Eosinophilic syndromes

DISEASE OVERVIEW: The eosinophilias encompass a broad range of non-hematologic (secondary or reactive) and hematologic (primary or clonal) disorders with the potential for end-organ damage. DIAGNOSIS: Hypereosinophilia (HE) has generally been defined as a peripheral blood eosinophil count greater than 1.5 × 109 /L, and may be associated with tissue damage. After the exclusion of secondary causes of eosinophilia, diagnostic evaluation of primary eosinophilias relies on a combination of various tests...

INTRODUCTION: Eosinophilic granulomatosis with polyangiitis (EGPA) was formerly known as Churg-Strauss syndrome. The condition is characterized by disseminated necrotizing vasculitis with extravascular granulomas associated with hypereosinophilia. The vasculitides affect small vessels and are associated with antineutrophil cytoplasmic antibodies (ANCAs) detectable in the blood. Distinguishing between type 2-mediated chronic airway inflammation such as chronic rhinosinusitis with nasal polyps (CRSwNP) without vasculitis can be clinically challenging and should be considered...

Eosinophil-associated diseases (EADs) refer to heterogeneous conditions in which eosinophils are believed to play critical pathological roles. They encompass common respiratory conditions, such as asthma, chronic rhinosinusitis with nasal polyps (CRSwNP), less common primary eosinophilic disorders of gastrointestinal tract, and rare conditions including eosinophilic granulomatosis with polyangiitis (EGPA) and hypereosinophilic syndrome (HES). A literature search was carried out in January 2024 in the MEDLINE and Scopus databases using the PubMed search engine (PubMed, National Library of Medicine, Bethesda, MD)...

Myocarditis is a polymorphic and potentially life-threatening disease characterized by a large variability in clinical presentation and prognosis. Within the broad spectrum of etiology, eosinophilic myocarditis represents a rare condition characterized by eosinophilic infiltration of the myocardium, usually associated with peripheral eosinophilia. Albeit uncommon, eosinophilic myocarditis could be potentially life-threatening, ranging from mild asymptomatic disease to multifocal widespread infiltrates associated with myocardial necrosis, thrombotic complications, and endomyocardial fibrosis...

OBJECTIVES: Antinuclear antibodies (ANA) of the IgE-type have been described in several connective tissue disorders (CTD) but not yet in systemic sclerosis (SSc). Aim of the study was, therefore, to establish an ELISA for the demonstration of IgE-autoantibodies to topoisomerase-I (topo-I) and the centromeric proteins A and B (CENP-A/B), to assess their prevalence and reactivity in SSc and to analyse their clinical relevance. METHODS: One hundred fifty-one patients with SSc and 88 with CREST-syndrome, 291 patients with other CTD, and 23 patients with fibromyalgia syndrome (FM) as a control collective were included into the study...

PURPOSE: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of vasculitis with multiorgan involvement. The incidence and prevalence of EGPA vary geographically and ethnically. This study investigated the incidence, prevalence, and mortality of EGPA in a nationwide population-based cohort in Korea. METHODS: This retrospective cohort study used data from the National Health Insurance database that covers almost all Korean residents. EGPA was identified using relevant diagnostic codes from 2007 to 2018...

Eosinophil sombrero vesicles (EoSVs) are large tubular carriers resident in the cytoplasm of human eosinophils, identifiable by transmission electron microscopy (TEM), and important for immune mediator transport. Increased EoSV formation occurs in activated eosinophils in vitro and in vivo. In tissue sites of eosinophilic cytolytic inflammation, extracellular EoSVs are noted, but their frequency and significance in eosinophil-associated diseases (EADs) remain unclear. Here, we performed comprehensive quantitative TEM analyses and electron tomography to investigate the numbers, density, integrity, and three-dimensional (3D) structure of EoSVs in different biopsy tissues from five prototypic EADs (eosinophilic chronic rhinosinusitis/nasal sinuses, ulcerative colitis/intestines, hypereosinophilic syndrome/skin, dermatitis/skin, and schistosomiasis/rectum)...

BACKGROUND AND AIMS: Elevated eosinophils typically indicate hypersensitive inflammation; however, their involvement in cardiovascular events remains incompletely understood. We investigated the association between the absolute eosinophil count (AEC) and major adverse cardiovascular and cerebrovascular events (MACCEs) in patients with acute coronary syndrome (ACS) undergoing percutaneous coronary intervention (PCI). Additionally, we determine whether the integration of AEC with the SYNTAX II score could improve predictive ability...

We present a rare case of a 45-year-old woman with pseudo-Meigs' syndrome and eosinophilic pleural effusion (EPE). She experienced cough, sputum, and dyspnea with a large right pleural effusion. Laboratory tests showed eosinophilia in the blood and pleural fluid. An ovarian tumor and ascites were also detected. After left salpingo-oophorectomy, the tumor was diagnosed as a mature cystic teratoma of the left ovary. The right-sided pleural effusion gradually resolved. Pseudo-Meigs' syndrome is characterized by benign ovarian tumor, ascites, and pleural effusion...

Patients with short bowel syndrome (SBS) have multiple risk factors for eosinophilic gastrointestinal diseases (EGIDs) including increased risk for intestinal dysbiosis and food allergy compared to their counterparts with normal anatomy. However, there is limited data on the prevalence of EGIDs in children with SBS. We aimed to define the prevalence of EGIDs in an SBS cohort and its association with different risk factors via a retrospective chart review of patients with SBS at Children's National Hospital...

