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Eosinophilic endomyocarditis is extremely rare in paediatrics. This case report aims to present the sequential changes from thrombotic to fibrotic stages of the cardiac involvement in a child with hypereosinophilic syndrome. Endomyocardial fibrosis progressively aggravated the severity of the mitral regurgitation. Bioprosthetic valve replacement was finally performed without recurrence of cardiac complications despite a late relapse of the underlying disease.

Eosinophil-mediated endomyocardial damage is a well-known complication in patients with hypereosinophilic syndromes (HES). Although management and survival have improved significantly, some patients continue to develop severe cardiomyopathy as a direct consequence of uncontrolled hypereosinophilia. Cardiologists play a key role in early detection and treatment. At the early generally asymptomatic stage, related to subendocardial eosinophilic infiltrates, elevation of the biomarker of cardiac damage (serum troponin) and cardiac MRI are the best tools for diagnosis. As disease progresses, patients typically develop intracardiac mural thrombi and may experience variable degrees of heart failure due to valve damage and/or subendocardial fibrosis, all of which are more readily detectable with traditional echocardiographic investigation. New imaging modalities such as strain imaging and specific sequences in MRI offer the perspective of detecting subtle perturbations and distinguishing inflammatory versus fibrotic stages. Endomyocardial biopsy may help in difficult settings, namely, when blood eosinophilia is not prominent, but may be non-contributive due to sampling issues or eosinophil degranulation or replacement by fibrosis, and must always be performed after careful consideration of the risk:benefit ratio. Although treatment of the HES itself should be managed by clinicians with expertise in this rare disorder with the aim of lowering eosinophil counts to prevent and treat eosinophil-mediated organ damage and dysfunction, cardiologists play a key role in managing the associated cardiopathy. There are no consensual disease-specific guidelines for treating eosinophil-mediated thrombotic complications and cardiopathy, which should be managed according to classical international recommendations.

Renal involvement in hypereosinophilic syndrome (HES) occurs in 5-10% of patients but remains poorly characterized. Diagnostic overlap with IgG4-related kidney disease (IgG4-RKD) presents clinical challenges. To characterize the clinicopathologic spectrum of histologically confirmed HES-related kidney disease, examine diagnostic differentiation from IgG4-RKD, and appraise therapeutic evidence. Systematic review with narrative synthesis following PROSPERO-registered protocol (CRD420261307200). Six databases were searched from inception to February 27, 2026. Dual independent screening and domain-specific GRADE assessment were performed. Due to uncontrolled study designs and high heterogeneity, narrative synthesis was conducted rather than quantitative meta-analysis. From 2, 247 records, 37 studies (42 cases) met inclusion criteria. Acute eosinophilic interstitial nephritis predominated (54.8%, 23/42), followed by thrombotic microangiopathy (19.0%, 8/42), chronic interstitial fibrosis (11.9%, 5/42), and glomerular lesions (14.3%, 6/42). Nine cases (21.4%) exhibited IgG4-RKD-like features with IgG4+/IgG+ ratios below 40% (median 21%, IQR 14-30%). However, substantial technical heterogeneity limits clinical applicability. Fourteen cases received anti-IL-5/IL-5Rα biologics; all reported improvement with no documented failures, suggesting publication bias. No randomized controlled trials exist for HES-related kidney disease. Descriptive pathology findings were rated low certainty (downgraded for selection bias from biopsy requirement). IgG4/IgG ratio observations were very low certainty (downgraded for risk of bias, inconsistency, and indirectness). Treatment efficacy conclusions were very low certainty (downgraded for all GRADE domains, with publication bias and indirectness being most critical). This systematic review provides hypothesis-generating observations on HES-related kidney disease with low to very low certainty evidence. The 40% IgG4/IgG ratio remains unvalidated and should not be used diagnostically. International prospective registries and individual patient data meta-analyses are needed to establish evidence-based management. These findings require prospective validation before clinical application.

