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Background
For many years, this disease was thought to be a variant of reflux disease. It is now known to be a distinct condition and thought to be an allergic reaction, predominately occurring in children. The gold standard for diagnosis is a normal 24 hour pH probe study despite severe esophagitis. This condition is part of the disease spectrum of eosinophilic gastroenteritis.
Outline
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS EE INCIDENCE Increasingly common AGE RANGE-MEDIAN All ages but more common in children May have slight male predominance
DISEASE ASSOCIATIONS CHARACTERIZATION ASTHMA >70%
Family history of allergy in 30-50%
Peripheral esoinphilia in 50%
Abnormal skin testing in >70%GERD
*Departments of Pathology and Medicine, Dallas Department of Veterans Affairs Medical Center †The University of Texas Southwestern Medical Center at Dallas, Texas.
Eosinophilic esophagitis has acquired the status of an independent adult condition only in the last few years, and criteria have been proposed to differentiate it from the eosinophilic infiltrates seen in a portion of patients with gastroesophageal reflux disease (GERD). Recent data, including the article summarized above, suggest that the interaction between GERD and eosinophilic esophagitis can be complex, and that the notion of establishing a clear distinction between the 2 disorders may be simplistic. Furthermore, the high frequency of GERD in adult patients with eosinophilic esophagitis suggests that there may be more than a chance association.
This report discusses the possible relationship between these 2 conditions, including the hypothesis that GERD may contribute to or cause eosinophilic esophagitis.
We also suggest that the differential diagnosis of eosinophilic and peptic esophagitis should not be based exclusively on arbitrary counts of eosinophils per high-power field; rather, there should always be a thoughtful consideration of histopathologic and clinical data.
Department of Medicine, Dallas Department of Veterans Affairs Medical Center, and The University of Texas Southwestern Medical Center at Dallas, Dallas, Texas 75216, USA.
Recent data suggest that the interaction between gastroesophageal reflux disease (GERD) and eosinophilic esophagitis can be complex, and that the notion of establishing a clear distinction between the two disorders may be too simplistic.
There are at least four situations in which GERD might be associated with esophageal eosinophils: (a) GERD causes esophageal injury that results in a mild eosinophilic infiltration, (b) GERD and eosinophilic esophagitis coexist but are unrelated, (c) eosinophilic esophagitis contributes to or causes GERD, or (d) GERD contributes to or causes eosinophilic esophagitis.
The high frequency of GERD described in adult patients with eosinophilic esophagitis suggests that there may be more than a chance association between the two disorders.
This report discusses potential mechanisms for the complex interaction between GERD and eosinophilic esophagitis. We hope that this information will serve as a conceptual basis for future studies on the relationship between the two disorders. Whereas there are a number of plausible mechanisms whereby GERD might contribute to the accumulation of eosinophils in the esophageal epithelium, it seems prudent to recommend a clinical trial of proton pump inhibitor (PPI) therapy even when the diagnosis of eosinophilic esophagitis seems clear-cut.
Furthermore, we suggest that a favorable response to PPI therapy does not preclude a diagnosis of eosinophilic esophagitis.
PATHOGENESIS CHARACTERIZATION Probable allergic basis Etiologic agent remains unknown
Suspected food allergen or inhaled or swallowed airborne allergen
Intraepithelial Langerhans cells recognize antigen and stimulate T cell proliferation with production of eosinophil cytokines
LABORATORY/
RADIOLOGIC/
OTHER TESTSCHARACTERIZATION RADIOLOGIC
Idiopathic eosinophilic esophagitis in adults: the ringed esophagus.
Zimmerman SL, Levine MS, Rubesin SE, Mitre MC, Furth EE, Laufer I, Katzka DA.
Department of Radiology, Hospital of University of Pennsylvania, 3400 Spruce St, Philadelphia, PA 19104, USA.
Radiology. 2005 Jul;236(1):159-65. Epub 2005 Jun 27. Abstract quote
PURPOSE: To retrospectively assess the findings of idiopathic eosinophilic esophagitis (IEE) at barium studies and determine the frequency of the ringed esophagus in patients with this condition.
MATERIALS AND METHODS: The institutional review board approved all aspects of this retrospective study and did not require informed consent from patients whose records were included in the study. The study was compliant with the Health Insurance Portability and Accountability Act. A review of the pathology and radiology databases at the authors' institution revealed 14 patients (11 men and three women; mean age, 41.3 years) with IEE (defined as more than 20 eosinophils per high-power field in biopsy specimens) who had undergone barium studies and endoscopy. The radiographs were reviewed for strictures, esophagitis, or other abnormalities. The endoscopic reports, clinical records, and laboratory data were also reviewed and compared with the radiographic findings.
