Eosinophilic gastroenteritis (EG) is an uncommon gastrointestinal tract disease, and diagnosis can be difficult. A combination of acute or recurrent abdominal pain with peripheral hypereosinophilia suggests the diagnosis. Surgery in patients with these features might therefore be avoided. Physicians must maintain a high index of suspicion and a working knowledge of the natural history of EG in order to establish the proper diagnosis. We present the case of a young man with EG who presented with relapsing severe abdominal pain and enteropathy with protein loss.
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Eosinophilic gastroenteritis (EG) is an uncommon gastrointestinal tract disease. The need for histological confirmation makes diagnosis problematic. The symptoms and signs of the condition can simulate an acute abdomen. Reports describe the disease mimicking acute appendicitis,1 an obstructing caecal mass,2 pancreatitis,3 cholecystitis,4 giant refractory duodenal ulcer,5 and intussusception.6 A combination of acute or recurrent abdominal pain with peripheral hypereosinophilia suggests the diagnosis. Surgery in patients with these features might therefore be avoided. Physicians must maintain a high index of suspicion and a working knowledge of the natural history of EG in order to establish the proper diagnosis. We present the case of a young man with EG who presented with relapsing severe abdominal pain and enteropathy with protein loss.
A 22 year old man presented to our emergency department with severe abdominal pain. Seven years before, he began developing abdominal pain, bloating, vomiting, and watery diarrhoea that lasted 3–4 months each winter. His symptoms remitted without treatment so he did not seek medical care. More recently, the symptoms had worsened and he had lost 13 kg over the previous 3 years. He had no personal or family history of allergic disorders, asthma, hay fever, drug hypersensitivity, or eczema. Past medical and surgical histories and personal habits were non-contributory.
Upon admission, vital signs were normal. A physical examination was unremarkable except for a hyperactive bowel sound and diffuse abdominal tenderness. Laboratory valuses were: haemoglobin, 124 g/l (normal range 135–180 g/l); mean corpuscular volume 63.5×10−15 l (normal range 79–100×10−15 l); leukocyte count, 7.94×109/l (normal range 4.5–11×109/l); platelet count, 364×109/l (normal range 150–400×109/l), with a differential count of 52% neutrophils (normal range, 40–74%), 25% lymphocytes (normal range 19–48%) and 13% eosinophils (normal range 0–7%). Stool samples were examined twice and no ova or parasites were found. No faecal leucocytes were detected and stool culture was negative. Serum electrolytes, blood urea nitrogen, creatinine, and liver function tests were within normal limits. The serum albumin level (18 g/l) was low (normal range 39–51 g/l). Proteinuria was absent.
The patient’s abdominal pain and recent weight loss prompted his admission for serial abdominal examinations and urgent investigation. An abdominal sonogram was normal. Barium visualisation of the upper gastrointestinal tract showed diffuse mucosal fold thickening involving the duodenum, jejunum, and ileum. An upper gastrointestinal endoscopy with gastric mucosal biopsy was performed. Histological sections of the gastric tissue demonstrated foci of active gastritis with eosinophilic infiltration (fig 1). We checked his gut α1-antitrypsin clearance (24 hour volume of faeces × stool concentration of α1-antitrypsin ÷ serum α1-antitrypsin concentration). The clearance value of 15 mL in 24 h was above the normal range (<13 ml/24 h).
A diagnosis of enteropathy with protein loss was made on the basis of the increased α1-antitrypsin clearance. Mucosal EG with protein losing enteropathy was confirmed. The patient was treated with prednisone and placed on an elimination diet, and his abdominal pain and diarrhoea improved over the next 2 weeks.
