Eosinophilic oesophagitis

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Summary
Eosinophilic oesophagitis (eosinophilic esophagitis) results in inflammation the oesophagus, the muscular tube that connects the mouth to the stomach. Most cases are seen in people with allergies such as hay fever and asthma. There is some evidence that this may be an unusual form of food allergy.

Last reviewed 23 September 2010

INTRODUCTION

The oesophagus is a muscular tube that connects the mouth to the stomach. Eosinophilic oesophagitis results from inflammation of the oesophagus. Instead of just muscle in the oesophagus that contracts as you swallow, the lining is inflamed with so-called “eosinophils”, the same type of “allergy white cells” seen with the inflammation of hay fever or asthma. This can result in abnormal function of the oesophagus. Most cases are seen in people with allergies such as hay fever and asthma. The frequency of eosinophilic oesophagitis appears to be increasing. The reasons are unclear, but it is known that allergies of all types have become more common then they were a generation or two ago.

It is important to note that knowledge about this condition is constantly evolving, so advice given now may be revised in the future as more information is obtained.

The condition affects mainly children and young adults

This condition has been described in mainly children and young adults and is more common in males. It should be suspected when a person complains of:

• Foods sticking on the way down, sometimes completely (impaction);

• Choking on food;

• Regurgitation of food, and sometimes;

• Severe acid reflux that does not respond to medicines used to suppress stomach acid production

• Chest pain after eating

• Slow eating (in children)

It should be noted that mild reflux and vomiting are common in children and adults, and most do not have eosinophilic oesophagitis.

Natural history

The limited information that we have suggests that this condition will not go away in the majority of those affected. In babies, it may present with severe vomiting or failure to thrive. In older children, slow eating, reflux of food and acid is more common. In teens and adults, food sticking on the way down may appear, and in older patients still, food “impaction” where food gets stuck on the way down, is more common and affects ~ 1/3 adults eventually with this condition..

Diagnosis

The diagnosis can be suspected based on symptoms, but confirmation needs an examination of your oesophagus using an instrument known as an endoscope. A tissue sample (biopsy) will be taken at the same time. This procedure is normally performed by a gastroenterologist (stomach/bowel specialist). Sometimes a blood sample will show a higher than normal levels of eosinophils in the blood as well, or a protein that they produce, called eosinophilic cationic protein.

If you have already been diagnosed by a gastroenterologist, then the next step will be to determine whether allergy is playing a role by undertaking food and inhalant allergy testing. If I suspect that you have this condition (but it is unproven), then we will perform screening tests for food and inhalant allergy, but you will also be referred to a gastroenterologist for review and possible endoscopy and biopsy to prove or disprove the diagnosis.

Eosinophilic oesophagitis may result from food or inhalant allergy

Around 3/4 of patients with this condition suffer from allergic conditions such as hay fever or asthma. When allergy testing is performed, many (but not all) will have positive allergy tests to foods, sometimes even when there are no obvious symptoms after they are consumed. Some researchers have found that patients benefit if these foods are removed from the diet. When food is involved, staples such as dairy products, wheat, meats, chicken, egg, soy, corn, nuts and sometimes seafood are the most common triggers. Other patients find that symptoms appear only during springtime with exposure to pollen, and it is assumed that inflammation occurs in response to swallowed pollen. These patients might respond (in theory) to immunotherapy /desensitisation to inhalant allergen such as pollen, but there are no published trials (only isolated cases reported) proving this so far.

Who manages this condition?

Most people are diagnosed by gastroenterologists, based on the history and results of biopsy (sampling) of the oesophagus. With the recognition that allergy may also play a role in some patients, co-management by gastroenterologists, allergy /immunology specialists and specialist dietitians is more common.

Why treat this condition?

