An article published in the journal Gastroenterology & Hepatology highlights recent updates to eosinophilic gastrointestinal disease nomenclature, as well as updates related to diagnosis, pathogenesis, and treatment standards.
Eosinophilic gastrointestinal diseases (EGIDs), which are characterized by eosinophilic infiltration in the gastrointestinal (GI) tract, can be difficult to describe and name due to the involvement of multiple areas and depths within the GI tract. An article published in the journal Gastroenterology & Hepatology highlights recent updates to EGID nomenclature, as well as updates related to diagnosis, pathogenesis, and treatment standards.
The most common type of EGID is eosinophilic esophagitis (EoE), and the recognition and understanding of other EGIDs affecting different parts of the GI tract has increased. Still, EGIDs aside from EoE have been understudied in comparison. The varied terminology used in reference to EGIDs has also led to practical challenges and has made collective understanding of the conditions more difficult.
A newly established framework for EGID nomenclature aims to provide a systematic method for naming EGIDs, hopefully to improve diagnostic accuracy and facilitate more specific data collection. Non-EoE EGIDs were often referred to with the blanket term eosinophilic gastroenteritis, but this term has often been used without specifying the involved areas in the GI tract.
The new nomenclature framework includes a 2-tiered naming system. The first tier describes the specific GI tract location the disease is affecting, and the second tier provides more specific disease involvement characteristics. The term eosinophilic gastroenteritis was also addressed in the guidelines—EGID is now considered the appropriate term for these disorders. An eo- prefix is also used in some instances to denote the involved area. The term EoG is used for eosinophilic gastroenteritis, and eosinophilic enteritis (EoN) is used when the small bowel is involved, for example. Further specification within the small bowel, such aseosinophilic duodenitis (EoD), eosinophilic jejunitis (EoJ), and eosinophilic ileitis (EoI), should be used to specify.
According to the contributing experts, identifying a primary location is key. Describing multiple areas of involvement is still a challenge, considering it is unclear whether these diseases are distinct or on a spectrum, the authors noted. This includes describing overlapping esophageal involvement in non-EoE EGIDs. More detail on the layers of the GI wall involved or on specific complications must also be considered to further classify these conditions.
Efforts to establish diagnostic guidelines for non-EoE EGIDs are still in progress, but diagnosis can be challenging due to the wide array of common and nonspecific GI symptoms patients often experience. A high level of suspicion is needed to diagnose non-EoE EGIDs, and other GI disorders are often considered first. Individuals often face significant delays in obtaining a correct diagnosis.
Non-EoE EGIDs can involve multiple layers of the luminal wall, and various complications can accompany them. When seemingly common symptoms or complications arise with no apparent explanation, EGID should be considered in the differential diagnosis. Endoscopic evaluation often returns normal findings, but efforts to create a grading system specifically for EGIDs are underway. Still, endoscopic examination is necessary for both visual examination and biopsy collection. Normal thresholds for biopsy measures are also not well defined yet. Ruling out secondary peripheral eosinophilia is another crucial step in the diagnostic process.
The pathogenesis of EGIDs is not well understood, especially non-EoE EGIDs, but there is evidence of allergic etiologies triggered by food antigens. Systemic corticosteroids such as prednisone are most commonly used to treat EGIDs, and patients typically respond—but long-term use of corticosteroids is not an ideal solution. Dietary interventions have also been employed with varying success. Further research is needed to form detailed treatment best practices.
“As with other eosinophil-mediated diseases, many patients who have non-EoE EGIDs face barriers across multiple domains,” the authors wrote. “Patients have reported a number of medical, psychosocial, and financial barriers based on their clinical presentation and ensuing diagnostic journey.”
Gaining a better understanding of the natural course of the disease and optimizing the diagnostic approach are both paramount to improving the management of EGIDs in general as well as non-EoE EGIDs. Well-designed studies are also needed to identify the optimal therapy approach for non-EoE EGIDs.
Redd WD, Dellon ES. Eosinophilic gastrointestinal diseases beyond the esophagus: an evolving field and nomenclature. Gastroenterol Hepatol (N Y). 2022;18(9):522-528.