what is Eosinophilic esophagitis ?? Causes, Symptoms, and Treatment

The illness

Eosinophilic diseases of the gastrointestinal tract (EGID) are divided into 2 main groups: eosinophilic esophagitis (EoE) and eosinophilic gastroenteropathies (EGE).

Eosinophilic esophagitis (EoE)

EoE , the most common of the EGIDs, is a chronic inflammatory disease localized in the esophagus, of presumable immuno-allergic etiopathogenesis, which shows an alternation of periods of clinical remission and episodes of activity . It is an entity characterized histologically by severe mucosal eosinophilia (> 15 eosinophils/HPF- in proximal, mid- and distal esophageal biopsies) and by symptoms secondary to dysfunction of the ‘esophagus.

The annual incidence of the disease varies according to the geographical origin of the population included, from 1.6 new cases/100,000 children/year in Denmark to 8 in the United Kingdom and from 0.7 to 10 in the United States 10.

EoE should be suspected in the presence of chronic symptoms of esophageal dysfunction and/or fibrosis unresponsive to maximal dose proton pump inhibitor (PPI) therapy (1 mg/kg/day) for 8-12 weeks .

As far as clinical symptoms are concerned, infants and preschool-aged children often have feeding difficulties, with symptoms almost superimposable to those of GERD (gastroesophageal reflux), while school-aged children more frequently have vomiting or painful symptoms. Dysphagia is a predominant symptom in adolescents and adults.

40-90% of children with EoE have signs and/or symptoms of atopy (asthma, allergic rhinitis, atopic dermatitis) and this finding can be of diagnostic help, together with the family history of atopy, which is also present in a high percentage of cases.

Eosinophilic gastroenteritis (EGE)

EGE , on the other hand, constitute a group of rare, heterogeneous and ill-defined diseases both clinically and in etiopathogenetic terms. They are diagnosed if hypereosinophilia is found in 1 or more parts of the gastrointestinal tract in the absence of a known cause of eosinophilia. Reported prevalence differs across geographic regions, with rates ranging from 50.2 cases/100,000 children in the UK to 8.9 cases in Australia to 43 cases/100,000 children in the US.

Diagnosis is mostly based on 3 diagnostic criteria:

1. presence of non-specific gastrointestinal symptoms (diarrhoea, vomiting, abdominal pain)
2. eosinophilic infiltration in one or more tracts of the gastrointestinal tract
3. exclusion of other causes of gastrointestinal eosinophilia (chronic inflammatory disease, parasitic infections, primary immunodeficiencies).

The clinical presentation of EGE varies according to the gastrointestinal tract involved, but the most frequent manifestations are characterized by vomiting, diarrhea, abdominal pain, weight loss or failure to thrive due to protein malabsorption and loss, frank or occult bleeding, anemia, and jaundice obstructive.

EGEs are then classified on the basis of the intestinal tract involved (gastritis, gastroenteritis, colitis, proctocolitis, etc.) or on the basis of the localization of eosinophils at the tissue level (serosic, mucosal or muscular). The mucosal form is the most frequent and the muscular form the rarest. Given the rarity of these diseases, common causes of tissue eosinophilia such as parasitic infections, inflammatory bowel disease, connective tissue diseases, drug allergies, lymphoproliferative disorders must be excluded before making the diagnosis of EGE.

If muscle layers are involved, abdominal pain, cramping, and intestinal obstruction may be the presenting symptoms, and in rare cases, organ perforation may occur. The serous forms are associated with ascites and abdominal distension.

Epidemiology

EGE has an estimated prevalence of 1-5:100,000 and belongs to the group of eosinophil-associated gastrointestinal disorders, affecting all ages, with an average age between 30 and 50 years, with a slight preponderance in males. The highest prevalence seems to be recorded in North America, Sweden and Australia with an incidence figure of about 5-7/100,000 inhabitants and a prevalence figure of 50-60/100,000 inhabitants. In Europe, the main data come from Switzerland with an estimated prevalence of about 23/100,000 inhabitants. A recent study of the Canadian population found an increase in EoE in terms of incidence from 2.1 to 11.0 per 100,000 people and this figure would be the result of an increase in the number of esophageal biopsies, even if at the same time accesses for endoscopy indicating dysphagia. It is estimated that the male/female ratio is 3/1 even if at the moment this figure has no explanations from a physiopathological point of view. White Americans appear more affected (58%) than African-Americans (34%) and other ethnicities (8%).

Diagnosis

The diagnosis of EGE is based on three main clinical signs that apply to all ages:

1. The presence of chronic gastrointestinal symptoms
2. The presence of eosinophilic infiltrate of one or more segments of the gastrointestinal tract
3. Exclusion of other causes of increased eosinophilic infiltrate in the gastrointestinal tract (e.g. any food allergies or drug reactions, celiac disease, parasitic infections, hypereosinophilic syndromes, inflammatory bowel disease, polyarteritis nodosa, eosinophilic granulomatosis lymphomas and leukaemias).

The diagnosis of EoE, as per the Guidelines of the Italian Society of Gastroenterology (SIGE) is defined by the following criteria:

1. Symptoms related to esophageal dysfunction.
2. A number ≥ 15 eosinophils in at least one high power field (EOS/HPF).
3. Presence of an eosinophilic infiltrate confined to the esophagus.
4. Need to exclude all secondary causes of oesophageal hypereosinophilia (e.g. any food allergies or drug reactions, celiac disease, parasitic infections, hypereosinophilic syndromes, inflammatory bowel disease, polyarteritis nodosa, eosinophilic granulomatosis, lymphomas and leukaemias).
5. Response to treatment (elimination nutritional therapy; drug therapy).

