INTRODUCTION

A 23-year-old young female patient presenting with persistent diarrhea and eosinophilia, who had clinical improvement after administration of steroids.

A 23-year-old Filipino female, without comorbidities presented with abdominal pain, diarrhea and vomiting. Initial work-up showed peripheral eosinophilia. Computed tomography (CT) of the abdomen revealed massive ascites. An esophagogastroduodenoscopy (EGD) was done which revealed acute duodenitis. Gastric and duodenal biopsy revealed tissue eosinophilia. Total Immunoglobulin E (IgE) was elevated while work-up for intestinal parasitic infection was negative. On bone marrow biopsy, there was no eosinophilic infiltration. Oral prednisone was then started at 60mg/day and was subsequently tapered over 30 days resulting in resolution of symptoms.

This report illustrates a rare case of eosinophilic gastroenteritis (EGE). EGE may mimic a wide spectrum of gastrointestinal disorders, hence, prompt recognition of EGE and awareness of its clinical symptoms, diagnosis and treatment is important.

Non-esophageal eosinophilic gastrointestinal disorder (non-EoE EGID) is a group of immune-mediated gastrointestinal diseases characterized by infiltration of eosinophils. Although most patients experience symptom relief after treatment, some still face the risk of persistent symptoms or relapse. Improving the prognosis for this subset of patients remains an urgent challenge. Identifying risk factors that affect the prognosis of non-EoE EGID and providing timely effective interventions are crucial for improving outcomes. This paper reviews the risk factors related to the prognosis of pediatric non-EoE EGID, including genetic factors, allergies, environmental factors, clinical characteristics, endoscopic findings, and pathological manifestations, with the aim of providing references for clinical decision-making.
Humans ; Prognosis ; Risk Factors ; Child ; Eosinophilia/etiology* ; Gastritis/etiology* ; Enteritis/etiology*

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multi-system disease that presents significant diagnostic challenges due to its complexity and low incidence (White and Dubey, 2023). It affects males and females equally, though males may exhibit more active disease at diagnosis and often require more aggressive treatment (Liu et al., 2023). The hallmark features of EGPA include delayed-onset asthma, eosinophilia in tissues and blood, and vasculitis affecting small to medium-sized arteries (White and Dubey, 2023). EGPA falls under the category of antineutrophil cytoplasmic antibody (ANCA)‍-associated vasculitis (AAV), whereas only about half of EGPA patients test positive for ANCA (Khoury et al., 2023).
Humans ; Male ; Hemorrhage/etiology* ; Granulomatosis with Polyangiitis/complications* ; Heart Arrest/etiology* ; Early Diagnosis ; Eosinophilia/diagnosis* ; Kidney Diseases/etiology* ; Churg-Strauss Syndrome/complications* ; Middle Aged

Objective:To identify the key epithelial cell characteristics that can accurately diagnose eosinophilic chronic sinusitis with nasal polyps（ECRSwNP） through nasal brush sampling and comparing with the pathological results of nasal polyp tissue sections. Methods:Ninety-one patients underwent surgery in the Ophthalmology and ENT Department of the Second People's Hospital of Longgang District, Shenzhen, from January 2022 to July 2024 were selected. The cohort comprised 58 males and 33 females（mean age: 41.4 years; range: 12.0-71.0）. The clinical characteristics of the patients, including gender, age, disease duration, smoking and drinking history, asthma history, subjective symptoms, sinus CT, and nasal endoscopy scores, were recorded. Nasal brush sampling of nasal polyps and inferior turbinate mucosa was performed before surgery to obtain cytological specimens, and nasal polyp tissues were collected during surgery. The demographic and clinical characteristics of patients with eosinophilic and non-eosinophilic nasal polyps were compared, as well as the relationship between nasal brush cytology of nasal polyps and inferior turbinate and nasal polyp histopathology. Statistical analysis was performed using SPSS 23.0 software. Results:Among the 91 patients, no significant differences were observed between ECRSwNP and NECRSwNP patients in terms of age, gender, smoking status, alcohol consumption, and disease duration. The nasal brush cell population in ECRSwNP patients was more likely to contain eosinophils（P<0.001） and less likely to contain lymphocytes and plasma cells（P<0.001）. Additionally, the ciliated cells in ECRSwNP patients exhibited larger widths（P=0.036）, shorter cilium lengths（P<0.001）, and more disordered arrangements（P<0.001） compared to NECRSwNP patients. In nasal brush cells from the inferior turbinate, ECRSwNP patients also showed shorter cilium lengths（P<0.001） and shorter cilia（P=0.024） compared to NECRSwNP patients. Conclusion:There are significant differences in obtaining epithelial cytological information from nasal polyps or inferior turbinates through nasal brush sampling between ECRSwNP and NECRSwNP patients.
Humans ; Male ; Female ; Middle Aged ; Adult ; Nasal Polyps/complications* ; Sinusitis/complications* ; Aged ; Chronic Disease ; Adolescent ; Nasal Mucosa/pathology* ; Young Adult ; Rhinitis/complications* ; Eosinophilia/pathology* ; Child ; Eosinophils/pathology* ; Rhinosinusitis