Treatment is undertaken to not only make people feel better, but also to reduce the risk of complications long-term. For example, there is preliminary evidence that scarring of the oesophagus and stricture formation may result from untreated disease. If this occurs, “dilatation” (stretching) of the oesophagus may be required to help stop food sticking. The problem is that we do not yet know whether this will occur in ALL or only some people with this condition. Some cases may also be diagnosed by “accident”, such as when a biopsy is done for another reason. When symptoms of this condition are not present, it is not currently clear whether such people should be treated, or simply observed over a period of time. Current practice is to NOT treat those who have inflammation alone without symptoms. This practice may also change in the future with further information about the natural history of this condition.

Treatment options

Time

Symptoms in infants often resolve in the first few years of life, particularly when only 1 or 2 foods are involved. Unfortunately, when symptoms arise in older children and adults, they usually last for many years. Follow-up studies so far indicate that few resolve in these groups.

Medication

• Medication to reduce acid production will reduce acid reflux and the risk of scarring that can result. These medications do not seem to reduce the inflammation in the oesophagus. e.g. Somac, Nexium, Zoton.

• Topical asthma steroid puffers (like fluticasone /Flixotide) can reduce inflammation in the oesophagus. These are "swallowed" instead of inhaled, are low dose, poorly absorbed, and extremely unlikely to cause cortisone/steroid tablet-like side-effects. They help reduce inflammation and the scarring that can result from untreated disease. When these puffers are taken, only a gentle inhalation or sucking movement should be made. Then swallow a few times. Do not eat or drink for 15-30 minutes afterwards, as otherwise the medicine will be washed out of the oesopahgus. If you get a husky voice from using a puffer, then there are alternatives, including using a mixture of liquid asthma cortisone liquid made up into a paste with an artificial sweetener.

• Montelukast (Singulair) is an asthma tablet that reduces inflammation by blocking the effects of inflammatory chemicals release by white cells known as "leucotrienes". Trials thus far show it can help symptoms but has little impact on the inflammation in the oesophagus and so is not being used much these days.

• Cortisone tablets. These might provide temporary relief for severe symptoms but regular use if avoided due to the risk of long term side-effects.

• Other medicines are also being studied but these are not currently available for clinical use.

Dilatation (stretching) of the oesophagus

Some times if the oesophagus is very narrow and symptoms are severe, you might need an endoscopy and a procedure known as "dilatation" to open up the oesophagus to allow food to pass more easily. This can help but is sometimes painful, can be associated with bleeding, and sometimes tearing of the oesophagus.

Allergy Testing and Diet manipulation

A number of studies have shown improvement in symptoms and inflammation with diet manipulation, even when allergy tests are negative, and even when no one food is identified by patients as the trigger for symptoms. There are two types of studies reported: (a) Using non-allergenic baby formula diets (amino acid/elemental diets) without any intact food protein, 90% resolve. The problem is that these are not really palatable or practical on older children or adults with “tube feeding”, or (b) Directed diets, where the major foods for which there is evidence of allergy are removed ~ 75% improve. If improvement occurs, food is introduced one at a time, starting with the foods that are least likely to cause problems. More information on how this process is undertaken is given below. It is important to note that:

• The diagnosis of eosinophilic oesophagitis should always be confirmed first, and

• That dietary manipulation should be temporary and supervised by a skilled dietitian to avoid the risk of malnutrition.

•That if diet helps, it is followed by a challenge phase to help determine the foods that cause problems, so that a more interesting and nutritious diet can follow long term, avoiding the foods associated with recurrence of symptoms.

Immunotherapy/desensitisation

Some patients do not have evidence of food allergy, but rather have evidence of allergy to inhaled and swallowed triggers like pollen. Some have a condition called oral allergy syndrome, where other plants like fruit and vegetables trigger and itchy mouth when eaten raw. Such people MAY benefit from immunotherapy/ desensitisation to switch off the pollen allergy but there are no published trials proving this yet. Instead, there are small numbers of case reports.