Overlap between rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) (\"rhupus\") is recognized, but coexistence with a severe eosinophilic asthma syndrome is exceptionally rare. We describe a triple autoimmune overlap of RA, SLE, and hypereosinophilic asthma with systemic manifestations (HASM), initially managed as ANCA-negative eosinophilic granulomatosis with polyangiitis (EGPA) and subsequently re-classified in light of evolving concepts. A 44-year-old woman with a 10-year history of seropositive RA developed alopecia, Coombs-positive hemolytic anemia, hypocomplementemia, and ANA and anti-Sm positivity, fulfilling SLE criteria. While receiving prednisone, hydroxychloroquine and conventional DMARDs, she subsequently developed adult-onset asthma, chronic rhinosinusitis with nasal polyps, and marked hypereosinophilia (>3.5×10 /L). Secondary causes were excluded; bone marrow showed reactive eosinophilia and ANCA (indirect immunofluorescence and ELISA for MPO/PR3) remained negative. She was diagnosed and treated as ANCA-negative EGPA with high-dose glucocorticoids plus methotrexate and hydroxychloroquine, leading to rapid normalization of eosinophils and durable remission of asthma and sinus disease. In retrospect, and according to the ERS/GERM'O'P proposal, this eosinophilic disorder is best classified as HASM within the EGPA-hypereosinophilic spectrum because ANCA and biopsy-proven vasculitis were absent. The case illustrates the evolving boundary between EGPA and hypereosinophilic syndromes and extends the concept of rhupus to include an EGPA-spectrum eosinophilic asthma syndrome. New-onset eosinophilic asthma in patients with established rheumatic disease should prompt evaluation for EGPA-spectrum or hypereosinophilic disorders. Even when the final label is HASM rather than definite EGPA, timely institution of EGPA-type immunosuppression may avert organ damage.

Hypereosinophilic syndrome (HES) is a heterogeneous disorder characterised by persistent, abnormal eosinophil elevation in blood and tissues, with multisystem involvement. Cardiac manifestations are frequent in HES and significantly worsen prognosis. Recent evidence establishes a direct link between HES and myocardial infarction (MI), demonstrating that HES induces MI primarily via non-atherosclerotic mechanisms. Critically, HES-related MI is fundamentally distinct from atherosclerotic MI in pathogenesis, diagnosis and management. This paradigm shift redefines diagnostic frameworks for cardiovascular disease and provides targeted strategies for non-atherosclerotic MI. This review synthesises current knowledge on HES-associated MI, emphasising its unique epidemiology, pathophysiology, evidence-based diagnostic criteria and mechanism-specific therapies. Future research priorities are discussed to advance clinical translation.

Hypereosinophilic syndrome (HES) is characterized by eosinophilia associated with organ damage. The disorder has substantial clinical heterogeneity and a highly variable prognosis. This report describes an interesting autopsy case of a 62-year-old lady presenting with itching and stroke-like symptoms. She was diagnosed with an \"idiopathic\" variant of HES after a thorough exclusion of all known causes. Despite adequate measures, she deteriorated rapidly. At autopsy, acute cerebral infarcts were identified in multiple vascular territories including infarcts in watershed areas. Additionally, her heart showed classic pathological features of eosinophilic myocarditis spanning all three stages.

Idiopathic hypereosinophilia (IHE) and hypereosinophilic syndrome (HES) are benign haematological disorders. Studies have suggested that venous thromboembolism (VTE) is a rare but sometimes fatal complication of hypereosinophilia; however, data are limited. We retrospectively analysed clinical features and short-term outcomes of 63 consecutive patients (82.5% men; mean age, 40.92 ± 10.89 years) with IHE or HES with concurrent VTE from January 1998 through December 2018. Risk factors for pulmonary embolism (PE) were explored by multivariate logistic analysis. DVT and/or PE was detected by imaging in all patients. Independent risk factors for PE were a body mass index of >24.1 kg/m 2 (odds ratio [OR]: 5.62, 95% confidence interval [CI]: 1.21–26.13, P = 0.028), peak absolute eosinophil count of >6.3 × 10 9 /L (OR: 5.55, 95% CI: 1.292–23.875, P = 0.021), and >13.9-month duration of hypereosinophilia (OR: 4.51, 95% CI: 1.123–18.09, P = 0.034). All patients were treated with corticosteroids and anticoagulants. The short-term hypereosinophilia remission rate was 100%; no recurrent VTE or major bleeding was observed. Hypereosinophilia is a potential risk factor for VTE. PE in patients with IHE/HES and DVT is associated with a higher body mass index, higher peak absolute eosinophil count, and longer duration of hypereosinophilia. Corticosteroids and anticoagulants provided effective short-term control of hypereosinophilia and VTE.