RESULTS: Seven of the 14 patients (50%) had a history of allergies, and two of nine patients with complete blood cell counts (22%) had peripheral eosinophilia. Thirteen patients (93%) had dysphagia, six (43%) had food impactions, and six (43%) had reflux symptoms. Ten patients (71%) had a total of 11 strictures at barium studies (two in the upper part of the esophagus, two in the middle part, three in the distal part, one in the middle and distal parts, and three at the gastroesophageal junction). The strictures had a mean length of 5.1 cm. In seven patients (50%), the strictures contained multiple fixed ringlike indentations that produced a ringed esophagus. The ringlike indentations appeared as multiple, fixed, closely spaced, concentric rings traversing the stricture. Four patients (28%) had esophagitis. Of 13 patients who underwent recumbent imaging, 10 (77%) had hiatal hernias and nine (69%) had reflux. Eight of the 10 patients (80%) with strictures underwent endoscopic dilation procedures, which resulted in only temporary relief of dysphagia.
CONCLUSION: The findings suggest that most patients with IEE have esophageal strictures, often with distinctive ringlike indentations that produce a ringed esophagus.LABORATORY MARKERS 24 hour pH probe Normal study
GROSS APPEARANCE/
CLINICAL VARIANTSCHARACTERIZATION GENERAL Endoscopy shows characteristic punctate white surface dots associated with erythema, loss of vascular pattern, ulcers, or ringed trachea-like appearance ENDOSCOPIC APPEARANCE
- Eosinophilic esophagitis: red on microscopy, white on endoscopy.
Straumann A, Spichtin HP, Bucher KA, Heer P, Simon HU.
Department of Gastroenterology, Kantonsspital Olten, Roemerstrasse 7, CH-4600 Olten, Switzerland.
Digestion. 2004;70(2):109-16. Epub 2004 Sep 20. Abstract quote
BACKGROUND/AIMS: The presenting symptom of eosinophilic esophagitis, a chronic T(H)2-type inflammatory disease, is uniform dysphagia attacks. Histology reveals a dense mucosal infiltration with eosinophils. Unfortunately, endoscopic findings are often unremarkable or misleading. This study characterizes the endoscopic manifestations of eosinophilic esophagitis and analyzes the nature and clinical features of the frequently observed white alterations.
METHODS: Thirty adult patients (22 males, 8 females; mean age 40.6 years) with previously confirmed EE prospectively underwent a structured interview, physical examination, laboratory tests and upper endoscopy with histomorphometric examination of the esophageal mucosa.
RESULTS: On endoscopy, all patients showed mucosal abnormalities in the esophagus. Findings included an unspectacular loss of vascular pattern (93.3%) and white exudates (53.3%). Biopsies demonstrated significantly increased eosinophilia in the white exudates (108.4 vs. 14.0 cells/hpf). A significant correlation was found between white exudates and dysphagia frequency (<1 attack/week = 20%; >1 attack/week = 70%).
CONCLUSION: Eosinophilic esophagitis evokes at least 12 different signs resulting in an individually unique endoscopic pattern, but no disease-specific picture. White exudates correspond to foci of dense eosinophilic infiltration reflecting inflammatory activity and are associated with significantly more frequent dysphagia attacks. Both the lack of a typical endoscopic picture as well as the heterogeneity of the eosinophilic infiltration impede diagnosis.
- White specks in the esophageal mucosa: An endoscopic manifestation of non-reflux eosinophilic esophagitis in children.
Lim JR, Gupta SK, Croffie JM, Pfefferkorn MD, Molleston JP, Corkins MR, Davis MM, Faught PP, Steiner SJ, Fitzgerald JF.
Division of Pediatric, Gastroenterology/
Hepatology/Nutrition, Division of Pediatric Pathology, James Whitcomb Riley Hospital for Children, Indiana University School of Medicine, Indianapolis, Indiana 46202-5225, USA.
Gastrointest Endosc. 2004 Jun;59(7):835-8. Abstract quote
BACKGROUND: White specks in the esophageal mucosa have been observed in children with eosinophilic esophagitis. The aim of this study was to determine the relationship between white specks in the esophageal mucosa and allergic (non-reflux) eosinophilic esophagitis.
METHODS: Endoscopic data, pH probe results, and histopathology reports for children with esophageal endoscopic abnormalities seen during a 17-month period were reviewed. Eosinophilic esophagitis was grouped according to the number of eosinophils per high power field (non-allergic, <15 eosinophils/high power field; allergic, > or =15 eosinophils/high power field).
