Eosinophilic gastroenteritis with severe gastrointestinal intolerance: A case report and literature review

Abstract

Eosinophilic gastroenteritis is a rare gastrointestinal disease characterized by eosinophilic infiltration of the gastrointestinal mucosa, muscle layer, or serosal layer. Gastrointestinal symptoms, laboratory tests, imaging, and endoscopy can provide important diagnostic basis for eosinophilic gastroenteritis, but the final diagnosis needs to be confirmed via pathological biopsy. We herein report the case of a middle-aged woman with abdominal pain for 5 days, which was aggravated for 1 day. Gastroscopic pathology showed individual nondiffuse eosinophils (concentration of <10 eosinophils/high power field) in the fundus and antrum. Eosinophils with a maximum concentration of 80/high power field were uniformly distributed in the stroma and specific epithelium of the duodenal bulb. Eosinophils were infiltrated in the submucosa as well as the perivascular and vascular walls of the descending duodenum, with a maximum concentration of approximately 130/high power field. After 5 days of intravenous meprednisolone treatment, the symptoms improved and the patient was discharged healthy. After reviewing the literature, we recommend that if a patient exhibits gastrointestinal symptoms and elevated blood eosinophil levels with co-existing atopic diseases, such as asthma and allergic rhinitis, the clinician should perform gastroenteroscopy and pathological biopsy after ruling out tumor, tuberculosis, and infection.

Introduction

Eosinophilic gastroenteritis (EGE) is a rare gastrointestinal disease characterized by eosinophilic infiltration in the digestive tract, which was first proposed by Kaijser et al. in 1937. ¹ There is no specific manifestation of this disease. Clinical symptoms of EGE include abdominal pain, nausea, vomiting, intestinal obstruction, and ascites. The site involved and the depth of infiltration determine the difference in EGE clinical presentation. ² Herein, we reported a case of EGE characterized by severe gastrointestinal intolerance and reviewed the literature.

Case report

A woman in her 40s was admitted to our hospital on 26 July 2020 due to abdominal pain for 5 days, which was aggravated for 1 day. Five days before admission, she developed persistent abdominal pain, mainly around the umbilical cord, accompanied with nausea, which worsened after eating. The symptoms were improved after emesis. Oral omeprazole therapy did not provide significant relief (dosage unknown). On the day of admission, the abdominal pain worsened, mainly in the upper abdomen and periumbilicus. She had a history of gestational diabetes, bronchial asthma, and allergy to streptomycin and erythromycin. After admission to our hospital, her laboratory examination showed an eosinophilic percentage of 6.5% (reference range: 1.0%–6.0%) and eosinophilic absolute value of 0.67 × 10⁹/L (reference range: 0.02–0.45 × 10⁹/L). Immunoglobulin E 323.1 (reference range: 0–100 IU/mL), allergen screening dust mite, egg white mild allergy, liver fluke IgG antibody positive, and other tests were normal. Gastroscopy showed a broad basal polyp with a size of 0.3 × 0.4 cm in the gastric bottom and a smooth surface (Figure 1(a)). Moreover, it revealed slight hyperemia and edema in the gastric mucosa (Figure 1(b)) and less smooth and red and white mucosa in the gastric antrum, with multiple erythema and scattered punctate erosions (Figure 1(c) and (d)). It also showed duodenal bulbar mucosal congestion, edema, scattered punctate erosions (Figure 1(e)), and duodenal descending mucosal congestion (Figure 1(f)). Multipoint biopsy was performed. Gastroscopic pathological examination revealed individual nondiffuse eosinophils (concentration of <10 eosinophils/high power field (HPF)) in the stroma of the fundus and antrum (not shown). Moreover, it revealed uniformly distributed eosinophils in the stroma and specific epithelium of the duodenal bulb, with a concentration of 80/HPF in dense areas (not shown). Eosinophilic infiltrates were observed in the submucosa as well as the perivascular and vascular walls of the descending duodenum, with a concentration of approximately 130/HPF (Figure 2(a) to (c)).

Figure 1.

Gastroscopy findings. (a) A broad basal polyp in the fundus of the stomach, with a size of approximately 0.3 × 0.4 cm and a smooth surface. (b) The gastric mucosa is slightly hyperemic and edema. (c, d) Gastric antrum mucosa appears red and white and is less smooth, with multiple erythema and scattered punctate erosions. (e) Duodenal bulb mucosal congestion, edema, and scattered punctate erosions and (f) duodenal descending mucosal congestion.

Figure 2.