Cytoskeletal network dysregulation is a pivotal determinant in various immunodeficiencies and autoinflammatory conditions. This report reviews the significance of actin remodeling in disease pathogenesis, focusing on the Arp2/3 complex and its regulatory subunit actin related protein 2/3 complex subunit 1B (ARPC1B). A spectrum of cellular dysfunctions associated with ARPC1B deficiency, impacting diverse immune cell types, is elucidated. The study presents a patient featuring recurrent and persistent eosinophilia attributed to homozygous ARPC1B mutation alongside concomitant compound heterozygous cystic fibrosis transmembrane conductance regulator (CFTR) gene mutations. We used ARPC1B antibody to stain the patient's peripheral blood lymphocytes and those of the control. The defect in the ARPC1B gene in the present patient caused absent/low expression by immunofluorescence microscopy. The intricate interplay between cytoskeletal defects and immunological manifestations underscores the complexity of disease phenotypes, warranting further exploration for targeted therapeutic strategies.
Humans ; Actin-Related Protein 2-3 Complex/genetics* ; Cystic Fibrosis Transmembrane Conductance Regulator/genetics* ; Eosinophilia/genetics* ; Homozygote ; Mutation ; Recurrence

Chronic rhinosinusitis（CRS） is an inflammatory disease involving the mucosa of the nasal and paranasal sinuses for more than 12 weeks and can be classified as CRS with nasal polyp（CRSwNP） and CRS without nasal polyp（CRSsNP） depending on the phenotype. Clinical treatments reveal significant differences in disease prognosis and improvement in quality of life in patients with the same clinical phenotype. Inflammatory cells infiltration and inflammatory mediators are important factors driving CRS endotypes. In particular, CRS with predominantly eosinophilic infiltration and type 2 CRS present severe clinical symptoms, comorbidities, and high recurrence rates. CRS endotype-oriented treatment methods may better contribute to improving patient prognosis and quality of life. This article summarizes the current progress of CRS endotype research and reviews the endotype-oriented treatment options.
Humans ; Rhinitis/therapy* ; Nasal Polyps/diagnosis* ; Quality of Life ; Sinusitis/diagnosis* ; Eosinophilia ; Chronic Disease

OBJECTIVE: To evaluate the clinical features, laboratory and imaging results, treatment and outcomes of eosinophilic fasciitis (EF) and assess the value of ultrasound in the diagnosis of EF. METHODS: We retrospectively analyzed the clinical data of 45 patients with EF treated in our center from January 1, 2006 to February 28, 2022. The consistency between the diagnoses of EF based on ultrasound and MRI findings was assessed. RESULTS: In the 45 EF patients (male/female ratio 3.5:1), the age of onset ranged from 16 to 64 years with a mean disease course of 22.6 months. The average time from symptom onset to diagnosis was 16 months. The most common possible trigger of the disease was vigorous exercise (10/45), causing symmetrical lesions in the limbs, most commonly in the forearms (86.7%) and lower legs (80%). Clinical features of EF included subcutaneous swelling and induration (95.6%), arthralgia and arthritis (55.6%), groove sign (42.2%), hand joint contractures (42.2%), skin pigmentation (37.8%), and peau d'orange appearance (13.3%). Eosinophilia was found in 31 patients (68.9%). Hypergammaglobulinemia was seen in 23/44 (52.3%) and positive antinuclear antibodies in 9 (20%) of the patients. Twentyone of the patients were treated with high-dose methylprednisolone (≥200 mg daily for 3 to 5 consecutive days), and compared with the patients who did not receive this treatment, these patients more frequently experienced relapse before admission, had more extensive involvement, and had a higher rate of hypergammaglobulinemia without fever, but these differences were not statistically significant. Of the 31 patients (68.9%) with follow-up data (for a median of 3.2 years [range 0.2-15.9]), complete remission was achieved in 12 (38.7%) patients, and the accumulative complete remission rate was 44.1% at 5.5 years. No specific baseline characteristics or immunosuppressants were found to correlate with the treatment response. A total of 26 patients underwent both ultrasound and MRI examination, and the Kappa value of the diagnostic results between ultrasound and MRI was 0.91. CONCLUSION EF is characterized by symmetrical subcutaneous swelling and induration in the limbs, accompanied by eosinophilia and hypergammaglobulinemia. Glucocorticoid is effective for treating EF. Ultrasound examination can identify thickening of subcutaneous fascia for an early diagnosis of EF.
Humans ; Female ; Male ; Infant ; Child, Preschool ; Retrospective Studies ; Hypergammaglobulinemia ; Eosinophilia ; Ultrasonography ; Hand ; Contracture ; Treatment Outcome