RESULTS: Of 1041 endoscopies performed during the study period, 153 revealed evidence of eosinophilic esophagitis. Of these 153, 61 had fewer than 15 eosinophils/high power field and 92 had 15 or more eosinophils/high power field. At 31 of the 153 procedures, white specks were noted in the esophageal mucosa. The sensitivity of white specks in the esophageal mucosa for allergic eosinophilic esophagitis was only 30%, but the specificity was 95%. pH probe testing was performed in 21 patients with white specks and was normal in all.
CONCLUSIONS: This report describes a new endoscopic finding associated with allergic eosinophilic esophagitis in children. Eosinophilic esophagitis tends to be severe when white specks are present (> or =15 eosinophils/high power field) and is not associated with pathologic gastroesophageal reflux, as demonstrated by pH probe testing.
ADULTS
- Eosinophilic esophagitis in adults, an emerging cause of dysphagia. Description of 9 cases.
[Article in English, Spanish]
Lucendo Villarin AJ, Carrion Alonso G, Navarro Sanchez M, Martin Chavarri S, Gomez Senent S, Castillo Grau P, Pascual Turrion JM, Gonzalez Sanz-Agero P.
Service of Digestive Diseases, Hospital Universitario La Paz, Madrid, Spain.
Rev Esp Enferm Dig. 2005 Apr;97(4):229-39. Abstract quote
BACKGROUND: Eosinophilic esophagitis is a rare condition mainly affecting children, although the number of cases reported in adults is on the increase. It is characterized by intense infiltration of eosinophilic leukocytes in the esophageal mucosa, without involvement of other sections of the alimentary canal.
MATERIAL AND METHODS: Over the past year, following the performance of endoscopies and biopsies, our service identified nine patients who were diagnosed with suffering from this disorder. Each patient sought medical help for episodes of long-term, self-limited dysphagia or food impaction in the alimentary canal.
RESULTS: Endoscopy revealed esophageal stenosis in the form of simultaneous contraction rings or regular stenosis. In six cases, the manometric study showed a nonspecific motor disorder of severe intensity affecting the esophageal body, and another patient had a disorder characterized by the presence of simultaneous waves and secondary peristaltic waves in the three thirds of the organ. These disorders are presumably due to eosinophilic infiltration of the muscular layer or ganglionar cells of the esophagus, and account for symptoms in these patients. Although the etiopathogenesis of this illness is uncertain, it is clearly an immunoallergic manifestation.
CONCLUSIONS: As the number of diagnosed cases is on the increase, eosinophilic esophagitis is in adults a specific entity within the differential diagnosis of dysphagia in young males with a history of allergies. Eosiniphilic esophagitis responds in a different number of ways to therapies used. We successfully used fluticasone propionate, a synthetic corticoid applied topically, which proved to be efficient in the treatment of this illness by acting on the pathophysiological basis of the process. It does not have any adverse effects, thus offering advantages over other therapies such as systematic corticoids or endoscopic dilations.
Gastrointest Endosc. 2004 Mar;59(3):355-61. Abstract quote
BACKGROUND: Eosinophilic esophagitis is an inflammatory condition in which there is dense eosinophilic infiltration of the surface lining of the esophagus. Reports of eosinophilic esophagitis pertain almost exclusively to pediatric populations. However, eosinophilic esophagitis is emerging as a clinical affliction of adults. This report describes the clinical and endoscopic findings of eosinophilic esophagitis in the largest cohort of adult patients reported to date.
METHODS: Twenty-nine patients (21 men, 8 women; mean age 35 years) with documented eosinophilic esophagitis (>/=15 eosinophils per high-power field in biopsy specimens) and a significant history of chronic dysphagia for solid food (24 patients) were evaluated clinically and endoscopically during a 3-year period (1999-2002). Fourteen patients (48%) had a history of asthma, environmental allergy, or atopy. In a subset of 15 patients, the diagnostic accuracy of endoscopy was compared with that of barium contrast esophagography.
RESULTS: Twenty-seven patients (93%) had abnormal endoscopic findings; 25 (86%) had unique esophageal structural changes, associated with a preserved mucosal surface, that were highly atypical for acid reflux injury. Structural alterations seen in adult patients with eosinophilic esophagitis may occur in combination or as a primary characteristic, e.g., uniform small-caliber esophagus, single or multiple corrugations (rings), proximal esophageal stenosis, or 1 to 2 mm whitish vesicles scattered over the mucosal surface. Barium contrast radiography combined with swallow of a barium-coated marshmallow identified 10 (67%) of the primary features observed endoscopically in 15 patients. However, radiography failed to detect other features noted at endoscopy (e.g., only 3/6 patients with proximal stenosis, 5/9 patients with concentric rings and none of 4 patients with small caliber esophagus). Eight of the 29 patients (20%) had a history of chronic heartburn. Twelve patients had been treated with a proton pump inhibitor and only 3 reported some improvement in the severity of dysphagia.
