Primary Eosinophilic Gastrointestinal Diseases Beyond Eosinophilic Esophagitis in Children.

There are many unknowns about primary eosinophilic gastrointestinal disease (EGID) in childhood. The aim of this study is to provide data about the frequency, management, control level, and prognosis of well documented primary EGID in childhood. This study was conducted in children who underwent endoscopy and/or colonoscopy at a single center over 10-year period up to August 2018. Primary EGID was diagnosed after exclusion of secondary EGID and classified as eosinophilic gastritis (EG), eosinophilic enteritis (EE), eosinophilic gastroenteritis (EGE: eosinophilic gastritis with eosinophilic enteritis) and eosinophilic colitis (EC) according to histopathological evaluation. The pathological number of eosinophil counts were accepted as >30 hpf for gastric mucosa in 5 hpf area, ≥20/hpf for duodenal, jejunal, and ileal mucosa, >50/hpf for right colonic mucosa, >35/hpf for transverse colonic mucosa, and >25/hpf for left colonic mucosa. Presenting symptoms, signs, management, follow-up, disease control level, and remission were analyzed. Remission is defined if the patient is controlled with all clinical, endoscopic/colonoscopic, and histopathologic parameters without any treatments or diet for at least a year. During the study period, 7457 biopsies were taken in 8262 endoscopy and/or colonoscopy procedures. Primary and secondary EGID frequencies were found 0.23% (n = 17 patients) and 0.1% (n =8 patients) per procedure with biopsy in children, respectively. Endoscopy/colonoscopy procedures were not able to performed in 9 patients because of short follow-up period (n = 6) or patients leaving follow-up (n = 3). Nine of the primary EGID patients had esophageal eosinophilia (EsE) at the time of diagnosis, 5 of them were previously managed as EoE. The median follow-up period of primary EGID patients excluding the ones without a control endoscopy/colonoscopy procedure was 3.35 years (min-max: 1.1-9.0 years). Proton pump inhibitors (PPI) were the most frequently used treatment alone or in combination with diet, systemic and/or topical corticosteroids. Disease control was evaluated in 8 of 17 patients and it was uncontrolled in 4, partially controlled in 1, and controlled in 3 patients. Remission was achieved in 2 patients. The frequency of primary EGID beyond eosinophilic esophagitis (EoE) in children is low. It may be difficult to achieve control in children with primary EGID in the long-term follow-up.

Egritas Gurkan O ，Ozturk H ，Karagol HIE ，Ceylan K ，Duztas DT ，Ekinci O ，Sari S ，Dalgic B ，Bakirtas A ... -  《-》

Eosinophilic Gastrointestinal Diseases in Childhood.

Eosinophilic gastrointestinal diseases (EGIDs) comprise a group of chronic, inflammatory diseases of the gastrointestinal (GI) tract, that are characterized, clinically, by symptoms related to the dysfunction of the involved segment(s) of the GI tract, and histologically, by dense eosinophilic inflammation, in the absence of an identifiable secondary cause. The group of EGIDs comprises eosinophilic esophagitis (EoE), eosinophilic gastritis (EG), eosinophilic gastroenteritis (EGE), and eosinophilic colitis (EC). EoE is the most common and the best described EGID compared to EG, EGE, and EC. The clinical presentation of the EGIDs differs depending on the location and the extent of the eosinophilic infiltration in the GI tract, as well as its depth through the bowel wall. In the absence of biological markers, the diagnosis is based on the combination of clinical symptoms with the histological features of EGIDs, after the exclusion of secondary causes of eosinophilic inflammation of the GI tract. Treatment is individualized and includes elimination diets (mainly empiric or elemental) and/or drugs, according to the involved GI segment: proton pump inhibitors or local steroids in EoE; local or oral systemic steroids in EG/EGE limited to the duodenum; oral systemic steroids in EGE with lower small intestine and/or colon involvement. In patients with EoE, maintenance treatment with lower doses may be considered following histological remission with the means of drugs. In patients treated with elimination diets, disease food triggers identified during food reintroduction need to be further eliminated. Esophageal stenosis despite medical treatment requires endoscopic dilation, while the use of thiopurines or anti-TNF drugs may be considered in refractory or steroid-dependent EGID (other than EoE). The aim of this review is to provide the available evidence on each of the above disorders, to aid clinicians to interpret the clinical manifestations and the laboratory findings and choose the best available treatment option.

