eosinophilic esophagitis is a chronic inflammatory disease of the esophagus.
The inflammatory process is supported by an immune-mediated reaction , in which a large number of eosinophils , a particular type of white blood cell , intervene .
The causes of eosinophilic esophagitis are still unknown, but the inflammation may be due to the combination of genetic and environmental factors. Often, this condition is associated with food antigen- induced allergic syndromes . Eosinophilic esophagitis can occur in both children and adults, predominantly males.
People with eosinophilic esophagitis most commonly experience dysphagia, food bolus occlusion , gastroesophageal reflux, and heartburn ( heartburn ). Over time, inflammation of the esophagus can lead to chronic narrowing ( stenosis ) of the esophagus .
Diagnosis of eosinophilic esophagitis is based on endoscopy of the upper digestive tract associated with biopsy of the esophageal mucosa .
In most patients, corticosteroid therapy , elimination of food antigens from the diet and possible endoscopic dilation of the esophagus allow good control of symptoms.
Eosinophilic esophagitis is a chronic disease which can affect the esophageal mucosa at any age.
The inflammation underlying the disorder is immune-mediated (i.e. caused by the immune system , according to the mechanism of autoimmune diseases ).
The symptoms of esophagitis Eosinophilic may include: pain or difficulty swallowing (dysphagia), reflux, heartburn and vomiting . In some patients, the esophagus may narrow to the point that food is blocked.
The exact causes of eosinophilic esophagitis are still not fully understood. However, the association of inflammation of the esophagus with allergic and / or atopic diseases is known (in about 70% of cases, eosinophilic esophagitis is concomitant with allergic rhinitis , bronchial asthma or atopic dermatitis ).
In people with genetic predisposition , eosinophilic esophagitis is mainly induced by the ingestion of particular food antigens (eg milk , eggs , soy, etc.), but the reaction can also be triggered by environmental allergens .
Eosinophilic esophagitis is associated with dysfunction of the esophagus resulting from predominantly eosinophilic inflammation.
As regards the maintenance of the inflammatory process, a significant role is played by the overexpression of some mediators (including interleukins and chemokines), which “recall” the eosinophils (through chemotaxis) and regulate their activation. This explains the high density of these immune cells at target tissue levels and the resulting injuries.
Eosinophilic esophagitis: what are eosinophils?
Eosinophilic esophagitis owes its name to the fact that the disease is characterized by a strong accumulation of eosinophils (also called eosinophilic granulocytes ) in the squamous epithelium of the esophagus; these cells are usually involved in immune responses to allergens or parasitic infestations .
In the case of eosinophilic esophagitis, large numbers of eosinophils infiltrate the epithelial tissue of the esophagus, causing a variety of gastrointestinal symptoms such as reflux, frequent vomiting, difficulty swallowing and abdominal pain .
Who is most at risk
- Eosinophilic esophagitis can begin at any time, but occurs mainly in the period from childhood to adulthood. Only on a few occasions, the disease appears for the first time in older people.
- Eosinophilic esophagitis is more common in men. The prevalence in men compared to women is 3: 1.
- Eosinophilic esophagitis is often associated with allergic syndromes. The condition is more likely to occur in people with bronchial asthmaand food allergies .
- A higher frequency of eosinophilic esophagitis is observed in patients with close family relationships, supporting the hypothesis of a genetic basis.
Symptoms and Complications
Eosinophilic esophagitis is characterized by alternating periods of remission and activity .
Symptoms of eosinophilic esophagitis vary with age and can include pain or difficulty in swallowing ( dysphagia ), reflux, heartburn, and vomiting. In some patients, the esophagus may narrow to the point of obstructing or blocking the passage of the food bolus.
In adults, the most common presenting symptom of eosinophilic esophagitis is difficulty in swallowing ( dysphagia ), especially for solid foods.
Less frequently, eosinophilic esophagitis may be associated with other esophageal disorders, similar to those of gastroesophageal reflux disease , such as heartburn , heartburn (a burning sensation in the sternum), and chest pain . The latter manifestations do not usually respond to proton pump inhibitor drugs .
Eosinophilic esophagitis can also manifest itself with:
- Retrosternal, epigastricand / or abdominal pain ;
- Anorexiaand premature satiety.
Over time, the inflammation also leads to the arrest of the food bolus and the narrowing of the esophageal caliber (or stenosis).
Sometimes, non-esophageal disorders may be present, such as diarrhea , recurrent or chronic laryngitis , recurrent asthma attacks and ab ingestis bronchopneumonia .
During childhood, eosinophilic esophagitis occurs mainly with typical reflux disorders not responsive to usual therapy, such as heartburn and food regurgitation .
Inflammation of the esophagus can also be associated with less specific symptoms, such as:
- Vomiting with frequent presence of mucus;
- Lack of appetite and refusal of food;
- Agitation and crying during the meal;
- Abdominal or chest pain
- Intolerance to some foods;
- Reduced weight gain or weight loss;
- Retrosternal burning;
- Epigastricor abdominal pain.
Complications and concomitant pathologies
- L ‘ chronic inflammationuntreated can lead to narrowing (stricture) of the esophagus.
- People with eosinophilic esophagitis may have manifestations of allergies or other atopic pathologies (such as asthma, allergic rhinitis, eczema,).
Eosinophilic esophagitis is considered by doctors when episodic dysphagia, food-related esophageal occlusion, or non-cardiac chest pain occurs. The diagnosis is made with endoscopy of the upper digestive tract, supported by biopsy.
Complete clinical history
Generally, the first symptoms appear in young adults (20 to 30-35 years), but the age at diagnosis can be highly variable (1-89 years). The disease can also be suspected by the physician when gastroesophageal reflux does not respond to acid-suppressive therapy with proton pump inhibitors (even at high doses).