CONCLUSIONS: Relatively young age, a history of chronic dysphagia for solid food, and endoscopic detection of unique structural alterations atypical for GERD in an adult patient should prompt a suspicion of EE and subsequent biopsy confirmation. Acid reflux appears to have a secondary role in eosinophilic esophagitis. In an uncontrolled comparison, endoscopy was superior to barium contrast radiography for the diagnosis of eosinophilic esophagitis. The incidence of eosinophilic esophagitis in adults appears to be increasing.
The spectrum of pediatric eosinophilic esophagitis beyond infancy: a clinical series of 30 children.
Orenstein SR, Shalaby TM, Di Lorenzo C, Putnam PE, Sigurdsson L, Kocoshis SA.
Division of Pediatric Gastroenterology, University of Pittsburgh School of Medicine, and Children's Hospital of Pittsburgh, Pennsylvania 15213, USA.
Am J Gastroenterol 2000 Jun;95(6):1422-30 Abstract quote
OBJECTIVES: Eosinophilic esophagitis, previously confused with esophageal inflammation due to gastroesophageal reflux, has recently begun to be distinguished from it. We undertook this analysis of our large series of children with the condition to clarify its spectrum: its presenting symptoms; its relation to allergy, respiratory disease, and reflux; its endoscopic and histological findings; and its diagnosis and therapy.
METHODS: We analyzed the details of our clinical series of 30 children with eosinophilic esophagitis, defining it as > or =5 eosinophils per high power field in the distal esophageal epithelium. Retrospective chart review was supplemented by prospective, blinded, duplicate quantitative evaluation of histology specimens, and by telephone contact with some families to clarify subsequent course. Presentation and analysis of the series as a whole is preceded by a case illustrating a typical presentation with dysphagia and recurrent esophageal food impactions.
RESULTS: Presenting symptoms encompass vomiting, pain, and dysphagia (some with impactions or strictures). Allergy, particularly food allergy, is an associated finding in most patients, and many have concomitant asthma or other chronic respiratory disease. A subtle granularity with furrows or rings is newly identified as the endoscopic herald of histological eosinophilic esophagitis. Histological characteristics include peripapillary or juxtaluminal eosinophil clustering in certain cases. Association with eosinophilic gastroenteritis occurs, but is not common. Differentiation from gastroesophageal reflux disease is approached by analyzing eosinophil density and response to therapeutic trials. Therapy encompasses dietary elimination and anti-inflammatory pharmacotherapy.
CONCLUSION: Awareness of the spectrum of eosinophilic esophagitis should promote optimal diagnosis and treatment of this elusive entity, both in children and in adults.
PEDIATRIC
- The spectrum of pediatric eosinophilic esophagitis beyond infancy: a clinical series of 30 children.
Orenstein SR, Shalaby TM, Di Lorenzo C, Putnam PE, Sigurdsson L, Mousa H, Kocoshis SA.
Division of Pediatric Gastroenterology, University of Pittsburgh School of Medicine, and Children's Hospital of Pittsburgh, Pennsylvania 15213, USA.
Am J Gastroenterol. 2000 Jun;95(6):1422-30. Abstract quote
OBJECTIVES: Eosinophilic esophagitis, previously confused with esophageal inflammation due to gastroesophageal reflux, has recently begun to be distinguished from it. We undertook this analysis of our large series of children with the condition to clarify its spectrum: its presenting symptoms; its relation to allergy, respiratory disease, and reflux; its endoscopic and histological findings; and its diagnosis and therapy.
METHODS: We analyzed the details of our clinical series of 30 children with eosinophilic esophagitis, defining it as > or =5 eosinophils per high power field in the distal esophageal epithelium. Retrospective chart review was supplemented by prospective, blinded, duplicate quantitative evaluation of histology specimens, and by telephone contact with some families to clarify subsequent course. Presentation and analysis of the series as a whole is preceded by a case illustrating a typical presentation with dysphagia and recurrent esophageal food impactions.