EGE should be suspected in persons presenting with chronic and/or recurrent gastrointestinal symptoms with episodes typically lasting 2-4 weeks. The symptomatology varies according to the gastrointestinal tract involved: if the localization is gastric, dyspeptic symptoms are usually present; if the localization is at the level of the small intestine, the following are described: abdominal pain, diarrhoea, meteorism. In general, symptoms are exacerbated by meals and accompanied by weight loss. Occasionally, malabsorption may be associated with steatorrhea, iron deficiency anemia, protein-losing enteropathy, ascites with high eosinophil counts, and even intestinal obstruction. Grossly visible blood in the stool or positive occult blood may be found, and approximately 50% of patients may have concurrent atopic conditions. Peripheral hypereosinophilia, pathologies such as asthma, allergic rhinitis and atopic dermatitis should raise suspicion of a diagnosis of EGE in the presence of chronic gastrointestinal symptoms.

EoE must be suspected both by evaluating the symptoms, which can vary according to the age of the patient; for infants/small children, in fact, we can observe: a reduction in the growth curve, greater difficulty in feeding up to the total refusal of food, nausea, vomiting, abdominal pain; in adults and children/adolescents, on the other hand, the most frequent symptoms are represented by: dysphagia, impact of the bolus, slow progression of the bolus, retrosternal heartburn, regurgitation, vomiting, chest pain, odynophagia, or in case of finding on endoscopic examination of the esophagus of concentric rings, vertical erosions, whitish exudate, edema and stenosis.

EGE should be suspected in persons presenting with chronic and/or recurrent gastrointestinal symptoms with episodes typically lasting 2-4 weeks. The symptomatology varies according to the gastrointestinal tract involved: if the localization is gastric, dyspeptic symptoms are usually present; if the localization is at the level of the small intestine, the following are described: abdominal pain, diarrhoea, meteorism. In general, symptoms are exacerbated by meals and accompanied by weight loss. Occasionally, malabsorption may be associated with steatorrhea, iron deficiency anemia, protein-losing enteropathy, ascites with high eosinophil counts, and even intestinal obstruction. Grossly visible blood in the stool or positive occult blood may be found, and approximately 50% of patients may have concurrent atopic conditions. Peripheral hypereosinophilia, pathologies such as asthma, allergic rhinitis and atopic dermatitis should raise suspicion of a diagnosis of EGE in the presence of chronic gastrointestinal symptoms.

EoE must be suspected both by evaluating the symptoms, which can vary according to the age of the patient; for infants/small children, in fact, we can observe: a reduction in the growth curve, greater difficulty in feeding up to the total refusal of food, nausea, vomiting, abdominal pain; in adults and children/adolescents, on the other hand, the most frequent symptoms are represented by: dysphagia, impact of the bolus, slow progression of the bolus, retrosternal heartburn, regurgitation, vomiting, chest pain, odynophagia, or in case of finding on endoscopic examination of the esophagus of concentric rings, vertical erosions, whitish exudate, edema and stenosis.

The patient, after specialist evaluation for suspected EoE or EGE, should be referred to the PDR, where, depending on the complexity, he will be followed up with external (outpatient) or internal (day hospital or inpatient hospitalization) management by the multidisciplinary team of the structure.

The patient management team, in addition to the essential professional figures such as the gastroenterologist or pediatrician, should also include the following specialists:

• Anatomo-Pathologist;
• allergist;
• Nutritionist

EGE therapy lacks solid scientific evidence and is therefore empirical and based on disease severity. In addition to the reduction of symptoms, some authors have suggested as therapeutic objectives the reduction >50% of peripheral eosinophilia, when present, and the eventual reduction of tissue eosinophilia.

In EoE, the therapeutic goal has been set to reduce the number of eosinophils on esophageal biopsies, which must be < 15/HPF, and to reduce symptoms after 6-12 weeks of therapy. Furthermore, in children, therapy must ensure normal growth and development. In children, where symptoms are mainly caused by inflammation and less by fibrosis, medical treatment alone may be sufficient to improve symptoms, while in adults, the presence of strictures may require endoscopic dilatation .

The nutritional therapies proposed for the treatment of EGE and EoE are essentially of three types: the elemental diet with an amino acid formula, the oligoantigenic diet based on the empirical removal of some food classes (6-food diet) and the one guided by the positivity of the tests allergy. Dietary therapy for 6 weeks can be considered a first line of treatment in mild disease. The possibility of following both a top-down dietary scheme , with elimination of the 6 foods and subsequent gradual reintroduction of one class at a time, followed by endoscopic and histological control, in order to identify the trigger allergens , has also been tested in adult patients. of a step-up schemeinitially eliminating two foods (preferably cow’s milk proteins and gluten/wheat) subsequently restricting the diet in case of lack of efficacy confirmed by endoscopic and histological control.

As regards the diet guided by positive allergy tests, it seems that skin prick tests , specific IgE dosage and atopy patch tests have a limited role, but further studies are needed.

Eosinophilic esophagitis is certainly associated with allergies and even when clinical examinations do not explicitly highlight them, it has been found that by putting the patient on an elemental diet (essential amino acids) the concentration of eosinophils in the esophagus also completely regresses. However, these products are not yet specifically associated with EoE in the NHS

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