How should I evaluate and manage a patient with suspected eosinophilic jejunitis presenting with abdominal pain, nausea, vomiting, weight loss, peripheral eosinophilia, and a history of atopic disease?

Eosinophilic Jejunitis: Evaluation and Management

Initial Diagnostic Approach

Eosinophilic jejunitis requires endoscopic evaluation with multiple biopsies from affected segments to confirm dense eosinophilic infiltration (>30 eosinophils per high-power field in at least five fields), while systematically excluding parasitic infections and other causes of gastrointestinal eosinophilia. 1

Critical History Elements

• Travel and exposure history: Detailed questioning about travel to endemic areas for parasitic infections (schistosomiasis, strongyloidiasis, ascariasis) is essential, as these can mimic eosinophilic gastroenteritis 2
• Atopic background: Most patients have concurrent atopic conditions including food allergies, asthma, eczema, or environmental allergies 3, 4
• Symptom pattern: Abdominal pain (present in 100% of cases), weight loss, nausea/vomiting (36%), diarrhea (59%), and bloating (27%) are characteristic 5
• Age and demographics: Typically presents between third and fifth decades, with female predominance (63%) 6, 5

Physical Examination Priorities

• Peripheral eosinophilia: Check complete blood count with differential for elevated eosinophils 1, 4
• Signs of atopic disease: Document presence of eczema, allergic rhinitis, or other atopic manifestations 3
• Nutritional status: Assess for malnutrition and weight loss 1

Diagnostic Workup Algorithm

Step 1: Exclude Parasitic Infections First

Before diagnosing primary eosinophilic jejunitis, parasitic infections must be ruled out through stool examinations and serology:

• Stool studies: Concentrated stool microscopy (minimum 3 samples) and stool PCR for parasites 2
• Serology: Testing for schistosomiasis, strongyloidiasis, and other helminths in specialized laboratories 2
• Specific parasites to consider:
  • Schistosomiasis (incubation 2-8 weeks, treat with praziquantel 40-60 mg/kg) 2
  • Strongyloidiasis (treat with ivermectin 200 μg/kg for 1-2 days) 2
  • Ascariasis (treat with albendazole 400 mg single dose) 2
  • Hookworm (treat with albendazole 400 mg single dose) 3

Step 2: Endoscopic Evaluation with Systematic Biopsies

• Upper endoscopy: Obtain multiple biopsies from stomach, duodenum, and proximal jejunum 5
• Enteroscopy or capsule endoscopy: May be needed to visualize jejunal involvement 1
• Histologic criteria: Dense eosinophilic infiltration >30 eosinophils per high-power field in at least five fields, with evidence of eosinophil degranulation extending to muscularis mucosa or submucosa 1
• Pattern documentation: Note whether infiltration is mucosal, muscular, or serosal, as this determines clinical presentation 1

Step 3: Additional Laboratory Assessment

• IgE levels: Often elevated in atopic patients 4
• Immunoglobulin panel: Check IgG, IgA, IgM to exclude immunodeficiency states that increase susceptibility to infections 7
• Celiac serology: IgA-tissue transglutaminase to exclude celiac disease 7

Treatment Strategy

First-Line: Corticosteroids

Systemic corticosteroids are the most effective first-line therapy for eosinophilic jejunitis, with prednisone 20-40 mg/day for 2-4 weeks inducing remission in the majority of patients. 2, 5, 4

• Efficacy: Corticosteroids were effective in 95% (21/22) of patients in inducing remission 5
• Duration: Typically 3 weeks of oral prednisone with complete symptom resolution 4
• Monitoring: Repeat endoscopy with biopsies to confirm histologic improvement 3

Alternative Dietary Approaches

• Elimination diets: Should be considered, particularly in patients with documented food allergies 1
• Amino acid-based formulas: May be effective in select cases with strong allergic component 3

Refractory Disease Management

For steroid-refractory cases, vedolizumab (integrin blocker) shows promise, inducing clinical and histological improvement in 75% (3/4) of refractory patients. 5

• Anti-TNF therapy: Limited efficacy, used in only 14% of patients with variable response 5
• Proton pump inhibitors: May have anti-inflammatory effects, used in 32% of cases 5

Critical Pitfalls and Complications

Steroid-Related Concerns

While corticosteroids are highly effective, they may mask serious complications including perforation, requiring careful monitoring during treatment. 6

• Perforation risk: Eosinophils can directly damage the gastrointestinal wall, and steroids may mask symptoms of perforation 6
• Monitoring strategy: Maintain high clinical suspicion for acute abdomen despite steroid therapy 6

Chronic Disease Complications

• Diverticulosis: Chronic intermittent obstruction from eosinophilic jejunitis can lead to small bowel diverticulosis from repeated increased intraluminal pressure 8
• Volvulus: Severe cases may develop mechanical complications requiring surgical intervention 8
• Stricture formation: Progressive fibrosis can occur with chronic untreated disease 9

Surgical Indications

Surgery should be reserved for complications (obstruction, perforation) rather than primary treatment, as medical management is usually effective. 6, 1

• Avoid unnecessary resection: Current knowledge supports medical management unless complications are inevitable 6
• Specific indications: Mechanical obstruction unresponsive to medical therapy or perforation 1

Natural History and Follow-Up

Eosinophilic gastroenteritis is a chronic disease with three evolutionary patterns: single outbreak, recurrent course, or chronic disease requiring long-term monitoring. 9, 1

• Chronicity: Few patients outgrow the illness, necessitating long-term follow-up 9
• Surveillance: Regular clinic visits to monitor symptoms, treatment compliance, and adverse effects 9
• Prognosis: Fatal outcomes are rare and disease does not limit life expectancy, though complications can occur 9
• Repeat endoscopy: Indicated if symptoms recur during treatment to assess for refractory disease, fibrostenotic complications, or treatment-related issues (e.g., candidiasis) 3

Multidisciplinary Management

Patients with refractory disease or significant concomitant atopic conditions should be co-managed by gastroenterology and allergy specialists to optimize treatment. 3

• Allergy consultation: Particularly important for patients with multiple food allergies requiring elimination diets 3
• Dietitian involvement: Essential for implementing and monitoring dietary restrictions while ensuring nutritional adequacy 3