RESULTS: Presenting symptoms encompass vomiting, pain, and dysphagia (some with impactions or strictures). Allergy, particularly food allergy, is an associated finding in most patients, and many have concomitant asthma or other chronic respiratory disease. A subtle granularity with furrows or rings is newly identified as the endoscopic herald of histological eosinophilic esophagitis. Histological characteristics include peripapillary or juxtaluminal eosinophil clustering in certain cases. Association with eosinophilic gastroenteritis occurs, but is not common. Differentiation from gastroesophageal reflux disease is approached by analyzing eosinophil density and response to therapeutic trials. Therapy encompasses dietary elimination and anti-inflammatory pharmacotherapy.
CONCLUSION: Awareness of the spectrum of eosinophilic esophagitis should promote optimal diagnosis and treatment of this elusive entity, both in children and in adults.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL NOTE: These changes are indicative of the disease only after documented anti-GERD therapy
Large numbers of intraepithelial eosinophils (>20/hpf) with extensive eosinophil degranulation
Preferential localization of eosinophils in upper half of epithelium
Surface eosinophil microabscesses
Surface slough of necrotic keratinocytes admixed with eosinophils
Long linear extent of esophagitis
Pathologic changes more severe in upper and mid-esophagus than near GE junctionIncrease in intraepithelial CD3 and CD8 lymphocytes and CD1a antigen presentin cells
- Eosinophilic esophagitis in adults: distinguishing features from gastroesophageal reflux disease: a study of 41 patients.
Parfitt JR, Gregor JC, Suskin NG, Jawa HA, Driman DK.
Department of Pathology, London Health Sciences Centre and University of Western Ontario, London, Ontario, Canada.
Mod Pathol. 2006 Jan;19(1):90-6. Abstract quote
Eosinophilic esophagitis in adults is a recently described entity occurring in young males with dysphagia, in whom esophageal biopsies show eosinophilic infiltration.
This study defines the clinical and histological features of patients with eosinophilic esophagitis, distinguishing it from gastroesophageal reflux disease. Esophageal biopsies from patients with dysphagia or esophagitis were reviewed blindly, and assessed for: epithelial eosinophil counts, presence of eosinophilic microabscesses, edema, basal zone hyperplasia, lamina propria papillae elongation, eosinophils and fibrosis.
Clinical and endoscopic findings were obtained. Eosinophilic esophagitis was diagnosed with epithelial eosinophils > or = 15 in > or = 2 high-power fields (hpfs) or > or = 25 in any hpf. Analysis was performed with Mann-Whitney, chi2 and ANOVA tests. Of 157 cases, 41 had eosinophilic esophagitis. Male gender (81%) and age < or = 45 (54%) were commoner in patients with eosinophilic esophagitis (P = 0.001, 0.010, respectively). Dysphagia was more common in eosinophilic esophagitis patients (63%, P < 0.001); heartburn was more common in noneosinophilic esophagitis patients (53%, P < 0.001). Endoscopic rings were more common in eosinophilic esophagitis patients (27%, P = 0.023); hiatus hernia was more common in noneosinophilic esophagitis patients (11%, P = 0.022). Eosinophils were more numerous in eosinophilic esophagitis biopsies (mean 39/hpf, P < or = 0.001). Only eosinophilic esophagitis biopsies had eosinophilic microabscesses (42%, P < or = 0.001). Edema, basal zone hyperplasia, lamina propria papillae elongation and lamina propria eosinophils were commoner in eosinophilic esophagitis (P < or = 0.001-0.002), while lamina propria fibrosis was specific for eosinophilic esophagitis (39%, P < 0.001).
Eosinophilic esophagitis is a disease with a predilection for young males with dysphagia and rings on endoscopy. Biopsies in eosinophilic esophagitis have high epithelial eosinophil counts, averaging nearly 40/hpf. Increased awareness of eosinophilic esophagitis is necessary, since treatment with allergen elimination or anti-inflammatory therapy may be more effective than acid suppression.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES ALLERGIC ESOPHAGITIS
Allergic esophagitis in children: a clinicopathological entity.
Walsh SV, Antonioli DA, Goldman H, Fox VL, Bousvaros A, Leichtner AM, Furuta GT.
Department of Pathology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, USA.
Am J Surg Pathol. 1999 Apr;23(4):390-6. Abstract quote
Infiltration of esophageal epithelium by eosinophils is seen in reflux esophagitis and allergic gastroenteritis.
This study was performed to identify differences between patients with acid reflux esophagitis and those with non-acid reflux, possibly allergic, esophagitis. Intraepithelial eosinophils were demonstrated in posttherapy esophageal biopsy specimens in 28 children treated for gastroesophageal reflux disease (GERD). These patients were divided into three groups based on their response to treatment and the results of esophageal pH probe monitoring.
