Asta Literature Retrieval: Pathophysiology and clinical mechanisms of Eosinophilic granulomatosis with polyangiitis. Core disease mechanisms, mo...

This report is retrieval-only and is generated directly from Asta results.

• Papers retrieved: 18
• Snippets retrieved: 20

Relevant Papers

[1] Eosinophilic granulomatosis with polyangiitis: etiopathogenesis, classification and clinical phenotypes

• Authors: С. Белевский, Н. П. Княжеская, E. K. Anaev, A. S. Belevskiy, N. P. Kniajeskaia
• Year: 2022
• Venue: PULMONOLOGIYA
• URL: https://www.semanticscholar.org/paper/6bc7ae346bde1bd7ec4340a06a5105c85dbb015f
• DOI: 10.18093/0869-0189-2022-4101
• Citations: 1
• Summary: Current knowledge of eosinophilic and ANCA-mediated aspects of the pathogenesis, classification and clinical phenotypes of EGPA are presented, and prospects for future research are considered.
• Evidence snippets:
• Snippet 1 (score: 0.659) > Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic disease that can be classified as both a hypereosinophilic condition and an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis and is characterized by granulomatous inflammation. The pathogenesis of EGPA is not completely understood. It is likely that this disease is Th2-mediated, and blood and tissue eosinophilia serves as the main diagnostic criterion. The hallmarks and main effectors of organ damage in EGPA include asthma-associated necrotizing vasculitis of small-to-medium vessels and eosinophilic proliferation. Endothelial injury and vascular inflammation in EGPA is caused by ANCA via activation of circulating neutrophils. Two clinical phenotypes of the disease have been described based on the detection of ANCA: ANCA-negative with manifestations of hypereosinophilia (for example, pulmonary infiltrates and cardiomyopathy) and ANCA-positive with clinical signs of vasculitis (for example, glomerulonephritis, purpura, and mononeuritis multiplex). Both phenotypes were confirmed by histological and genomic research. However, these two coexisting mechanisms cannot be separated in clinical practice. The aim of the article is to present current knowledge of eosinophilic and ANCA-mediated aspects of the pathogenesis, classification and clinical phenotypes of EGPA, and consider prospects for future research. Conclusion. The development of EGPA is based on eosinophilic dysfunction. This dysfunction means that patients with a genetically determined predisposition to recognize the ANCA antigen and with HLA-DQ (human leukocyte antigen DQ) alleles produce anti-myeloperoxidase autoantibodies and later develop an aberrant autoimmune process. Further comprehensive post-genomic studies are needed to identify the pathogenetic mechanisms and characterize molecular features of EGPA clinical phenotypes. The elaboration of molecular endotypes will lead to the identification of new activity biomarkers and therapeutic targets that can improve the diagnosis of

[2] New therapeutic approaches with biological drugs for eosinophilic granulomatosis with polyangiitis

• Authors: A. Carrón-Herrero, C. Pelaia, G. Paoletti
• Year: 2023
• Venue: Exploration of Asthma & Allergy
• URL: https://www.semanticscholar.org/paper/8b38e78eeb89ade72e24d1e4c20baff80eebcfdd
• DOI: 10.37349/eaa.2023.00006
• Citations: 2
• Summary: The present review describes the new therapeutical approaches with biological drugs for EGPA and suggests emerging targeted biotherapies under evaluation could lead to a phenotype-based approach and personalised treatment regimens for EGpa patients.
• Evidence snippets:
• Snippet 1 (score: 0.652) > Eosinophilic granulomatosis with polyangiitis (EGPA) is a multiorganic syndrome that affects the cardiovascular, neurologic, renal, and gastrointestinal systems with an incidence ranging from 0 case to 67 cases per one million person-years, and its pathophysiology remains unknown. It is believed that genetic factors, the environment, and changes in immune system function contribute to the development of EGPA, overlapping the immune mechanisms of vasculitides and the pathologic mechanisms in eosinophilic syndromes. This disease is commonly divided into two phenotypes depending on the presence of antineutrophil cytoplasmic antibodies (ANCA). ANCA-positive patients usually have more vasculitic manifestations like peripheral neuropathy, purpura, renal involvement, and biopsy-proven vasculitis. The keystone of EGPA therapy is systemic corticosteroids (CS) as monotherapy or in combination with other immunosuppressive treatments, and recently the efficacy of eosinophil-targeted biotherapy, anti-interleukin-5 (IL-5), has been shown to be efficacious in EGPA. Although this phenotype/phase distinction has not yet had an impact on the current treatment strategies, emerging targeted biotherapies under evaluation could lead to a phenotype-based approach and personalised treatment regimens for EGPA patients. The present review describes the new therapeutical approaches with biological drugs for EGPA.