Pathological findings. Eosinophilic infiltrates were observed in the submucosa as well as the perivascular and vascular walls of the descending duodenum. Specimens observed at magnifications of (a) 200×, (b) 400× and (c) 400×, with a concentration of approximately 130/high power field.

Based on the history, clinical manifestations, laboratory, imaging, and pathological findings, the patient was diagnosed with mucosal EGE. Subsequently, meprednisolone was given orally (24 mg/time, once daily), and the symptoms of abdominal pain were improved (pain score at admission: 7 points; pain score after 6 days of meprednisolone treatment: 2 points). However, the symptoms were not completely relieved, and the pain was aggravated at night. The symptoms of abdominal pain appeared again, and nausea and vomiting were aggravated. Subsequently, meprednisolone was given intravenously (20 mg/time, once daily), and the symptoms were gradually relieved 5 days later. After discharge, methylprednisolone (20 mg/time, once daily) was maintained orally for approximately 1 week. The dose of methylprednisolone was reduced by 4 mg/week for 40 days and then reduced to 2 mg for 60 days. Reexamination with gastroscopy and biopsy of the gastric antrum, stomach body, duodenal bulb, and descending duodenum showed no eosinophilia (not shown). After regular follow-up for 6 months, the patient had no abdominal pain or other discomfort, and her condition was stable until April 2024. The reporting of this study conforms to the Case Report (CARE) guidelines. ³

Discussion

EGE is characterized by eosinophilic infiltration of the stomach wall or intestinal wall and peripheral blood eosinophilia. The peak age of EGE occurrence is 3–50 years, and a slight male preponderance has been reported. ⁴ According to the extent of eosinophilic infiltration of the gastrointestinal tube wall in pathological tissues, EGE can be divided into mucosal lesions, muscular lesions, and serosal lesions, which can exist separately or in combination. ⁵ The etiology and pathogenesis of this disease have not yet been clarified. Studies have shown that it is related to allergic reactions caused by internal and external allergens, such as bronchial asthma and allergic bronchitis. ⁶ In terms of pathophysiology, a number of studies have shown that excessive production of interleukin-13 and interleukin-5 can recruit eosinophils into the intestinal epithelium. Eosinophils exert their cytotoxic effects by producing major basic proteins, eosinophil-derived neurotoxins, eosinophilic cationic proteins, and eosinophilic peroxidase.^7,8

The clinical manifestations of EGE are diverse and nonspecific, which include common epigastric pain, nausea, vomiting, diarrhea, and weight loss. Muscular lesions may present with intestinal obstruction and perforation. Subserous lesions are rare and may include ascites and abdominal distension. ⁹ We reviewed all EGE cases reported in PubMed (https://pubmed.ncbi.nlm.nih.gov/) within the past 1 year. These cases were mainly reported in the stomach and intestines, and the symptoms, endoscopy, pathological findings, and treatment options were reviewed (Table 1). Based on the available evidence, EGE diagnosis can be made according to the following criteria: ¹⁰ (a) digestive tract symptom; (b) histological evidence of eosinophilic infiltration in the gastrointestinal tract (stomach: ≥30 eosinophils in each five HPFs, duodenum: ≥30 eosinophils); ¹¹ and (c) other diseases causing gastrointestinal eosinophilia have been ruled out. In general, EGE is characterized by a lack of clinical, laboratory, and imaging findings and is diagnosed mainly via biopsy and exclusion of other diseases. Clinically, it also needs to be distinguished from the following other diseases: (a) infectious diseases caused by parasites, amoeba, Mycobacterium tuberculosis, and fungi; (b) connective tissue diseases such as polyarteritis nodosa; (c) hypereosinophilia syndrome; (d) inflammatory diseases of the digestive tract such as inflammatory bowel disease; and (e) tumors such as lymphomas and hematological tumors. ¹²

Table 1.

EGE cases reported in PubMed website within the past 1 year.

