A 51-year-old woman with a history of childhood asthma presented with a sensation of food impaction. Upper gastrointestinal endoscopy revealed classic “feline” esophagus, with mucosal rings (Panel A) and an esophageal stricture near the gastroesophageal junction. Biopsy specimens of the proximal and distal esophagus showed extensive mucosal eosinophilic infiltrates (Panel B, hematoxylin and eosin). The distal esophageal stricture was dilated with the use of a balloon dilator. The patient was treated with a fluticasone inhaler (four 220-μg puffs twice daily), with instructions to swallow and to rinse her mouth. During the next 2 months, her symptoms diminished, and the histologic findings improved. Adult onset of eosinophilic esophagitis is still not recognized by many practitioners. This condition is often confused with gastroesophageal reflux disease and is associated with esophageal strictures. The presence of more than 20 eosinophils per high-power field in an esophageal biopsy specimen is strongly suggestive of this diagnosis. Optimal treatment remains unclear.
The first cases of probable eosinophilic esophagitis were reported in the late 1960s to 1970s. The incidence of eosinophilic esophagitis appears to be increasing.
- A population-based study evaluated the incidence of eosinophilic esophagitis in Olmsted County Minnesota over thirty years [29]. The incidence increased significantly during the last three of the five-year intervals examined (from 0.35 per 100,000 population between 1991 and 1995 to 9.45 per 100,000 between 2001 and 2005). The prevalence was estimated to be 55 per 100,000 in 2006.
The majority of affected adults have been men in their 20s or 30s, although later presentations have been described.Among children, the disease is also more common in boys (71 percent in the series described above). In another population-based study, children with eosinophilic esophagitis were significantly more likely to be Caucasian (84 percent compared with 73 percent of the surrounding community as a whole). Patients were also more likely to be male (76 versus 48 percent).
PATHOGENESIS — incompletely understood, Adaptive T-cell immunity driven by type 2 T-helper (Th2) cells, involving interleukin (IL)-13, IL-5, and IL-15 expression appears to play a major role in the pathogenesis of eosinophilic esophagitis.
Genetic factors — A genetic predisposition to eosinophilic esophagitis is supported by evidence of familial clustering. In addition, a possible susceptibility locus has been identified on chromosome 5q22
Eosinophilic esophagitis (EoE) is an allergic disorder characterized by the accumulation of eosinophils in the esophagus. We report association of EoE with variants at chromosome 5q22 encompassing TSLP and WDR36 (rs3806932, combined P = 3.19 x 10(-9)). TSLP is overexpressed in esophageal biopsies from individuals with EoE compared with unaffected individuals, whereas WDR36 expression is unaltered between the two groups. These data implicate the 5q22 locus in the pathogenesis of EoE and identify TSLP as the most likely candidate gene in the region.
Nat Genet. 2010 Apr;42(4):289-91. doi: 10.1038/ng.547. Epub 2010 Mar 7.
Common variants at 5q22 associate with pediatric eosinophilic esophagitis.
CLINICAL MANIFESTATIONS — The clinical manifestations of eosinophilic esophagitis vary with age. Adults and teenagers frequently present with dysphagia and food impactions, whereas in younger children symptoms often include feeding difficulties and abdominal pain.
Clinical manifestations in adults — Common clinical manifestations seen in adults include [5,18,22,29,75-100]:
- Dysphagia
- Food impaction
- Chest pain that is often centrally located and does not respond to antacids
- Gastroesophageal reflux disease-like symptoms/refractory heartburn
- Upper abdominal pain
Clinical manifestations in children — Symptoms in children vary depending in part upon their age [23,105-109]. In one series, the most common presenting symptoms included [23]:
- Feeding dysfunction (median age 2.0 years)
- Vomiting (median age 8.1 years)
- Abdominal pain (median age 12.0 years)
- Dysphagia (median age 13.4 years)
- Food impaction (median age 16.8 years)
ASSOCIATIONS WITH OTHER DISORDERS — There is a strong association of eosinophilic esophagitis with allergic conditions such as food allergies, environmental allergies, asthma, and atopic dermatitis. It has been estimated that 28 to 86 percent of adults and 42 to 93 percent of children with eosinophilic esophagitis have another allergic disease
DIAGNOSIS — The diagnosis of eosinophilic esophagitis should be based upon symptoms, endoscopic appearance, and histological findings. In patients suspected of having eosinophilic esophagitis, the first diagnostic test is typically an upper endoscopy with esophageal biopsies following two months of treatment with a proton pump inhibitor. In addition, other disorders that can cause esophageal eosinophilia, such as gastroesophageal reflux disease (GERD), should be ruled out.
Endoscopy —
- Stacked circular rings (“feline” esophagus) (picture 1): 44 percent
- Strictures (particularly proximal strictures) (picture 2): 21 percent
- Attenuation of the subepithelial vascular pattern: 41 percent
- Linear furrows (picture 3): 48 percent
- Whitish papules (representing eosinophil microabscesses) (picture 1): 27 percent
- Small caliber esophagus: 9 percent
A 14-month-old with failure to thrive and loose stools. Endoscopy demonstrates a thickened furrowed esophagus consistent with eosinophilic esophagitis. These patients commonly have dysphagia with, as well as without, evidence of stricture. Eosinophilic esophagitis is also a common cause of dysphagic in atopic school-aged children. Histology would demonstrate sheets of eosinophils in the lamina propria.
Courtesy of Karen Murray, MD.
Upper endoscopy in a 36-year-old man with dysphagia. Multiple rings are present in the proximal to mid esophagus giving it the appearance of a trachea. Small whitish papules are also visible representing eosinophilic abscesses on histology. The patient's symptoms responded to oral fluticasone.
Courtesy of Eric D Libby, MD.
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