vomiting, and stomach and chest pain, among other symptoms.^1-3

EoE can affect both children and adults.³ There has been some reported cases of family history with EoE. People with EoE may often have other allergic conditions such as asthma, hay fever or eczema.^1-3

Universal rare disease classifications provide a common language for recording, reporting and monitoring diseases. Please visit the Rare Disease Classifications page for more information about these internationally recognised classifications.

ICD-11: DA24.1 Eosinophilic oesophagitis

There is no ORPHAcode.

Symptoms of EoE may present in infants or may arise in older children and adults.² The symptoms vary between individuals, and may include:^1-3

• Feeding difficulties (slow chewing of food, needing pureed food, avoidance of certain textures, oral aversion to food)
• Poor appetite or not wanting to eat
• Difficulty in swallowing food and/or regularly requiring a drink whilst eating
• Food getting stuck in the eosophagus when eating, also known as food impaction/Food Bolus Obstruction (FBO)
• Nausea, persistent choking or gagging on food, retching, and vomiting/regurgitation of food
• Stomach (abdominal) pain or chest pain
• Severe acid reflux (heartburn) that does not respond to medications
• Failure to thrive/poor weight gain

EoE can also lead to scarring and narrowing of the oesophagus (stricture).^1,2

Please speak to your medical team to learn more about the symptoms and complications of EoE.

Rare diseases are often serious and progressive, exhibiting a high degree of symptom complexity, leading to significant disability. Majority of the estimated two million Australians living with a rare disease meet the Australian Government’s definition for disability (in accordance to the Australian Public Service Commission and Australian Bureau of Statistics), and many experience severe and permanent disability impacts. If you or someone you care for is experiencing disability-related impacts from a rare condition, please speak with a health or disability professional for advice. Information about relevant disability support can be found at the RARE Portal’s Disability Support Information page.

EoE may be caused by an allergy to food or the environment, and in some cases, the exact cause may be unknown.¹ There has been some reported cases of family history with EoE.³

A diagnosis of EoE should be considered in the presence of chronic symptoms of oesophagus dysfunction.³ Diagnosis of EoE usually involves an endoscopy and biopsies performed by a gastroenterologist.^1-3 The endoscopy involves the insertion of an endoscope (a tube with a light and attached camera) to examine the oeosphagus and to take tissue samples (biopsy). The tissue samples are examined by a pathologist to identify if there is accumulation of eosinophils (a type of white blood cell) – the presence of more than 15 eosinophils per high-power field (hpf) is indicative of EoE;^1,3 however other possible causes of increased eosinophils still need to be ruled out.³

The Updated International Consensus Diagnostic Criteria for Eosinophilic Esophagitis (Proceedings of the AGREE Conference) was developed in 2018 by paediatric and adult physicians and researchers from gastroenterology, allergy, and pathology subspecialties representing 14 countries, including Australia.

Please speak to your medical team to learn more about the available diagnostic pathways for EoE.

EoE is a chronic condition that require ongoing management and monitoring supervised by a multidisciplinary medical team.¹ Treatment and management of EoE may include dietary changes, medication and endoscopic interventions.^1,2 Regular endoscopies and repeat biopsies may be required to monitor the condition.

Dietary changes should only be made under the direction and supervision of the appropriate medical professional.² This may include elimination diets to identify if EoE is caused by a food allergen or an elemental diet to replace food intake with a liquid formula.^1,2 Medications for EoE may include swallowed corticosteroids to reduce inflammation and proton pump inhibitors to reduce acid production as well as for its anti-inflammatory effect; whilst endoscopic interventions such as oesophageal dilation (to widen the oesophagus) may be used in specific cases when the oesophagus is very narrow.^1,2

Please speak to your medical team to learn more about the possible treatment or management options for your condition. Treatment will depend on an individual’s specific condition and symptoms. It is also important to stay connected to your medical team so that you can be made aware of any upcoming clinical trial opportunities.

Clinical care for rare diseases often involves a multidisciplinary team of medical, care and support professionals. Please note that the information provided here is as a guide and that RVA does not necessarily monitor or endorse specific clinics or health experts.

Healthcare professionals involved in the treatment of EoE may include general practitioners (GP), gastroenterologists (stomach/bowel medical specialists), clinical immunology/allergy specialists and specialist dietitians.² The need for different healthcare professionals may change over a person’s lifetime and extend beyond those listed here.