Koutri E ，Papadopoulou A 《-》

Eosinophilic gastrointestinal diseases with esophageal involvement: Analysis of 212 patients.

Primary eosinophilic gastrointestinal diseases (EGID) are chronic inflammatory disorders of the gastrointestinal tract with unknown etiology. Features, utility, and evolution are still unknown in screening for EGID in adult patients with eosinophilic esophagitis (EoE). To evaluate the prevalence, characteristics, comorbidities, and evolution of EGID in adults diagnosed with EoE and investigate differences between both groups. Prospective unicenter observational and analytical study. Gastric and duodenal biopsies were obtained during upper baseline endoscopy in all consecutive EoE adult patients evaluated. A colonoscopy with colon biopsies was performed upon persistent diarrhea and normal duodenal biopsies. 212 EoE patients were included. Nine patients (4.3%) also showed significant eosinophilic infiltration in at least one organ within the digestive tract. The most common site affected was the small bowel (78%). Gastrointestinal symptoms (43% vs. 100%, p<0.002) and, more specifically, either abdominal pain or diarrhea (17% vs. 78%, p<0.001), some food sensitizations, and digestive comorbidities (p<0.05) were significantly more common in patients with EGID. Gastrointestinal symptoms were present in 94/212 (44%) patients, of whom 9 (10%) had EGID. Considering only abdominal pain or diarrhea, 20% suffered from it. EGID rarely coexist with EoE, even when gastrointestinal symptoms are present. These findings advise against routine gastric, duodenal, or colon biopsies in adult EoE patients with gastrointestinal symptoms. Most of the characteristics of EoE do not change due to having EGID except gastrointestinal symptoms, digestive comorbidities, and sensitizations to some foods. The evolution was generally favorable despite intermittent adherence to treatment, especially maintenance.

Gratacós Gómez AR ，Palacios Cañas A ，Meneses Sotomayor J ，Clar Castelló M ，Muñoz Rodriguez JR ，Gomez Torrijos E ... -  《-》

Esophageal Fibrosis in Eosinophilic Gastrointestinal Diseases.

Eosinophilic esophagitis (EoE), the most common eosinophilic gastrointestinal disease (EGID), is associated with lamina propria (LP) fibrosis. The relationship of EoE to other EGIDs is still unclear. We frequently observe cases of concurrent esophageal eosinophilia and extra-esophageal mucosal eosinophilia. The purpose of this study was to compare clinical, endoscopic, and histologic features, as well as the prevalence of esophageal LP fibrosis in children with EGID and concurrent esophageal eosinophilia to children with EoE. We also examine the current practices of pathologists in evaluating fibrosis. We reviewed esophageal biopsies from index cases of EoE (N = 38), EGID with significant esophageal eosinophilia (≥15 eos/hpf) (EGID-SEE, N = 38), EGID with mild esophageal eosinophilia (1-14 eos/hpf) (EGID-MEE, N = 12), and EGID with no esophageal eosinophilia (EGID-NEE, N = 12) for LP presence, adequacy, and fibrosis. EoE and EGID-SEE cases share similar demographics, esophageal endoscopic features, and symptoms. A majority of EGID-SEE cases (71%) had adequate LP for the evaluation of fibrosis, similar to EoE cases (87%). The prevalence of esophageal fibrosis in EoE (79%) and EGID-SEE (55%) cases were similar, whereas no fibrosis was detected in the EGID-MEE and EGID-NEE cases. The fibrosis was patchy and often detected in the distal esophagus. Fourteen cases were reclassified from their original clinical diagnosis as having fibrosis by the study pathologists. Cases of EGID-SEE have overlapping features with EoE, suggesting that all EGIDs are part of a disease continuum. A consensus for the evaluation of LP fibrosis is needed.

Thaker AI ，Melo DM ，Samandi LZ ，Huang R ，Park JY ，Cheng E ... -  《-》

Eosinophilic Gastrointestinal Disorders.

Gonsalves N 《-》