EG is a rare inflammatory disease characterised by abdominal pain with bloating, nausea and diarrhoea, eosinophilic infiltration of the gastrointestinal tract, and the absence of extraintestinal involvement and parasitic disease.7 Peripheral blood hypereosinophilia is evident in 20–90% of patients with the disease.8,9 The aetiology of EG is unknown, but speculation has focused on the selective release of eosinophil major proteins as a prelude to intestinal epithelial damage. The disease can affect any age group.10
Clinical features of EG correlate with the depth of eosinophilic infiltration in the bowel wall and the location of lesions within the gut.11 EG is therefore classified into mucosal, muscular, and serosal subtypes.11 The mucosal form, which is most common,11 presents like most other inflammatory gastrointestinal disorders, with abdominal pain, diarrhoea, anorexia, bloating, nausea, and vomiting. Patients may have protein losing enteropathy. There is a frequent association of the disease with seasonal allergy, food sensitivity, eczema, allergic rhinitis, atopy, and bronchial asthma.12 The muscular subtype typically presents with pyloric or intestinal obstruction.13 The eosinophilic infiltrates often involve the muscularis layer of the stomach but can also involve the small intestine.10 Serosal EG is the least common subtype (10% of cases). It produces eosinophilic peritonitis and ascites.14
Peripheral eosinophilia is found in a variable number of EG patients. The magnitude of the eosinophilia seems to correlate with the severity of symptoms. Iron deficiency anaemia may be evident. Elevated IgE, allergy evaluation by skin and serum radioallergosorbent tests, hypoalbuminaemia, increased faecal α-1 anti-trypsin levels, elevated erythrocyte sedimentation rate and the presence of eosinophils in ascites fluid provide further diagnostic support.11 Mild to moderate steatorrhoea is present in approximately 30% of patients. A sterile exudative peritoneal effusion with up to 95% eosinophils is characteristic of serosal eosinophilic gastroenteritis.15
Radiological tests are typically not helpful in diagnosing EG. Barium studies may show a variable degree of antral stenosis with mucosal irregularity, gastric pseudopolyposis, or thickened mucosal folds due to oedema.7 The most common computed tomography finding is nodular and irregular fold thickening in the distal stomach and proximal small bowel. Ascites fluid is usually detected in patients with serosal layer involvement. The gastric antrum and contiguous small bowel are affected in about 70% of patients.8 Uncommonly, only the small bowel is affected and, rarely, only the colon is affected. Mucosal biopsies are not diagnostic in 10% of cases,16 owing to inappropriate sampling or a lack of mucosal involvement.
EG is characterised by a patchy distribution of lesions, necessitating the need for at least six biopsy specimens. Surgical biopsy is needed in patients with muscular or serosal EG subtypes.11 Eosinophils are a normal component of the gastrointestinal mucosa, therefore greater than 19 eosinophils per high power field are necessary for diagnosis.7 Large numbers of eosinophils are often present in the muscularis and serosal layers. The localised eosinophilic infiltrates may cause crypt hyperplasia, epithelial cell necrosis and villous atrophy.7
Once the diagnosis of EG is confirmed, it is useful to test for food allergy, as an elimination diet may provide symptomatic relief.10 Corticosteroids are the mainstay of therapy. Symptom remission usual occurs within a few weeks of treatment initiation.10 The standard treatment regimen is 20–40 mg (paediatric dose 0.14–2 mg/kg) of prednisone daily in divided doses for 7–10 days followed by a taper over 2–3 months.8,14 Patients with refractory or relapsing symptoms are usually maintained on long term, low dose corticosteroids.
Three alternative treatments can be used.8 Sodium cromoglycate, a mast cell stabiliser effective in the treatment of allergies or bronchial asthma, gives variable results, and the recommended dosage for oral administration varies between 100 and 300 mg per dose four times daily.10 Ketotiphene, an antihistaminic agent and mast cell membrane stabiliser, produces clinical improvement, normal eosinophil values and disappearance of the intestinal eosinophilic infiltrate within 1–4 months when administered at 2–4 mg daily.17 Montelukast, a selective and competitive leukotriene receptor antagonist, can be a successful corticosteroid sparing agent.18 The treatment regimen is 10 mg (paediatric dose 5 mg) of montelukast daily.
The diagnosis of EG is challenging because it is a rare disease, is not often considered, presents with non-specific signs and symptoms, is marked by periods of remission and relapse, and the final diagnosis requires histological confirmation.7 However, EG should be considered in the differential diagnosis of patients with unexplained acute or recurrent abdominal pain and peripheral hypereosinophilia.11,19 Stool examinations to exclude parasitic infection are mandatory. Endoscopy with multiple biopsies is required to confirm the diagnosis.
Competing interests: none declared