Eosinophilic Gastroenteritis

An Update

Alfredo J Lucendo; Angel Arias

Disclosures

Expert Rev Gastroenterol Hepatol. 2012;6(5):591-601. 

In This Article

Treatment of EGE

Heterogeneity in the clinical presentation, severity, and evolution of EGE, together with its low prevalence, has made it difficult to establish ideal treatment strategies for these patients. As in the case with other EGID, including EoE, no drugs have been approved specifically for the treatment of EGE and comparative studies between different therapeutic modalities are lacking. To make matters more confusing, patient age (children or adults) and the medical specialty area in which they are attended tend to determine which treatments are administered.

Dietary Treatment

In some pediatric patients the disease appears before the age of 1 year and resolves after the elimination of cow's milk from the diet;[79] similar results have also been reported for adult patients.[80] Complete resolution of eosinophilic infiltrate in EGE can also be achieved by exclusive feeding with an amino acid-based elemental diet. However, the elimination of these foods after skin prick tests or radioallergosorbent test has shown variable results.[5,13,81] Thus, a series of pediatric EGE patients showed remission of symptoms in 40% of cases after dietary treatment, which consisted of an elemental diet in children under 6 months and hypoallergenic feeding in older children.[17] Once the remission of EGE is achieved, specific foods should be reintroduced gradually, identifying problem foods by the reappearance of symptoms or through bioptic monitoring. Evidence of further tolerance to offending foods has not been clearly assessed. In the case of adult patients, allergic sensitization test results did not correlate with foods responsible for the disease. Generally speaking, from the literature we can infer that the later EGE appears during childhood, the worse it responds to dietary modification.[13] No agreement exists in the literature as to which allergic evaluations or tests should be carried out on EGE patients.

Drug Therapy

Corticosteroids have by far been the most widely used drugs for treating EGE in both children and adults.[14,16] Corticosteroids also constitute the main treatment for patients in whom dietary therapy is not feasible or after failing to achieve improvement.[5] Prednisone, used at doses of 0.5–1 mg/kg/daily, has proven highly effective in the initial control of symptoms,[14] eosinophilic tissue infiltration, blood hypereosinophilia and also for controlling ascitis, as described in various studies and case reports. Usually, after an initial treatment period of 7–10 days, the dose is gradually reduced until the drug is withdrawn after a period of up to 4 months. Response to steroids in EGE is significantly superior to the mere control of symptoms.[15]

Different series have described steroid-dependent patients in whom symptoms reappeared during steroids tapering.[15] These patients had to either resume taking previous doses, maintain remission by using low doses, substitute prednisone for budesonide,[17] or maintain remission with other antiallergic or immunosuppressant drugs. Approximately 20% of patients require maintenance therapy over time.[17] Budesonide has a better safety profile than prednisone and is especially useful in EGE affecting the distal small bowel and right colon,[82] although it is also helpful in more proximal disease.[57]

Disease recurrence is more likely in those patients requiring treatment at the moment of diagnosis as compared with those who exhibit spontaneous remission; patients with recurrent disease may also present a higher blood eosinophil count at diagnosis than those who show spontaneous remission.[13]

Steroid-dependent or refractory patients can be also managed with thiopurins (azathoprine or 6-mercaptopurin), similar to the treatment of inflammatory bowel disease.[83,84]

Unfortunately, with regard to the utility of other antiallergic drugs in treating EGE, most of the available information comes from isolated cases or small series, which limits our ability to ensure its real usefulness. Some EGE patients have obtained benefit from mast cells stabilizers[13,85,86] such as sodium cromolyn or nedocromil, contrary to what has been observed in EoE, in which they have not demonstrated efficacy.[87] Ketotifen and histamine-1 blockers have shown efficacy in reducing tissue eosinophilia and symptoms in patients with EGE;[88,89] suplatast tosylate was effective in the only case in which it was used.[90] Information regarding the leukotriene inhibitor montelukast is contradictory, since it showed no efficacy in some cases[91–93] while it successfully acted as a steroid-sparing agent in isolated steroid-dependent patients.[16,94]

Finally, biological therapies with the anti-IL-5 monoclonal antibodies mepolizumab and reslizumab to treat hypereosinophilic syndrome have provided only limited data to date. A pilot study in which four EGE patients were treated with a single dose of mepolizumab showed an average drop in blood eosinophilia of 75 and 50–70% in tissue eosinophilia, but with minimal symptoms improvement.[95] In addition, one patient experienced a noticeable increase in gastrointestinal tissue eosinophil count 4 weeks after treatment, while two additional patients underwent an increase in their peripheral eosinophilia, with a worsening of baseline gastrointestinal symptoms after 7–8 weeks of treatment.[96] Intravenous immunoglobulin was successfully used in a patient with erythematous lupus associated with steroid-refractory EGE.[97]

Surgical Treatment

While the muscular form of EGE may cause obstructive symptoms[98] due to bowel wall thickening and narrowing of the lumen, some cases of EGE have been diagnosed after intestinal resection of the affected area after acute abdomen,[47] intestinal obstruction or perforation.[64,65,99] Note that these complications occur more often in the duodenum or the distal ileum. Unfortunately, data on the long-term outcome and possible recurrence of cases after resection of the affected segment is lacking.

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