Physical examination should include palpation of the thyroid because of the potential for a thyroid mass to cause extrinsic compression of the esophagus, palpation of the abdomen for masses or organomegaly, and a complete neurologic evaluation.9
Laboratory tests should be ordered based on the information obtained from the history and physical. Testing may include thyroid studies to eliminate hypothyroid or hyperthyroid causes of dysphagia, and complete blood count (CBC) with differential to rule out inflammatory or infectious processes.9 While eosinophilia may be present in the differential, it is not a universally accepted marker for establishing the diagnosis of EE.2,5 Stools should be checked for occult blood, because a positive finding may suggest esophageal carcinoma.9
Diagnosis
In the primary care setting, a barium esophagram may be used during the initial workup to evaluate the anatomic structures of the esophagus and to differentiate between a mechanical obstruction and a neuromuscular disorder.1,9 This noninvasive test requires the patient to swallow a radiopaque liquid as x-rays are taken.
The gold standard for diagnosing EE, however, is upper endoscopy with biopsy of the esophageal mucosa.6 Endoscopic findings that indicate EE are atypical of GERD; they may include a narrowed, small-caliber esophagus, concentric mucosal rings, proximal stenosis, linear ulcerations, atrophic changes, and white papules associated with eosinophilic microabscesses.6
Although there is no consensus regarding the number of eosinophils that should be present for an accurate diagnosis of EE, microscopic interpretation of the biopsy from both the proximal and the distal esophageal epithelia5 usually shows 15 or more eosinophils per high-power field.2,11 It has been suggested that mucosal biopsies be taken along the entire length of the esophagus, as eosinophilic infiltration may extend from the proximal to the distal esophagus.2
GERD and trauma induced by medication use may also be associated with esophageal eosinophilic infiltration5; however, eosinophils are usually present only in the distal esophageal mucosa3 and are not as abundant as in EE.7 If endoscopy reveals persistent eosinophilia despite four to eight weeks’ treatment with a PPI, the diagnosis of EE is confirmed.2
Treatment
Treatment for EE is still under investigation. Research has examined the association between EE and food allergies or aeroallergens.4 Evaluation by an allergist using skin prick tests or RAST is recommended in the adult patient to help determine the source of the underlying inflammation.5,7 Eliminating any identified allergen should help alleviate symptoms.4
For patients in whom no source of inflammation can be identified, treatment with 1.0 to 2.0 mg/kg/d of oral prednisone for acute exacerbations has been shown to significantly improve symptoms and histology12; however, because of the associated risk for adverse systemic effects, long-term use is not recommended.
In many patients, the inhaled corticosteroid fluticasone has also proved successful in reducing EE—associated inflammation.6 Current evidence supports adult dosing between 880 and 1,760 mcg per day for six to eight weeks, administered with a metered-dose inhaler and no spacer. Fluticasone should be sprayed directly into the mouth and swallowed, after which the patient should take nothing by mouth for 30 minutes.13 Prolonged fluticasone use has been associated with esophageal candidiasis.2 There are currently no recommendations regarding its use as maintenance therapy.
Montelukast, a leukotriene receptor antagonist, has also been shown in some studies to reduce the inflammatory process11; however, one study team recently found it to have no therapeutic effect.13
PPIs may be effective for improving EE symptoms even in the absence of GERD because of the reduced gastric acid production,7 but they do not usually improve EE’s histologic features.3
Use of esophageal dilation in patients with EE is controversial because of an associated risk for perforation.14 If this intervention is to be performed, the patient should be treated in advance with oral corticosteroids to reduce esophageal inflammation.15,16 In addition, the endoscopist should start with small-sized dilators and carefully proceed to larger sizes.11 Critics of esophageal dilation argue that the procedure is only a temporary solution and does nothing for the underlying condition.4,8
Regarding endoscopic surveillance, an interval of at least four weeks between interventions is recommended.13
Role of the Primary Care Clinician
Undiagnosed EE can cause the patient discomfort, frustration, and anxiety, as seen in the case study. Many patients with undiagnosed EE have been exposed to unnecessary medical therapy and antireflux surgery.3 Without proper diagnosis and treatment, EE may worsen, causing complications associated with chronic inflammation (ie, esophageal fibrosis and strictures).2,6
The long-term prognosis of EE is unknown at this time.8 The disease is usually chronic, with periods of remission and exacerbation. With an understanding of EE and appropriate therapies, the primary care practitioner can team with the gastroenterologist to provide effective disease management through endoscopic surveillance and intervention for acute exacerbations. Guidelines recommend that patients be closely followed with regular office visits to reassess symptoms, compliance with therapy, and adverse effects, with the goal of preventing complications associated with EE.13