The following guidelines and recommendations are available regarding clinical care of EoE:

• Updated International Consensus Diagnostic Criteria for Eosinophilic Esophagitis (Proceedings of the AGREE Conference) was developed in 2018 by paediatric and adult physicians and researchers from gastroenterology, allergy, and pathology subspecialties representing 14 countries, including Australia.
• International Consensus Recommendations for Eosinophilic Gastrointestinal Disease Nomenclature , which involved participation from Australian experts, contains important clarification about the nomenclature of EGIDs for clinical settings
• Australasian Society of Clinical Immunology and Allergy resources on EoE:
  • Two Food Elimination Diet (2FED) for EoE
  • Four Food Elimination Diet (4FED) for EoE
  • Action Plan for Eosinophilic Oesophagitis (EoE)
  • Management Plan for Eosinophilic Oesophagitis (EoE)
• Australia’s National Allergy Strategy to improve the health and quality of life of Australian with allergic diseases, including conditions such as EoE

The following guidance is available from international experts outside Australia; however, there may be information that is not relevant or applicable to the Australian context, and may not be up to date:

• AGA Institute and the Joint Task Force on Allergy-Immunology Practice Parameters Clinical Guidelines for the Management of Eosinophilic Esophagitis (United States of America’s American Gastroenterological Association, AGA); published in 2020
• British Society of Gastroenterology (BSG) and British Society of Paediatric Gastroenterology, Hepatology and Nutrition (BSPGHAN) joint consensus guidelines on the diagnosis and management of eosinophilic oesophagitis in children and adults (United Kingdom); published in 2022

Individuals living with rare diseases may have complex medical issues and disabilities, which are not always visible. It is often useful to refer to their medical history as well as personal information such as a medical card, doctor’s letter, or if available, a rare disease passport, for relevant information.

It may be important to consider the following when managing individuals living with EoE at emergency departments/services:

Individuals with EoE may present to emergency departments with food impactions. When treating patients with EoE, it is important to refer to their emergency and management plans:

• Australasian Society of Clinical Immunology and Allergy (ASCIA): Action Plan for Eosinophilic Oesophagitis (EoE)
• Australasian Society of Clinical Immunology and Allergy (ASCIA): Management Plan for Eosinophilic Oesophagitis (EoE)

ausEE Inc.: Research provides information about research on eosinophilic diseases in Australia.

There are specific considerations around participating in rare disease research, including clinical trials. It is important to be mindful of issues such as data privacy, research ethics, consent and differences in research regulations between Australia and other countries.

If you are interested in finding clinical trials for your condition, please visit the following websites; however, there may not be any clinical trials available:

• Australian Clinical Trials
• ClinicalTrials.gov

It is best to discuss your interest in any clinical trials with your medical team to determine suitability and eligibility.

Please note that RVA does not necessarily monitor or endorse each group/organisation’s operational governance and activities.

Australian Organisation:

ausEE Inc.
Website: https://ausee.org

ausEE Inc. is Australia’s peak national support and patient advocacy organisation representing Australians living with an eosinophilic disease, including EGPA and eosinophilic gastrointestinal diseases (EGIDs). ausEE’s mission is to improve the lives of those affected by eosinophilic diseases through providing support, evidence-based information, resources, advocacy and by campaigning to raise awareness and funds for research in Australia.

Please note that RVA does not monitor or endorse each group/organisation’s operational governance and activities. When engaging with a group, please consider the information on the RARE Portal’s Finding Helpful Peer and Community Supports page.

Eosinophilic Oesophagitis (EoE) varies between individuals, and each person’s experience is unique.

Please visit ausEE Inc. (EOSaware): My EoE Stories to read the personal stories of people living with EoE.

If you would like to share your personal story with RVA, please visit the Rare Voices Australia: Share Your Story page. RVA will consider your story for publishing on our website and inclusion on the RARE Portal.

ausEE Inc. offers support programs, including for peer support.

For information on available government and social services that provide support for individuals with a rare disease, please visit the National and State Services pages.

People living with a rare disease, including families and carers, often face unique challenges such as diagnostic delays, misdiagnoses, limited treatment options, and limited access to rare disease specialists and support. These challenges may impact people’s emotional wellbeing and quality of life. Many people find it helpful to seek mental health and wellbeing support to cope with ongoing stress and uncertainty. Connecting with people who have shared experiences through a support group may also be helpful. Information about relevant mental health and wellbeing support can be found at:

• Mental Health and Wellbeing Support for Australians Living with a Rare Disease
• The National and State Services pages underneath the ‘Mental Health’ sections listed

Further information on EoE, or allergies in general, can be found at:

• Australasian Society of Clinical Immunology and Allergy: Food – Other Adverse Reactions Eosinophilic Oesophagitis (EoE)
• National Organization for Rare Disorders (NORD): Eosinophilic Esophagitis
• Consortium of Eosinophilic Gastrointestinal Disease Researchers (CEGIR)
• AGA (American Gastroenterological Association) – EoE Patient Resource Center
• Allergy & Anaphylaxis Australia (A&AA)
• National Allergy Council

• Human Phenotype Ontology (HPO): Eosinophilic Esophagitis
• International Consensus Recommendations for Eosinophilic Gastrointestinal Disease Nomenclature
• ausEE Inc.: Information and Resources for Health Professionals
• National Allergy Council: Shared care for allergy
• National Allergy Council: Allergy Aware – Resources for Health Professionals
• Consortium of Eosinophilic Gastrointestinal Disease Researchers: Resources For Researchers and Clinicians

This page has been co-developed by Rare Voices Australia (RVA)’s RARE Portal team in consultation with ausEE Inc.

If you are aware of any additional information that may benefit stakeholders with an interest in this page, or if you notice any broken links or inaccurate information, please let us know via the Contribute page.