Eleven patients (Group A) had incomplete clinical response and normal pH probe monitoring results. Ten patients (Group B) had incomplete response but did not have pH probe monitoring. These two groups formed the index population. Seven patients (Group C) had clinical improvement with GERD therapy and abnormal pH probe monitoring characteristic of GERD; they constituted the control population. Clinical, laboratory, and pathologic features were evaluated to detect differences between index and control populations. Dysphagia, food impaction, failure to thrive, peripheral eosinophilia, and abnormal allergen skin test results were detected only in Group A and B patients. Biopsy specimens of the distal 9 cm of the esophagus, after GERD therapy, contained larger numbers of eosinophils in Groups A and B than in Group C as shown on high-power fields (HPF) (A: 31/HPF +/- 19.5; B: 28/HPF +/-23.7; versus C: 5/HPF +/-6.7; p = 0.009). Eosinophil aggregates were identified only in Groups A and B (p = 0.07). Eosinophils located preferentially in the superficial layers of the squamous epithelium were noted only in Groups A and B (p = 0.02). Group A and B patients demonstrated clinical improvement when given antiallergic therapy.
The authors identified a group of pediatric patients characterized by an allergic history, lack of adequate response to GERD therapy, normal esophageal pH probe monitoring results, and large numbers of eosinophils in esophageal biopsy specimens obtained after GERD treatment.
On the basis of these features, the authors propose that these patients represent examples of allergic esophagitis.GERD
- Eosinophilic infiltration of the esophagus: gastroesophageal reflux versus eosinophilic esophagitis in children--discussion on daily practice.
Cury EK, Schraibman V, Faintuch S.
Division of Pediatric Surgery, Department of Surgery, Federal University of Sao Paulo, Sao Paulo, Brazil.
J Pediatr Surg. 2004 Feb;39(2):e4-7. Abstract quote
BACKGROUND/PURPOSE: Children presenting with persistent symptoms attributed to gastroesophaeal reflux disease (GERD) that are unresponsive to both medical and surgical therapies are commonly submitted to esophageal biopsies, the results of which show an abnormal presence of eosinophils. In this setting, eosinophilic esophagitis may be the correct diagnosis. The purpose of this report is to clarify the importance of esophageal eosinophilic infiltration, regardless of whether associated with acid reflux, ie, as an independent symptomatic entity, when treating a patient with refractory GERD.
METHODS: Two boys, aged 8 and 7 years, had the classic symptoms of GERD. They were treated with antacid without improvement of the esophagic lesions. Subsequent esophageal biopsy results showed marked eosinophilic infiltration. From this moment on, eosinophilic esophagitis started to be considered the main diagnosis.
RESULTS: Although eosinophilic infiltration caused by GERD is very frequently found in esophageal biopsy, in case of refractory drug treatment and microscopic findings of a great number of eosinophils and mast cells, eosinophilic esophagitis must be considered. This disease is better treated with corticoids instead of antacid drugs. It explains the reason some patients do not respond to antacid and surgical treatment and remain symptomatic with esophagic lesions.
CONCLUSIONS: In refractory cases of GERD, eosinophilic esophagitis must be considered before any surgical measure.
INFECTIONS TRAUMA Pill-induced damage
Secondary to chemo- and radiation therapy
PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSIS
- Eosinophilic esophagitis: a 10-year experience in 381 children.
Liacouras CA, Spergel JM, Ruchelli E, Verma R, Mascarenhas M, Semeao E, Flick J, Kelly J, Brown-Whitehorn T, Mamula P, Markowitz JE.
Division of Gastroenterology and Nutrition, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA.
Clin Gastroenterol Hepatol. 2005 Dec;3(12):1198-206. Abstract quote
BACKGROUND & AIMS: Eosinophilic esophagitis (EoE) is a disorder characterized by a severe, isolated eosinophilic infiltration of the esophagus unresponsive to aggressive acid blockade but responsive to the removal of dietary antigens. We present information relating to our 10-year experience in children diagnosed with EoE.
METHODS: We conducted a retrospective study between January 1, 1994, and January 1, 2004, to evaluate all patients diagnosed with EoE. Clinical symptoms, demographic data, endoscopic findings, and the results of various treatment regimens were collected and evaluated.