What we do not know

There is some evidence that scarring of the oesophagus may occur in some people with time, increasing the risk that they may need to have dilatation (stretching) of the oesophagus. But we don’t know how often this occurs, and we don’t know if we should also be treating people who have abnormal biopsies, but minor or no symptoms. At this time, we usually only recommend treatment in people who have symptoms. We also don’t know whether we should be treating people only until their symptoms are gone (like we do with asthma), or whether we should be aiming to get rid of all detectable inflammation in the oesophagus. Around a quarter of sufferers have no evidence of allergy on testing. Some have underlying conditions that can cause similar inflammation in the gut. These people may or may not respond to diet manipulation, but there is little published information on this at present although my experience is that these patients too often respond even when tests have been completely negative.

Evaluation for food allergy

The aim is to identify triggers, to end up on a sustainable nutritious diet, and minimise the need for medication. The following information is taken into account.

1. The History. Are there some foods that stick more than others? While this can simply reflect the tough texture of meat, or the consistency of dry bread, sometimes the foods that stick may be the problem. Second, are there foods that make you itchy in the mouth and throat when you eat them? This is known as oral allergy syndrome, and represents a mild food allergy. Finally, is there a history of current or past food allergy as a child? This information may sometimes be important.

2. Skin prick testing. A small lancet takes food allergen into the skin. The development of an itchy lump within 15 minutes may represent sensitivity to that food. Results from this type of allergy testing are available within 15-20 minutes of being done, and are commonly performed to identify the causes of food allergy and hay fever/asthma. Positive allergy tests to some foods like beef, milk or egg is very rare in adults, so their detection is usually considered to be significant.

3. Patch testing. Allergen extracts (commercial extracts and sometimes real food extracts) are applied to marked areas of rash-free skin, usually over the back. Allergen extracts are applied using low allergen tape & left in place for 2-3 days. The results are then “read” over the next few days. The presence of an eczema/dermatitis-like rash under an individual allergen indicates sensitivity to this substance. Patch testing is most commonly used to assess contact allergy to substances like nickel metal, garden plants, perfumes and so on, but has also been used in assessing eosinophilic oesophagitis as well. The patch is normally applied on one day with the results being read a few days later.

Diet manipulation

Sorting out whether dietary restrictions are useful for treatment can be a difficult process.

The first option is to simply see if people feel better if a few foods are removed. This process involves removing a large number of foods that have been described as causing problems in other people in published trials, specifically dairy, soy, egg, wheat, rice, seafood, corn, potato and nuts. Because this removes a large number of important foods temporarily, it can affect nutrition. For this reason, this is done with the assistance of a dietician who can advise on what foods to include, what to avoid, and help prescribe an elemental formula as an energy and protein supplement and calcium/vitamin supplements if needed while on the diet.

After 4-6 weeks on the diet, one then assesses benefit by examining the frequency and severity of symptoms, and repeating the biopsy to see if the inflammation has resolved. If so, this is followed by a challenge phase, introducing a new food every 5-7 days, and looking for recurrence of symptoms. The aim is to identify triggers, to end up on a sustainable nutritious diet, and minimise the need for medication. It’s important to note that there is no guarantee that a stricter diet will help, or that the challenges will allow us to pick out a clear dietary trigger in people with rare symptoms.

Patient support organisations

American Partnership for Eosinophilic Disorders http://www.apfed.org

http://www.medicinenet.com/eosinophilic_esophagitis/article.htm

Australian Support Network for Eosinophilic Oesophagitis and related disorders

http://www.ausee.org/

Further Information

eMedicine article: http://emedicine.medscape.com/article/174100-overview

References

1: Heine RG. Eosinophilic esophagitis: example of an emerging allergic

manifestation? Nestle Nutr Workshop Ser Pediatr Program. 2009;64:105-15;

discussion 116-20, 251-7.

2: Whitney-Miller CL, Katzka D, Furth EE. Eosinophilic esophagitis: a