Eosinophilic gastroenteritis is a rare disease with an unknown cause, which can manifest independently or as part of a hyper-eosinophilic syndrome. The severity of the condition depends on the extent of eosinophilic infiltration and damage to the digestive tract. Diagnosis relies on histological examination, which reveals a significant presence of eosinophilic polymorphonuclear leukocytes in the digestive wall. The authors present a new case of eosinophilic gastroenteritis in a 28-year-old patient who exhibited obstructive symptoms but lacked peripheral eosinophilia...

This article reports the case of a 30-year-old female patient who suffered for many years from initially unspecific symptoms, such as recurrent, nonallergic and noninfectious sinusitis, late-onset bronchial asthma and pronounced lymphadenopathy; however, the correct diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) could only be made by histological investigations after the appearance of skin symptoms. The EGPA is a severe systemic disease which, if left untreated, can cause multiple organ damage and even be fatal...

Pancreatic neuroendocrine microtumor (PNEMT) is a neuroendocrine tumor (NET) < 0.5 cm in diameter, and it is considered benign. We report a PNEMT with high-grade transformation (HGT). A man in his 60s with von Hippel-Lindau syndrome underwent surgical resection of a NET. A second sub-centimeter nodule with a nodule-in-nodule pattern was discovered. The 0.4 cm outer nodule contained clear columnar cells with round nuclei and indistinct nucleoli, while the 0.1 cm inner nodule had eosinophilic cells with an increased nuclear to cytoplasmic ratio, vesicular nuclei, and prominent nucleoli...

Cow's milk allergy (CMA) is one of the most common and complex presentations of allergy in early childhood. CMA can present as IgE and non-IgE mediated forms of food allergy. Non-IgE mediated CMA includes food protein-induced enterocolitis syndrome (FPIES), eosinophilic gastrointestinal disorders (EGIDs), and food protein-induced proctocolitis (FPIAP). There are recent guidelines addressing CMA diagnosis, management, and treatment. Each of these guidelines have their own strengths and limitations. To best manage CMA, individualized avoidance advice should be given...

Background. Fixed drug eruption and Rowell syndrome stand as intriguing entities with overlapping clinical and pathological features. Case Presentation . A 32-year-old female patient presented with a tender and pruritic rash on the left upper chest for 3 days. Clinical examination revealed a flaring rash on the chest, under her left eye, tongue, and lips. The patient had a significant past medical history of systemic lupus erythematous with positive (ANA, Sm, dsDNA, ribosomalP, RNP) antibodies, hypocomplementemia, inflammatory arthritis, discoid lupus, leukopenia, thrombocytopenia, and immune thrombocytopenic purpura, and avascular necrosis affecting both hips and her right knee...

Despite its recognition as an 'ANCA-associated vasculitis' (AAV), eosinophilic granulomatosis with polyangiitis (EGPA) is ANCA negative in up to 60% of cases. Herein, we report the case of a young man with a clinical syndrome highly suggestive of EGPA but with repeated negative ANCA serology, ultimately presenting with cardiac arrest before recognition of the primary systemic vasculitis, whereupon he received successful induction therapy with high dose glucocorticoids and cyclophosphamide. The case illustrates the importance of awareness of ANCA negative AAV among general physicians in order to minimise morbidity and mortality...

BACKGROUND: Systemic inflammatory response syndrome (SIRS) and sepsis are the most common causes of in-hospital death. However, the characteristics associated with the improvement in the patient conditions during the ICU stay were not fully elucidated for each population as well as the possible differences between the two. GOAL: The aim of this study is to highlight the differences between the prognostic clinical features for the survival of patients diagnosed with SIRS and those of patients diagnosed with sepsis by using a multi-variable predictive modeling approach with a reduced set of easily available measurements collected at the admission to the intensive care unit (ICU)...

Wells' syndrome, or eosinophilic cellulitis, is an inflammatory disease of unknown origin, uncommon in the pediatric age. It usually appears clinically as erythematous and edematous plaques, nodules, papules, blisters, among other symptoms. Here we describe the case of a female pediatric patient with generalized, asymptomatic subcutaneous nodules associated with severe eosinophilia. The histopathological examination of the lesions was compatible with Wells' syndrome. An interdisciplinary evaluation was performed to establish the cause and look for associated eosinophilic disorders; the results were negative...

Kimura disease (KD), also known as eosinophilic lymphogranuloma, is a rare chronic inflammatory or allergic disease. It can present with immune-related diseases such as nephrotic syndrome, asthma, and ankylosing spondylitis. In this study, we report a case of KD combined with immunoglobulin A nephropathy that first presented as a mass in the inguinal region, followed by recurrent renal involvement. Previous reports suggested that renal involvement caused by KD was due to direct infiltration of eosinophils; however, in this case, no eosinophil infiltration was found in the renal tissue after renal biopsy...

Combined radiation with hemorrhage (combined injury, CI) exacerbates hematopoietic acute radiation syndrome and mortality compared to radiation alone (RI). We evaluated the effects of RI or CI on blood cell depletion as a biomarker to differentiate the two. Male CD2F1 mice were exposed to 8.75 Gy γ-radiation (60 Co). Within 2 h of RI, animals were bled under anesthesia 0% (RI) or 20% (CI) of total blood volume. Blood samples were collected at 4-5 h and days 1, 2, 3, 7, and 15 after RI. CI decreased WBC at 4-5 h and continued to decrease it until day 3; counts then stayed at the nadir up to day 15...