First Licensed Drug for Eosinophilic Esophagitis (EoE) Brings Relief to UK Sufferers

The UK saw the launch of the budesonide orodispersible tablet Jorveza, the only licensed drug for the treatment of the inflammatory disease Eosinophilic Esophagitis (EoE). Only identified in the late 1980s as a disease, this highly debilitating condition is thought to be caused by immune response to food, environmental or other triggers which results in the production and multiplication of eosinophils in the oesophagus.

Sometimes termed ‘asthma of the oesophagus’ with 50% of patients having a history of other allergic conditions, EoE causes pain and difficulties swallowing food (dysphagia) as well as oesophageal food impaction (bolus), whilst children with the disease can fail to thrive and are often mistaken for fussy eaters. Untreated the inflammation can lead to fibrosis and complications

EoE is more common in men than women. It can occur at any age but is more commonly seen in males aged between 30-50. Latest figures put the prevalence of EoE at 28 people per 100,000, with disease rates rising every year. Some experts believe that the condition may be either underdiagnosed or misdiagnosed as other upper GI conditions such as GORD or reflux and, in areas where EoE is actively diagnosed, the rate is often higher. Even when a diagnosis is made, treatment pathways are still unclear, leading to some patients receiving ineffective treatments with poor outcomes.
With the UK launch of Jorveza, clinicians have a medication which is both clinically effective and specifically designed to deliver to the target area of inflammation with the minimum of side effects.

Here Professor Stephen Attwood, Consultant Surgeon and Honorary Professor at Durham University, and the doctor who first identified and highlighted the condition, explains the significance of EoE and the need for greater awareness of the condition and how to correctly diagnosis and treat it.
‘EoE is an extremely unpleasant condition which, if left undiagnosed, is strongly associated with a poor quality of life,’ says Professor Attwood. ‘Depending on the stage of the disease, adults can suffer from food obstruction or endure feelings of choking or pain when swallowing food, whilst children may be branded as fussy eaters or even wrongly diagnosed with eating disorders. Patients often develop eating strategies such as taking lots of water with food, eating very slowly or cutting up food into tiny pieces. They may have to get up from the table to spit food out, or regularly experience choking type symptoms which are very embarrassing.

‘After a while, the simple act of eating becomes fraught with all sorts of physical and psychological barriers and, of course, if you are unable to eat properly you are likely to feel tired or even become malnourished.

‘It is unfortunate then that EoE is still very much an undiagnosed disease at both primary and secondary care level. Any patient reporting pain on eating or swallowing to their GP is most likely to be initially diagnosed with reflux, or GORD and put on PPIs. Whilst PPIs may bring some relief they do not solve the problem and can actually confuse diagnosis.

‘If the patient specifically reports a sensation of sticking food the GP will usually red flag them for an endoscopy but, if there is not specific request to take biopsies to test for EoE, the patient will still go undiagnosed. Overall it is frustrating that despite increasing knowledge of the disease and clinical guidelines, the average time to diagnosis is currently several years.

‘The irony of all this is that, if you look for it correctly, EoE is actually very easy to diagnose. Usually there are little or no eosinophils in the oesophagus (less than 15 per hpf) but in a patient with EoE it is not unusual for there to be literally hundreds per hpf. They are very easy to spot under biopsy, although it is vital a minimum of six biopsies are taken around the oesophagus.

‘When I first took up my post in Northumberland in 2005 I initiated a policy of requesting the pathologist to always look for eosinophils whenever they were examining any oesophageal biopsies. Initially there was some opposition – one consultant said the disease was in my imagination – but as the pathologists started to find EoE more regularly, we began to consider the possibility that EoE is actually more prevalent than we had thought. Our rates in Northumberland, at around 45 per 100,000, are higher than the national average but we believe that this is because we are regularly looking for it.

‘Unfortunately, due to the current situation around treatment pathways, even when the patient receives an EoE diagnosis they may not always receive an effective treatment. This is significant because around 10% of untreated sufferers will go on to develop oesophageal strictures leaving them at risk of multiple dilations, which is extremely exhausting and unpleasant for them.

‘On the current treatment pathways, patients may be offered allergy testing, which is unproven and ineffective or dietary exclusion which, in my opinion, is a non-starter. Alternative dietary strategies are to eliminate the 6 common foods that promote EoE. Even this dietary restriction is very difficult for the patient to adhere to, even with close support from a dietician, but you need take follow up biopsies at six weeks after the re-introduction of every potential allergen foods. This would not only overwhelm the NHS but be very inconvenient for the patient. Another treatment pathway is PPIs which although it may treat some of the symptoms it does not deal with the underlying problem.

‘The good news however is that EoE is not just easy to diagnose but it is also relatively easy to treat with topical steroids. Given correctly, these will suppress inflammation including a complete resolution of all eosinophil activity. They begin to work immediately but symptoms take on average six weeks to resolve depending on disease severity and other factors. Topical steroids can also reverse some of the fibrosis which can mean that strictures may resolve without the need for dilation.
‘The current recommended course of steroid treatment is 12 weeks but >70% of patients will relapse within two years. I believe that, as with asthma, patients may need to stay on long term steroids because they are topical and do not cause side effects elsewhere in the body.

‘Left undiagnosed and untreated, EoE is deeply unpleasant and harmful so it is vital that there is greater awareness of it. A key message for GPs has to be that any patient who presents with pain on eating or feeling that food is sticking in the throat – especially if they have a history of allergic illnesses such as rhinitis asthma and eczema – should be referred for biopsies with a specific request to look for eosinophils. And, when diagnosed, a long-term topical steroid is the only treatment with proven efficacy and lasting outcomes.

‘The take home message is that EoE is easy to diagnosis as long as you are looking for it and easy to treat with steroids.’