OBJECTIVE: To investigate the risk factors and prognosis of cardiovascular damage in hypereosinophilia (HE). METHODS: The clinical data of 62 patients with HE in Gansu Provincial Hospital from January 2015 to December 2020 were retrospectively analyzed, including clinical characteristics and laboratory indicators, and the influencing factors of survival and prognosis were also analyzed. RESULTS: In this study, there were 34 males and 28 females, with a median age of 53.5 (20-79) years, 35 patients without cardiovascular damage, 27 patients with cardiovascular damage, including 22 patients with abnormal electrocardiogram (ECG) (81.5%), 18 patients with abnormal echocardiography (ECHO) (66.7%), 9 patients with single ECG abnormality, 5 patients with single ECHO abnormality, and other 13 patients with multiple abnormalities. In cardiovascular damage group, peripheral white blood cell count, absolute value of eosinophils, troponin T (TNT), N-terminal pro-B-type natriuretic peptide (NT-proBNP), interleukin (IL)-4 and IL-5 levels at initial diagnosis were significantly higher than those in the non-cardiovascular damage group (P <0.01), while hemoglobin, IL-2 and interferon-γ levels were significantly lower (P <0.01). There were no significant differences in age, sex, course of disease, etiological classification, platelet count, serum creatine kinase, serum creatine kinase isoenzyme and lactate dehydrogenase between the two groups (P >0.05). The 5-year overal survival rate of patients with cardiovascular damage was 88.9%, and that of patients without cardiovascular damage was 100%, the difference was statistically significant (P =0.012). The 5-year event-free survival (EFS) rate of patients with cardiovascular damage was 59.3%, and the median time was 37 (21-52) months, while that of patients without cardiovascular damage was 80%, and the median time was 63 (51-74) months (P =0.002). Age (>60 years old), course of disease (>24 months), NT-proBNP (>3 000 pg/ml), TNT (>100 ng/L), elevated IL-4 and IL-5 were associated with EFS shortening in patients with cardiovascular damage, which were independent risk factors for EFS. CONCLUSION The EFS rate in HE patients without cardiovascular damage is significantly higher than patients with cardiovascular damage. Age, course of disease, NT-proBNP, TNT, IL-4 and IL-5 are independent risk factors affecting EFS of patients with cardiovascular damage.
Male ; Female ; Humans ; Middle Aged ; Aged ; Interleukin-4 ; Biomarkers ; Retrospective Studies ; Interleukin-5 ; Prognosis ; Risk Factors ; Eosinophilia ; Peptide Fragments ; Natriuretic Peptide, Brain

Eosinophilic gastroenteritis (EGE) is a gastrointestinal disorder of unclear etiology that is characterized by eosinophilic infiltration of the stomach and small intestine, and consists of mucosal, muscular, and serosal subtypes. Eosinophilic infiltration of the gastrointestinal tract is a fundamental histopathological characteristic of EGE and is driven by several T-helper type 2 (Th2)-dependent cytokines and induced by food allergy. Due to the lack of a diagnostic gold standard, EGE has a high rate of delayed diagnosis or misdiagnosis. However, several new diagnostic strategies have been developed, such as novel genetic biomarkers and imaging tests. Although dietary therapy and corticosteroids remain the common choices for EGE treatment, recent decades have seen the emergence of novel treatment alternatives, such as biologics that target particular molecules involved in the pathogenic process. Preliminary investigations and clinical trials have demonstrated the efficacy of biologics and provided additional insights for the era of refractory or corticosteroid-dependent EGE biologics.
Humans ; Enteritis/drug therapy* ; Gastritis/drug therapy* ; Eosinophilia/therapy* ; Abdomen ; Adrenal Cortex Hormones

Humans ; Carcinoma, Mucoepidermoid/pathology* ; Thyroid Neoplasms/pathology* ; Eosinophilia/pathology*