好酸球性消化管疾患

Eosinophils are tissue-adapted granulocytes with important roles in host defense, immune regulation, barrier responses, and inflammatory disease. Beyond classical degranulation, activated eosinophils can release eosinophil extracellular traps (EETs), extracellular DNA and protein structures that arise through cytolytic nuclear EETosis or rapid mitochondrial DNA (mtDNA) release. These traps may contribute to host protection by immobilizing pathogens and concentrating eosinophil granule proteins at sites of mucosal or tissue inflammation. However, when EET formation is excessive, persistent, or insufficiently cleared, the same structures may promote epithelial injury, mucus viscosity, thromboinflammation, tissue remodeling, fibrosis, and chronic eosinophilic inflammation. This review summarizes the cellular mechanisms, structural organization, inducing stimuli, and disease relevance of EETs. Particular attention is given to infectious settings, eosinophilic airway and middle-ear disease, systemic vasculitic and hypereosinophilic disorders, inflammatory skin and ocular diseases, eosinophilic gastrointestinal disease, and cancer. Current evidence indicates that EETs should be interpreted as context-dependent eosinophil effector structures with dual biological potential. Although their mechanistic relevance is increasingly recognized, further histological, functional, and clinical validation is needed before EETs can be established as reliable biomarkers or therapeutic targets.

Pediatric intestinal pseudo-obstruction (PIPO) is a rare, severe gastrointestinal motility disorder characterized by recurrent symptoms of bowel obstruction in the absence of a mechanical cause. Although most pediatric cases are attributed to primary neuromuscular abnormalities, secondary inflammatory disorders should be considered when symptoms are progressive, refractory to conventional therapy, or associated with peripheral eosinophilia and atopic disease. Localized eosinophilic ileitis is an uncommon manifestation of eosinophilic gastrointestinal disease (EGID) and rarely presents as secondary PIPO, making diagnosis particularly challenging. We describe a four-year-old Hispanic boy with a history of egg allergy, atopic dermatitis, lactose intolerance, chronic constipation, progressive abdominal distension, anemia, and peripheral eosinophilia who developed recurrent intestinal pseudo-obstruction beginning at two years of age. Hirschsprung disease was excluded by both rectal suction biopsy and subsequent full-thickness rectal biopsy, each demonstrating normal ganglion cells. Persistent symptoms despite maximal medical therapy prompted creation of an end ileostomy for bowel decompression, which required conversion to a loop ileostomy after early prolapse. During the following two years, the patient experienced recurrent ileostomy prolapse requiring multiple operative revisions. Previous surgical specimens demonstrated ischemic and inflammatory changes without eosinophilic infiltration. Because of persistent obstructive symptoms and recurrent prolapse, definitive ileostomy reversal was performed. Intraoperatively, the prolapsed ileal segment appeared erythematous, mildly thickened, and friable, while the remaining bowel appeared grossly normal. Histopathologic examination of the resected specimen demonstrated focal patchy intramucosal eosinophilia exceeding 70 eosinophils per high-power field, establishing the diagnosis of localized eosinophilic ileitis after exclusion of secondary infectious causes. No eosinophilic infiltration had been identified in previous rectal or ileostomy specimens. The patient experienced rapid postoperative recovery and remains asymptomatic 12 months after surgery without recurrent pseudo-obstruction or prolapse. This case highlights the diagnostic challenges posed by localized eosinophilic ileitis, demonstrates the limitations of negative biopsies obtained from uninvolved bowel, and emphasizes the importance of considering EGID in children with refractory intestinal dysmotility, peripheral eosinophilia, and atopic disease.

BACKGROUND: Primary immunodeficiencies (PIDs) and eosinophilic gastrointestinal diseases (EGIDs) may share common underlying defects. However, no studies have investigated the frequency of PIDs among patients with EGIDs. In this study, we aimed to assess the frequency and spectrum of PIDs among our pediatric EGIDs patients. METHOD: Patients were prospectively evaluated over a two-year period. All patients were questioned according to ten warning signs of the Jeffrey Model Foundation. Routine laboratory tests and basic immunologic tests [serum immunoglobulin levels, isohemagglutinin titres, anti-HBs and anti-rubella Ig G titres] were performed on all participants. Advanced immunologic workup was performed in selected cases. All PID diagnoses were established according to the European Society for Immunodeficiencies' criteria. Electronic health records were screened for EGIDs' specific features, comorbidities, and previous laboratory tests. RESULTS: A total of 88 EGID patients [76% male, mean age: 12.02 years, 78 patients (88.6%) with EoE] were included. Fourteen patients were diagnosed with predominantly antibody deficiencies [unclassified antibody deficiency (n = 11), selective Ig A deficiency (n = 2), transient hypogammaglobulinemia of infancy (n = 1)]. Eighteen patients had abnormal Ig levels. Comparisons across the groups revealed no statistically significant differences in demographic, endoscopic, and pathologic features. Only topical swallowed budesonide unresponsiveness was significantly higher among cases with PID (p = .004). CONCLUSION: PIDs may not be rare among patients with EGIDs. JMF's warning signs alone may be insufficient to identify affected patients in this population; therefore, additionally, basic immunologic tests as first step and advanced evaluation in suspected cases may be appropriate. Neither the key diagnostic endoscopic and pathological features nor the presence of strictures reliably distinguish PIDs.

Eosinophilic gastrointestinal disorders encompass chronic, immune-mediated disorders indicative of eosinophil infiltration and inflammation causing a range of gastrointestinal symptoms. Eosinophilic gastrointestinal disorders are subdivided into the part of the gastrointestinal tract that is involved, including eosinophilic esophagitis, eosinophilic gastritis, eosinophilic enteritis, and eosinophilic colitis. Treatment for eosinophilic gastrointestinal disorders can vary widely, and treatment modalities include dietary intervention, drugs, and endoscopy-based dilation therapy. With these wide range of therapeutic options, shared decision-making with the patient is important for finding the right treatment for each individual patient. In this review, treatment modalities for eosinophilic gastrointestinal disorders will be summarized for both pediatric and adult populations. This review will focus on shared decision-making aspects in treating eosinophilic gastrointestinal disease with balancing efficacy, risks, patient preference, and quality of life.

Eosinophilic gastrointestinal disorders (EGIDs) are chronic, immune-mediated conditions characterized by eosinophil-predominant inflammation of the gastrointestinal tract, encompassing eosinophilic esophagitis (EoE) and non-EoE EGIDs (eosinophilic gastritis, gastroenteritis, enteritis, and colitis). Over the past 3 decades, their clinical recognition has increased substantially, driven by rising incidence and prevalence-particularly for EoE, which is no longer considered rare in Western countries. In contrast, non-EoE EGIDs remain uncommon and incompletely characterized. Data reveal significant geographic variation and evolving epidemiologic patterns in EGIDs, including rapidly increasing EoE rates in East Asia. Early-life environmental exposures, gene-environment interactions, and Westernization-associated factors may contribute to these trends. EoE is typically chronic and progressive, with diagnostic delay associated with fibrostenotic complications, thus highlighting the importance of early recognition and maintenance therapy. Although non-EoE EGIDs were historically considered episodic, emerging data indicate that persistent disease is common, especially in pediatric populations. Across the spectrum, atopic comorbidities are frequent, and psychological burden is substantial. Together, these findings highlight EGIDs as chronic, evolving disorders with increasing prevalence that require earlier recognition, and long-term monitoring and management. Coordinated research initiatives are needed to close major gaps in diagnostic tools and thresholds, natural history, and long-term care.