RESULTS: A total of 381 patients (66% male, age 9.1 +/- 3.1 years) were diagnosed with EoE: 312 presented with symptoms of gastroesophageal reflux; 69 presented with dysphagia. Endoscopically, 68% of patients had a visually abnormal esophagus; 32% had a normal-appearing esophagus despite a severe histologic esophageal eosinophilia. The average number of esophageal eosinophils (per 400 x high power field) proximally and distally were 23.3 +/- 10.5 and 38.7 +/- 13.3, respectively. Corticosteroids significantly improved clinical symptoms and esophageal histology; however, upon their withdrawal, the symptoms and esophageal eosinophilia recurred. Dietary restriction or complete dietary elimination using an amino acid-based formula significantly improved both the clinical symptoms and esophageal histology in 75 and 172 patients, respectively.
CONCLUSIONS: Medications such as corticosteroids are effective; however, upon withdrawal, EoE recurs. The removal of dietary antigens significantly improved clinical symptoms and esophageal histology in 98% of patients.
- Natural history of primary eosinophilic esophagitis: a follow-up of 30 adult patients for up to 11.5 years.
Straumann A, Spichtin HP, Grize L, Bucher KA, Beglinger C, Simon HU.
Department of Gastroenterology, Kantonsspital, Olten, Switzerland.
Gastroenterology. 2003 Dec;125(6):1660-9. Abstract quote
BACKGROUND & AIMS: Primary eosinophilic esophagitis is a chronic, increasingly recognized, interleukin 5-driven inflammatory disorder of the esophagus. The leading symptom in adults is uniform attacks of dysphagia, and the established histologic sign is a dense eosinophilic infiltration of the esophageal epithelium. Before this study, the natural course of eosinophilic esophagitis had not been defined and information regarding potential long-term risks was lacking.
METHODS: This prospective case series included 30 adult patients with eosinophilic esophagitis (22 men and 8 women; mean age, 40.6 years) whose diagnosis had been made >1 year before study debut based on typical history, consistent endoscopic abnormalities, and infiltration of the esophageal epithelium with >24 eosinophils/high-power field. After a mean of 7.2 years, patients underwent a comprehensive follow-up examination.
RESULTS: All patients survived the study period in good health and stable nutritional state. Dysphagia persisted in 29 patients, exerting a major negative effect on socioprofessional activities on 1 patient and a minor impact on 15. Attacks of dysphagia were more frequent in patients with blood eosinophilia or pronounced endoscopic alterations. The esophageal eosinophilic infiltration persisted in all symptomatic patients, but cell numbers spontaneously decreased significantly (78.7 vs. 40.3 cells/high-power field). The inflammatory process evoked fibrosis of the esophageal lamina propria but did not spread to the stomach or duodenum. No case evolved to a hypereosinophilic syndrome.
CONCLUSIONS: Eosinophilic esophagitis, a primary and chronic disease restricted to the esophagus, leads to persistent dysphagia and structural esophageal alterations but does not impact the nutritional state. To date, no malignant potential has been associated with this disease.
STRICTURES
- Eosinophilic esophagitis: strictures, impactions, dysphagia.
Khan S, Orenstein SR, Di Lorenzo C, Kocoshis SA, Putnam PE, Sigurdsson L, Shalaby TM.
Division of Pediatric Gastroenterology, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, Pennsylvania 15213-2583, USA.
Dig Dis Sci. 2003 Jan;48(1):22-9 Abstract quote.
Eosinophilic esophagitis, long known to be a feature of acid reflux, has recently been described in patients with food allergies and macroscopically furrowed esophagus. The pathophysiology and optimal management of patients with eosinophilic esophagitis is unclear.
We describe our clinical experience related to eosinophilic esophagitis and obstructive symptoms in children and propose etiopathogenesis and management guidelines. Twelve children with obstructive esophageal symptoms (11 male), median age 5 years, and identified to have eosinophilic esophagitis with > 5 eosinophils per high-power field (eos/hpf) are reported. Of these, four had strictures, six had impactions, and two had only dysphagia. A diagnostic evaluation included esophagogastroduodenoscopy with biopsies in all and upper gastrointestinal series, IgE, radioallergosorbent tests, and skin tests for food allergies in some cases.
Esophageal histology specimens were independently analyzed for eosinophil density by two authors. Four of five children with > 20 eos/hpf responded to elimination diets/steroids. The fifth child responded to a fundoplication. Seven children had 5-20 eos/hpf and three of them with no known food allergies responded to antireflux therapy alone. Three others in this group with positive food allergies responded to treatment with elimination diets and/or steroids. The seventh patient in this group was lost to follow-up.
