eMedicine Specialties > Gastroenterology > Intestine

Eosinophilic Gastroenteritis

Author: MyNgoc T Nguyen, MD, Clinical Assistant Professor, Department of Internal Medicine, University of California at San Francisco
Coauthor(s): Jean-Luc Szpakowski, MD, Chief of Gastroenterology, Kaiser Permanente Medical Center; Clinical Faculty, University of California at San Francisco
Contributor Information and Disclosures

Updated: Jun 15, 2009

Introduction

Background

Eosinophilic gastroenteritis (EGE) is an uncommon gastrointestinal disease affecting both children and adults. Eosinophilic gastroenteritis is characterized by the following:

• The presence of abnormal GI symptoms, most often abdominal pain
• Eosinophilic infiltration in one or more areas of the GI tract, defined as 20 or more eosinophils per high-power field
• The absence of an identified cause of eosinophilia
• The exclusion of eosinophilic involvement in organs other than the GI tract

A history of atopy or food allergies is often present.

Clinical symptoms are determined by the anatomical locations of eosinophilic infiltrates and the depth of GI involvement. Kaijser was probably the first to report a patient with eosinophilic gastroenteritis in 1937; since then, the number of case reports has increased. Treatments are often unsatisfactory, and long-term outcomes are uncertain.

Pathophysiology

The underlying molecular mechanism predisposing to the clinical manifestation of eosinophilic gastroenteritis is unknown. Eosinophilic gastritis, enteritis, and gastroenteritis are diseases characterized by the selective infiltration of eosinophils in the stomach, small intestine, or both. The disorders are classified into primary and secondary subtypes. The primary subtypes, which have also been called idiopathic or allergic GE, include the atopic, nonatopic, and familial subtypes.

In patients, manifestations of their diseases are based on histologic involvement: mucosal, muscularis, or serosal forms. Any layer of the GI tract can be involved. The secondary subtypes may be divided into 2 groups: systemic eosinophilic disorders (ie, hypereosinophilic disorders) and noneosinophilic disorders (eg, celiac disease, inflammatory bowel disease, vasculitis).

Although these diseases are idiopathic, recent investigations support the role of eosinophils, T helper 2 (Th2) cytokines (interleukin [IL]-3, IL-4, IL-5, and IL-13), and eotaxin as the critical factors in the pathogenesis of eosinophilic gastroenteritis. Eosinophils function as antigen presenting cells as they express major histocompatibility complex (MHC) class II molecules.

In addition, eosinophils can mediate proinflammatory effects, including the up-regulation of adhesion systems, modulation of cell trafficking, and cellular activation states by releasing cytokines (IL-2, IL-4, IL-5, IL-10, IL-12, IL-13, IL-16, IL-18, and transforming growth factor [TGF]-alpha/beta), chemokines (RANTES and eotaxin), and lipid mediators (platelet activating factor [PAF] and leukotriene C4).

Finally, eosinophils can serve as major effector cells, inducing tissue damage and dysfunction by releasing toxic granule proteins (major basic protein [MBP], eosinophilic cationic protein [ECP], eosinophil peroxidase [EPO], and eosinophil-derived neurotoxin [EDN]) and lipid mediators, which are cytotoxic.

Atopy is present in a subset of patients, as these patients demonstrate increased total immunoglobulin E (IgE) on food-specific IgE radioallergosorbent assay test (RAST) or skin tests. Furthermore, lamina propria T cells from the duodenum of these patients proliferated in response to milk proteins and secreted Th2 cytokines (IL-13). However, other studies suggest a non–IgE-mediated mechanism.

Frequency

United States

The disease is rare, and the incidence is difficult to estimate. However, since the description of eosinophilic gastroenteritis by Kaijser in 1937, more than 280 cases have been reported in the medical literature.

International

Although cases have been reported worldwide, the exact incidence of eosinophilic gastroenteritis is unclear. A confounding factor is lack of diagnostic precision.

Kim et al reported 31 new cases of eosinophilic gastroenteritis in Seoul, Korea, between January 1970 and July 2003.¹

Chen et al reported on 15 patients, including 2 children, with eosinophilic gastroenteritis who were evaluated over an 18-year period at a hospital in China in 2003.²

Venkataraman et al reported 7 new diagnoses of eosinophilic gastroenteritis over a 10-year period in India.³

Mortality/Morbidity

• Death from eosinophilic gastroenteritis has been reported only rarely.
• Morbidity includes malnutrition and intestinal obstruction and perforation.

Race

Cases of eosinophilic gastroenteritis are reported mostly in whites, with some cases occurring in Asians.

Sex

A slight male preponderance has been reported.

Age

Patients present clinically in the third to fifth decades of life, but the disease can affect any age group, from infancy through the seventh decade.

Clinical

History

Patients may have various clinical presentations depending on the region of the GI tract involved and the depth of the bowel wall involvement. The disease most often involves the stomach and the small bowel. A history of atopy and allergies is present in many of the cases.

• The mucosal form of eosinophilic gastroenteritis is characterized by vomiting, dyspepsia, abdominal pain, diarrhea, blood loss in the stools, iron deficiency anemia, malabsorption, protein-losing enteropathy, and failure to thrive.
• The muscularis form, characterized by infiltration of eosinophils predominantly in the muscularis layer, may present with gastrointestinal obstructive symptoms mimicking pyloric stenosis or gastric outlet syndrome.
• The serosal form, which is less common, presents with significant bloating, exudative ascites, and higher peripheral eosinophil counts.

Physical

The heterogeneity in the clinical presentation of eosinophilic gastroenteritis is determined by the site and depth of eosinophilic intestinal infiltration.

• Approximately 50% of patients have a history of atopy (eg, hay fever, asthma, food allergy). In children, a history of allergy is even more common.
• Children and adolescents can present with growth retardation, failure to thrive, delayed puberty, or amenorrhea. Adults have abdominal pain, diarrhea, or dysphagia.
• Patients with muscle layer involvement typically present with pyloric or intestinal obstruction.
  • The eosinophilic involvement often is localized to the stomach but can involve the small bowel.
  • Cramping and abdominal pain associated with nausea and vomiting occur frequently.
  • Food allergy and past history of allergy are less common in these patients than in patients with mucosal layer disease.
• Involvement of the serosal layer is the least common form of the disease.
  • The entire GI wall usually is involved.
  • These patients typically present with eosinophilic ascites.
  • Serosal and visceral peritoneal inflammation leads to leakage of fluids.

Causes

The cause or mechanism of eosinophilic infiltration is not known.

• Patients with eosinophilic gastroenteritis have elevated IgE and eosinophilia of tissue and blood. An imbalance in the T-cell paradigm causing an increase in the production of IL-13, IL-4, and IL-5 and cytokines has been postulated as the cause of IgE synthesis and eosinophilia.
• In one study, immunohistochemistry detected IL-3, granulocyte-macrophage colony-stimulating factor (GM-CSF), and IL-5 in the granule matrix of eosinophils, the release of which is thought to be involved in the perpetuation of intestinal eosinophil infiltration and inflammation.

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