Hypereosinophilic syndrome and mastocytosis are relatively rare proliferative diseases encountered in the general population. However, allergists frequently consider these disorders in the differential of patients presenting with gastrointestinal, pulmonary, cutaneous, and allergic symptoms. Gastrointestinal symptoms are some of the most frequent and/or debilitating aspects of both disease states and in many cases lead to poor quality of life and functional limitation for the patient. They are the third most common clinical manifestation in hypereosinophilic syndrome and have been found to be the most distressful aspect of the disorder in those with systemic mastocytosis. Both eosinophils and mast cells play integral parts in normal gut physiology, but when and how exactly their effector functionality translates into clinically significant disease remains unclear, and the available literature regarding their pathophysiology remains sparse. Eosinophils and mast cells even, in fact, may not necessarily function in isolation from each other but can participate in bidirectional crosstalk. Both are affected by similar mediators and can also influence one another in a paracrine fashion. Their interactions include both production of soluble mediators for specific eosinophil and mast cell receptors (for example, eosinophil recruitment and activation by mast cells releasing histamine and eotaxin) as well as direct physical contact. The mechanistic relationship between clonal forms of hypereosinophilia and systemic mastocytosis has also been explored. The nature of gastrointestinal symptomatology in the setting of both hypereosinophilic syndrome and mast cell disease is frequently manifold, heterogeneous, and the lack of better targeted therapy makes diagnosis and management challenging, especially when faced with a substantial differential. Currently, the management of these gastrointestinal symptoms relies on the treatment of the overall disease process. In hypereosinophilia patients, systemic corticosteroids are mainstay, although steroid-sparing agents such as hydroxyurea, IFN-α, methotrexate, cyclosporine, imatinib, and mepolizumab have been utilized with varying success. In mastocytosis patients, anti-mediator therapy with antihistamines and mast cell stabilization with cromolyn sodium can be considered treatments of choice, followed by other therapies yet to be thoroughly studied, including the role of the low-histamine diet, corticosteroids, and treatment of associated IBS symptoms. Given that both eosinophils and mast cells may have joint pathophysiologic roles, they have the potential to be a combined target for therapeutic intervention in disease states exhibiting eosinophil or mast cell involvement.

Hypereosinophilic Syndrome (HES) is a rare disorder characterized by persistent elevation of eosinophils, leading to multi-organ infiltration and damage. Eosinophilic Myocarditis (EM) is one of its severe complications contributing significantly to morbidity and mortality. Herein, we describe the diagnostic and therapeutic challenges of EM, emphasizing the significance of early recognition and multidisciplinary management. A 51-year-old female with a history of EM, heart failure, and peripheral eosinophilia presented with NYHA class 3b symptoms. Laboratory findings revealed elevated peripheral eosinophil count, NT-Pro BNP, and characteristic electrocardiogram abnormalities. Imaging studies confirmed biventricular thrombi and myocardial abnormalities consistent with EM. Treatment involved Solu-Medrol for HES and heparin for ventricular thrombi, leading to initial clinical improvement. However, refractory heart failure necessitated urgent heart transplantation. EM, an under-recognized complication of HES, poses diagnostic and management challenges. Management includes standard heart failure treatments, steroids, and emerging therapies like Mepolizumab. Early diagnosis and aggressive management are pivotal for improving outcomes in this rare and potentially fatal condition. Advancements in the detection of complications, surgical management, and therapeutic options have improved outcomes in HES. Ongoing research is essential to further understand and address the diagnostic and therapeutic challenges of HES and EM.