In conclusion, on the basis of response to therapy, eosinophilic esophagitis can be subdivided into two groups: those with likely gastroesophageal reflux disease if < 20 eos/hpf and no food allergies, and others with allergic eosinophilic esophagitis associated with food allergies and often with > 20 eos/hpf.TREATMENT Dietary restrictions
Systemic corticosteroidsCORTICOSTEROIDS
Eosinophilic esophagitis in adults: clinical, endoscopic, histologic findings, and response to treatment with fluticasone propionate.
Remedios M, Campbell C, Jones DM, Kerlin P.
Department of Gastroenterology and Hepatology, Princess Alexandra Hospital, Brisbane, Queensland, Australia.
Gastrointest Endosc. 2006 Jan;63(1):3-12. Abstract quote
BACKGROUND: Eosinophilic esophagitis is an increasingly recognized disorder characterized by intense eosinophilic infiltration of the esophageal mucosa. The aim of this study was to define the clinical syndrome, the endoscopic features, and the distribution of the eosinophil infiltrate in adults with eosinophilic esophagitis. We undertook a prospective evaluation of the symptomatic and histologic response to treatment with fluticasone propionate.
METHODS: Twenty-six patients (18 men; mean age 36 years) had symptom assessment and barium studies, esophageal motility recordings, and 24-hour esophageal pH studies. Upper-GI endoscopy was performed with quantitative eosinophil counts of biopsy specimens from the proximal and distal esophagus, the gastric antrum, and the duodenum. Nineteen subjects received 4 weeks of swallowed fluticasone propionate. After treatment, symptom assessment and endoscopic biopsies were repeated.
RESULTS: All 26 patients had a history of dysphagia, and 11 presented acutely with food-bolus obstruction. Esophageal peristalsis was normal in most and gastroesophageal reflux coexisted in 10 patients. Characteristic endoscopic findings of furrows (20) and rings (18) were observed. All 19 treated patients had symptom improvement and a significant decrease in esophageal eosinophil counts.- CONCLUSIONS: Eosinophilic esophagitis is a distinct entity that may coexist with gastroesophageal reflux. Swallowed fluticasone propionate is an effective treatment.
Primary eosinophilic esophagitis in children: successful treatment with oral corticosteroids.
Liacouras CA, Wenner WJ, Brown K, Ruchelli E.
Division of Gastroenterology and Nutrition, The University of Pennsylvania School of Medicine, The Children's Hospital of Philadelphia 19104, USA.
J Pediatr Gastroenterol Nutr. 1998 Apr;26(4):380-5. Abstract quote
BACKGROUND: The histologic appearance of esophageal eosinophils has been correlated with esophagitis and gastroesophageal reflux disease in children. Esophageal eosinophilia that persists despite traditional antireflux therapy may not represent treatment failure, but instead may portray early eosinophilic gastroenteritis or allergic esophagitis. In this study, a series of pediatric patients with severe esophageal eosinophilia who were unresponsive to aggressive antireflux therapy were examined and their clinical and histologic response to oral corticosteroid therapy assessed.
METHODS: Of 1809 patients evaluated prospectively over 2.5 years for symptoms of gastroesophageal reflux, 20 had persistent symptoms and esophageal eosinophilia, despite aggressive therapy with omeprazole and cisapride. These patients were treated with 1.5 mg/kg oral methylprednisolone per day, divided into twice-daily doses for 4 weeks. All patients underwent clinical, laboratory, and histologic evaluation before and after treatment.
RESULTS: Histologic findings in examination of specimens obtained in pretreatment esophageal biopsies in children with primary eosinophilic esophagitis indicated significantly greater eosinophilia (34.2+/-9.6 eosinophils/high-power field [HPF]) compared with that in children with gastroesophageal reflux disease who responded to medical therapy (2.26+/-1.16 eosinophils/HPF; p < 0.001). After corticosteroid therapy, all but one patient with primary eosinophilic esophagitis had dramatic clinical improvement, supported by histologic examination (1.5 +/-0.9 eosinophils/HPF, p < 0.0001).
CONCLUSIONS: Pediatric patients in a series with marked esophageal eosinophilia and chronic symptoms of gastroesophageal reflux disease unresponsive to aggressive medical antire-flux therapy had both clinical and histologic improvement after oral corticosteroid therapy.Macpherson and Pincus. Clinical Diagnosis and Management by Laboratory Methods. Twentyfirst Edition. WB Saunders. 2006.
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Dig Dis Sci 1993;38:109-116
J Pediatr Gastroenterol Nutr 1998;27:90-93
J Pediatr Gastroenterol Nutr 2000;30:S28-S35
Am J Gastroenterol 2000;95:1422-1430
Am J Gastroenterol 2000;95:1572-1575
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