Australian Doctor 14th June Issue | Page 20

14 JUNE 2024 ausdoc . com . au

CLINICAL PRESENTATION

ADULTS present with symptoms of dysphagia ( 60-100 % of patients ), food impaction ( more than 25 %, see figure 2 ), non-cardiac chest pain ( 8-44 %) and heartburn ( 30-60 %). 13 , 20 The clinical presentation of EoE in children varies with age , and they may present with non-specific symptoms . 13 Babies and toddlers may present with feeding difficulties , faltering growth and vomiting . 6 Primary school – aged children may present with symptoms of gastro-oesophageal reflux , and adolescents present with food impaction , chest pain and dysphagia . 7 , 21 Children and young to middle-aged adults with food bolus impaction more often than not have EoE .

Acute complications of EoE include mucosal tears or oesophageal perforation from food impaction or endoscopic removal of food , which may be the initial presentation of this condition . 2

As many patients have had longstanding symptoms , dysphagia is often under-reported . 22 Patients often describe adaptive mechanisms , such as drinking a significant amount of water postprandially , eating slowly , swallowing multiple times and avoiding ‘ difficult ’ foods . 13 The acronym IMPACT ( see box 2 ) has been described to characterise these behaviours . 22

While symptoms are required to meet the diagnostic criteria of EoE , the symptom of dysphagia does not correlate well with mucosal inflammation ; this may be due to under-reporting or the chronicity of the condition . 23

Adults who have anxiety related to having a choking episode report more severe dysphagia and troublesome swallowing . 23 Those with EoE who also have generalised anxiety or other anxiety conditions have more severe symptoms as they may demonstrate hypervigilance around food . 23

INVESTIGATIONS

Endoscopic features of eosinophilic oesophagitis

CHARACTERISTIC endoscopic findings are highly suggestive , but not diagnostic and can be localised or present throughout the entire oesophagus . 24 Features consistent with EoE include rings ( trachealisation ), strictures , plaques / exudates , furrows and oedema ( decreased vasculature ). 13 Crepe-paper oesophagus is described if the mucosa tears because of the passage of the endoscope . 13

The presence of more than one endoscopic finding of EoE has a sensitivity and specificity of 87 % and 47 %, respectively . 24 , 25 Endoscopic findings of EoE are reported using a standardised EREFS score ([ o ] Edema , Rings , Exudates , Furrows and Strictures ; see box 3 ). 26

Figure 1 . Pathophysiology of eosinophilic oesophagitis .

Figure 2 . Endoscopic image of patient with oesophageal food bolus obstruction from a grape in the setting of eosinophilic oesophagitis .

Box 1 . Factors implicated in the development of eosinophilic oesophagitis

• Environmental factors : — Early childhood exposure to pathogens , resulting in decreased immune tolerance . — Caesarean delivery . — Exposure to antibiotics , thus altering the microbiome .

• Other : — A decline in the frequency of Helicobacter pylori . — An increase in gastro-oesophageal reflux disease , and associated increase in PPI use .

It is important to note that EoE may affect the oesophagus in a patchy distribution ; the American College of Gastroenterology therefore recommends several biopsies ( about six from multiple levels ) to improve the diagnostic yield . 6 The oesophagus has a normal appearance on endoscopy in up to one-quarter of patients with EoE , so tri-level biopsies are required in all patients with symptoms of dysphagia . 6

Histological features of eosinophilic oesophagitis

The hallmark histological feature diagnostic of EoE is increased intraepithelial eosinophils ( 15 or more / high-powered field [ hpf ], see figure 4 ). This may be associated with an acanthotic epithelium ( indicating an increase in total epithelial thickness ), basal zone hyperplasia and the presence of micro abscesses with deposition of eosinophil granule proteins extracellularly . 27 Endoscopic features of EoE have been shown to correlate with histological inflammation . 24 The EREFS score is greater in patients with active histological disease than in those in remission , but it does not reliably predict histological remission . 24

Radiographic features of eosinophilic oesophagitis

A barium oesophagram / swallow ( see figure 5 ) may be a helpful adjunct for patients with EoE ; however , it exposes the patient to ionising radiation . The barium swallow allows a global examination of swallowing function and motility and can assess for oesophagitis and strictures . 28 If performed before a gastroscopy , a barium swallow can be helpful in identifying features consistent with EoE , including a ringed oesophagus and a small-calibre oesophagus .