Endoscopy of the upper digestive tract
The diagnosis of eosinophilic esophagitis is confirmed after performing an upper digestive tract endoscopy with a flexible tube ( esophagogastroduodenoscopy , EGDS).
Endoscopic pictures often associated with eosinophilic esophagitis include:
- Longitudinal narrowing of the esophageal caliber or isolated stenosis (proximal or distal);
- Longitudinal discontinuities of the mucosa along the entire course of the esophagus;
- Exudates or diffuse whitish punctuation of the mucosa;
- Diffuse nodularity and / or graininess;
- Multiple esophageal rings, not completely smoothed by insufflation (feline folds or felinization of the esophagus);
When passing the endoscope, the esophageal mucosa , thanks to its frequent fragility , can bleed or crack.
The radiological study with barium swallow may be indicated as a complementary examination to endoscopy. In addition to confirming the reduction in the caliber of the esophagus, this assessment provides information on the distensibility of the walls.
Biopsy of the esophageal mucosa
During endoscopy, the doctor takes tissue samples to be analyzed under a microscope ( biopsy ). The biopsy examination demonstrates the presence of a significant eosinophilic infiltration (more than 15 eosinophils / at high microscopic magnification) in the squamous epithelium of the esophagus. Biopsy samples are essential to establish the diagnosis of eosinophilic esophagitis, as the appearance of the esophageal mucosa may be apparently normal on endoscopic vision .
In the patient with associated gastrointestinal disorders (eg diarrhea and abdominal pain), tissue sampling from the stomach and duodenum should be added to the biopsy sampling of the esophagus ; this allows to verify the involvement of the eosinophilic infiltration and to exclude other concomitant pathologies.
Number of eosinophils and differential diagnosis
The presence of eosinophilic infiltrate in the squamous epithelium of the esophagus is common to several pathological conditions, including: gastroesophageal reflux disease , parasitosis , Crohn’s disease and lymphoma . The “discriminating” element is the quantity: in eosinophilic esophagitis, eosinophils are numerically superior to these conditions.
For this reason, it is essential for the pathologist to quantify the density of eosinophils per microscopic field. Conventionally, to diagnose eosinophilic esophagitis, it is necessary to find a number equal to or greater than 15 eosinophils for HPF (high power field, i.e. x400 magnification), in association with other characteristics of the eosinophilic infiltrate.
In order to identify the possible factors involved in eosinophilic esophagitis, the doctor may test the patient for food and respiratory allergies , possibly associated with skin tests ( Prick test ) or radioallergoabsorption tests ( RAST ).
In summary: diagnostic criteria of eosinophilic esophagitis
The criteria for establishing the diagnosis of eosinophilic esophagitis are:
- Presence of esophageal symptoms(dysphagia, food bolus occlusion, heartburn and reflux);
- ≥ 15 eosinophils / HPFon histological analysis of the biopsy specimen;
- Absence of clinical responseto full-dose proton pump inhibitor
Treatment options for eosinophilic esophagitis include various interventions. In most cases, topical corticosteroid therapy, the elimination of food antigens from the diet and possible endoscopic dilatation allow good control of symptoms.
Changes in diet
- If a food allergy is strongly suspected based on the patient’s medical history and is found with objective allergy tests, the doctor may indicate a targeted elimination diet.
- In the absence of specific sensitizations to foods, the diet of the patient with eosinophilic esophagitis can provide for the empirical elimination of the main allergens(milk, egg, wheat , soy, peanuts and fish ), approximately for 8-12 weeks.
- In patients with multiple allergies, however, the doctor can prescribe an elementary (amino acid) diet, with the exclusion of all whole proteins .
The decision to use a specific dietary approach is more effective in children than adults.
Management of eosinophilic esophagitis in childhood
In children, initial therapy of eosinophilic esophagitis can:
- Be the deprivation dietbased on specific allergy tests;
- Provide for the eliminationof the most common allergenic foods .
Generally, the results of this surgery are satisfactory and, in many cases, limit the need for corticosteroid therapy.
The most useful drugs for controlling the symptoms of topical esophagitis are topical corticosteroids (such as fluticasone propionate and budesonide ).
The route of administration with the multi-dose oral inhaler is the preferred one (in consideration of the absence of side effects secondary to systemic therapy and the high topical anti-inflammatory efficacy). In case of eosinophilic esophagitis, it is possible to spray the drug in the mouth and swallow it: in this way, the drug coats the esophagus and does not enter the lungs . Alternatively, the budesonide can be mixed with a sugar substitute , before being swallowed.
After taking, the patient should rinse the mouth with water (to avoid a fungal infection, such as candidiasis ) and do not drink or eat for at least 30 minutes (to maximize the topical anti-inflammatory action in the esophageal mucosa ).
Topical corticosteroids for the treatment of eosinophilic oesophagitis are usually given for 6 to 8 weeks, 30 minutes before breakfast and 30 minutes before dinner. This therapy must be repeated when symptoms recur.
- When people experience recurrent episodes of dysphagia and have significant stenosis, doctors can intervene using a hydrostatic balloonor solid esophageal dilator . Endoscopic esophageal dilation is performed by experienced endoscopists with extreme caution to prevent esophageal lacerations or perforations.
- In the case of food bolus occlusion, endoscopic dilatationallows rapid unblocking of the esophagus.
Complications of untreated eosinophilic esophagitis include fibrosis of the lamina propria and the formation of narrowings that increase the risk of food occlusion and perforation of the esophagus .
Proper management of the disease allows the patient to maintain a good quality of life.