Author(s)	Age (years)/Sex	Symptoms	Endoscopic and pathohistological features	Treatment
Fu et al. 17	59/F	Bloating, acid reflux, abdominal pain, and nausea	Peak eosinophil counts of 48 and 70/HPF in the stomach and duodenum, respectively	Upadacitinib (15 mg daily)
Zhang et al. 18	73/F	Abdominal pain and fatigue	Rough duodenum mucosa, polyp of the stomach body.Peak eosinophil counts of 50 and 140/HPF in the gastric body polyp and duodenal mucosa, respectively	Methylprednisolone (32 mg daily for 1 week)
Horino et al. 19	81/M	High fever, wheezing, eruption, and stomach discomfort	Erosion and swelling of the mucosa of the stomach and duodenumThe mucosa of the stomach and duodenum showed numerous eosinophil infiltrations in the submucosa	Discontinuation of clopidogrel bisulphate
Fu et al. 20	35/F	Heartburn, abdominal pain, diarrhea, rectal bleed, and spine pain	Eosinophil counts of 68, 53, 120, and 87/HPF in the stomach, duodenum, terminal ileum, and colon, respectively	Budesonide and adalimumab
Ito et al. 21	46/M	Fever and abdominal pain	Mild erosion in the gastric fundus, jejunum had edematous changes in the mucosa with erythema and multiple ulcers, scattered mucosal edema and erosions from the descending colon to the rectumEosinophilic infiltrates of the esophageal, gastric, duodenal, and colonic mucosa	Mepolizumab
Fan et al. 22	57/F	Deformity, pain, morning stiffness of the proximal and distal interphalangeal joints in both hands, nausea, vomiting, and abdominal distension	Eosinophilic infiltration in the gastric antrum and duodenum	Tofacitinib (5 mg twice daily)
Nizam et al. 23	39/F	Abdominal pain, abdominal distension, dysuria, perianal itching, body ache, and bilateral foot pain	Mild erosive gastritis with ulcerations in the small bowelEosinophil counts of >20, 20, 30, and 30/HPF in the esophagus, stomach, duodenum, and colon, respectively	Budesonide

M: male; F: female; HPF: high power field; EGE: eosinophilic gastroenteritis

EGE treatment is primarily empirical. There is no definitive consensus on the optimal treatment for EGE. Several therapeutic options have been suggested and proven to be efficient, such as dietary intervention, corticosteroids, mast cell stabilizers (cromolyn sodium), leukotriene receptor antagonists (montelukast), and immunomodulators. ¹³ Dietary intervention can eliminate the influence of suspected allergic foods on the condition, but there is no clear conclusion that this method is effective in the long term. ¹⁴ The first drug treatment is adequate hormone therapy, mainly comprising prednisone (0.5–1.0 mg/kg/d), which can relieve symptoms within 2 weeks, and the dose is gradually reduced after 6–8 weeks. ¹⁵ If hormones are ineffective, immunosuppressants and biological agents can be used, and even surgical resection can be performed. ¹⁶ After admission, the patient underwent gastroscopy and other examinations and was given methylprednisolone oral treatment after excluding drug and food allergies, intestinal tuberculosis, intestinal parasites, Crohn’s disease, tumors, and other diseases that could cause gastrointestinal symptoms. At the initial stage of treatment, after the patient’s symptoms of abdominal pain were relieved, the symptoms appeared again within 1 week and worsened at night; nausea and vomiting were also noted. Considering the patient’s clinical manifestations, auxiliary examination, and pathological results, the EGE diagnosis was confirmed. However, during the course of oral hormone therapy, recurrent abdominal pain, accompanied with nausea and vomiting, may affect the effect of oral hormone intolerance, nausea, and vomiting. Therefore, adjusting the administration mode to intravenous hormone therapy has remarkable effects, which also confirms the diagnosis of EGE. The typical symptoms of the patient were abdominal pain at night, nausea and vomiting, and oral hormone intolerance. Combined with the previous history of “bronchial asthma,” the diagnosis was made after systematic differential investigation, and a good treatment effect was achieved.

Most EGE cases have atypical early symptoms, resulting in a high rate of missed diagnosis and misdiagnosis, and it may take months or years to diagnose such cases. Therefore, it is necessary to improve the understanding and diagnostic awareness of this disease, pay attention to endoscopic multipoint biopsy and pathology tracking, focus on early identification of EGE, ensure early treatment, and try to avoid missed diagnosis and misdiagnosis, thereby facilitating timely and correct treatment and improving the quality of life of patients. In EGE treatment, oral hormone intolerance is not common, and it is more likely to occur in patients with muscular lesions complicated with intestinal obstruction. The effect of oral drugs is further affected by frequent nausea and vomiting. After timely adjustment of the intravenous hormone therapy to improve the symptoms of patients, switching to oral hormone therapy can achieve a better effect.

Conclusion

EGE was diagnosed on the basis of pathological evaluation, laboratory tests, and clinical manifestations. Early diagnosis and treatment of EGE will benefit organ protection and clinical symptom remission. If a patient has gastrointestinal symptoms, co-existing atopic diseases, and elevated blood eosinophil levels, the clinician should perform gastroenteroscopy and pathological biopsy after ruling out tumor, tuberculosis, and infection.