Box 2 . Adaptive mechanisms : IMPACT

• Imbibe fluids with meals .

• Modify food ( puree / cut into small pieces ).

• Prolong mealtimes .

• Avoid hard-textured foods .

• Chew excessively .

• Turn away tablets / medication . Source : Hirano I et al 2020 22

Doc James / CC BY : bit . ly / 3G7pDJX

This allows the gastroenterologist to prepare by ensuring that a paediatric gastroscope is available . 28 Additional modalities , such as CT , may reveal findings suggestive of EoE , such as a diffusely thickened oesophageal wall . 28 In addition , endoscopic ultrasound has been demonstrated to show features of fibrosis and thickening of the oesophageal wall as demonstrated by a loss of normal echo layers of the oesophagus in EoE . 28

DIAGNOSIS

THE diagnostic criteria include symptoms of oesophageal dysfunction ( dysphagia , food bolus impaction , heartburn and / or non-cardiac chest pain ) with histological biopsies demonstrating 15 or more eosinophils per hpf in the absence of alternative causes ( see box 4 ). 4 Symptoms or endoscopic appearance alone are insufficient to either

IL-33 = Interleukin 33 , TSLP = thymic stromal lymphopoietin

diagnose EoE or to assess the response to treatment ; histological examination is always required . While oesophageal reflux is associated with oesophageal eosinophilia , it is rare to find eosinophil counts greater than 10 / hpf . 29

Historically , guidelines recommended a trial of a PPI as eosinophilia may be seen in reflux disease ; however , the AGREE ( Appraisal of Guidelines for Research and Evaluation II ) consensus confirmed that patients did not require a non-response to a PPI in order to establish the diagnosis of EoE . PPI-responsive EoE was indistinguishable from non- PPI responsive EoE on clinical , endoscopic , histological and genetic grounds . 30 , 31 Instead , PPI therapy is utilised as a treatment option for EoE , with the mechanisms of action involving more than acid suppression alone . A trial of PPI is therefore no longer required as a diagnostic

13 , 31 tool .

A clinical severity index ( I-SEE ) has recently been published but is yet to be validated . The I-SEE has three components : first , symptoms and complications ; second , inflammatory features ; and third , fibrostenotic features . It is completed at diagnosis and subsequent appointments . 32 The I-SEE is a useful tool to categorise patients as having inactive , mild , moderate or severe disease . 32 The I-SEE tool also facilitates doctor / patient understanding and helps to monitor the condition over time . 32

Differential diagnosis

Oesophageal eosinophilia is present in a number of other conditions , and it is important to consider alternative gastrointestinal and systemic conditions that may account for its presence . 33 These can include eosinophilic gastroenteritis , Crohn ’ s disease , drug hypersensitivity , connective tissue diseases , infections ( fungal , viral and parasitic ), hypereosinophilic syndrome and graftversus-host disease , among others ( see box 5 ). 33 Pathology testing is beneficial to exclude coeliac disease ; infection ; or rarer conditions , such as hypereosinophilic syndrome ( persistently elevated eosinophils greater than 1.5x10 9 for six months with associated organ damage ). 34 Endoscopic evaluation with histological analysis is also essential to distinguish between the differential diagnoses .

There is an association between non-specific functional motility disorders — such as ineffective oesophageal motility and fragmented peristalsis — in patients with EoE , but major motility disorders are rare . Therefore , high-resolution oesophageal manometry can also be