[3] Eosinophilic Granulomatosis With Polyangiitis: Dissecting the Pathophysiology

• Authors: F. Fagni, F. Bello, G. Emmi
• Year: 2021
• Venue: Frontiers in Medicine
• URL: https://www.semanticscholar.org/paper/f76c16b5a594f6a860c76ddf5053860096b9b61d
• DOI: 10.3389/fmed.2021.627776
• PMID: 33718405
• PMCID: 7943470
• Citations: 84
• Influential citations: 6
• Summary: The present review describes the current knowledge on the eosinophilic and ANCA-mediated aspects of EGPA pathogenesis and reviews the rationale of the currently proposed EGPA dichotomy and future research perspectives.
• Evidence snippets:
• Snippet 1 (score: 0.641) > Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare multisystemic disease classified both amongst hypereosinophilic disorders and ANCA-associated vasculitis. Vessel inflammation and eosinophilic proliferation are the hallmarks of the disease and main effectors of organ damage. Two distinct disease phenotypes have classically been described according to ANCA-status: the ANCA-negative subset with eosinophil-driven manifestation and the ANCA-positive one with vasculitic manifestations. An analogous dichotomization has also been backed by histological findings and a distinct genetic background. EGPA is typically consider a Th2-mediated disease and blood and tissue eosinophilia represent the cornerstone of diagnosis. Besides, ANCA are known for inducing endothelial injury and vascular inflammation by activating the circulating neutrophils. Thus, the pathogenesis of EGPA seems to be mediated by two coexisting mechanisms. However, the verbatim application of this strict dualism cannot always be translated into routine clinical practice. In the present review we describe the current knowledge on the eosinophilic and ANCA-mediated aspects of EGPA pathogenesis. Finally, we review the rationale of the currently proposed EGPA dichotomy and future research perspectives.
• Snippet 2 (score: 0.588) > Eosinophilic Granulomatosis With Polyangiitis: Dissecting the Pathophysiology
• Snippet 3 (score: 0.573) > Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare disease characterized by granulomatous and eosinophil rich inflammation and systemic necrotizing vasculitis affecting small-to-medium sized vessels. EGPA occurs in patients with asthma and peripheral and tissue eosinophilia, and ∼30% of the patients present antineutrophil cytoplasm antibodies (ANCA) mainly specific for myeloperoxidase (MPO) (1). The disease is unique in its genre as it combines asthmatic manifestations with hypereosinophilic disorders and ANCA-associated vasculitis (AAV) features. Therefore, a full comprehension of its pathophysiology still lies beyond our reach. > An increasing amount of evidence indicates that EGPA's clinical phenotypes tends to segregate according to ANCA-status, as the major eosinophil-driven complications are most frequently found in the ANCA-negative subset of EGPA, namely lung infiltrates, myocardiopathy, and gastrointestinal manifestations. In contrast, MPO-ANCA-positive patients present a more "vasculitic phenotype, " which comprises palpable purpura, peripheral neuropathy, rapidly progressive glomerulonephritis and, rarely alveolar hemorrhage (2, 3) (Table 1). An analogous dichotomy is also supported by histological findings, as biopsy-proven vasculitis is found more frequently in ANCA-positive patients than in ANCA-negative ones, whereas eosinophilic infiltrates and granulomas are found with a similar frequency in the two groups (24). > The dualism between ANCA-positive and ANCA-negative EGPA is also supported by genetic background. A recent genome wide association study (GWAS) found differential association of genetic variants between the two serological subsets.

[4] Heterogeneity and individualized therapy for eosinophilic granulomatosis with polyangiitis

• Authors: Lijuan Hua, Min Xie
• Year: 2025
• Venue: Therapeutic Advances in Respiratory Disease
• URL: https://www.semanticscholar.org/paper/ddebbd3725a5b8ba3ba88406b72519aa67a2704d
• DOI: 10.1177/17534666251318615
• PMID: 39980304
• PMCID: 11843704
• Citations: 2
• Summary: It is believed that EGPA has a highly heterogeneous phenotype spectrum, and the treatment patterns targeting key molecules in the pathogenesis are of great value for individual treatment of EGPA.
• Evidence snippets:
• Snippet 1 (score: 0.636) > Eosinophilic granulomatosis with polyangiitis (EGPA), as a heterogeneous component of antineutrophil cytoplasmic antibody-associated vasculitis, may be induced by a series of environmental and genetic factors, involved with a variety of immune cells and immune components, and presented with various clinical manifestations, with multiple organs and systems (respiratory, skin, heart, kidney, nerve, etc.) involved. The choice of glucocorticoid (GC) dosage and immunosuppressant in traditional treatment strategies varies greatly from individual to individual and is not universally applicable in all the EGPA phenotype spectrum, especially in relapsing or refractory diseases. With the understanding of the heterogeneity of EGPA, a variety of therapeutic approaches are emerging and improving the traditional treatment model. In this review, we summarized the heterogeneity of EGPA etiology and pathogenesis. Clinical and pathological manifestations of the same organ involved also show significant differences and there are even gender differences. Biological treatments that mainly target type 2 inflammatory pathways are widely used in clinical practice for remission induction and maintenance of EGPA. Targeted biological therapy has shown excellent performance in reducing GC dosage and controlling symptoms and recurrence. However, a large number of high-quality randomized controlled studies are still under research for relapsing or refractory EGPA with special organ involvement. We believe that EGPA has a highly heterogeneous phenotype spectrum, and the treatment patterns targeting key molecules in the pathogenesis are of great value for individual treatment of EGPA. Plain language summary More favorable treatment options for eosinophilic granulomatosis with polyangiitis – a rare blood vessel disease 1. Eosinophilic granulomatosis with polyangiitis is a rare disease characterized by inflammation of small and medium blood vessels. 2. Manifestations of the disease are diverse, and multiple organs may be involved. A variety of immune cells (cells that are part of the immune system and help fight infections and diseases) and mediators (molecules) secreted by these cells are involved in the development of diseases. 3. Traditional treatments use powerful medicines such as glu

[5] Pathogenesis of Pulmonary Manifestations in ANCA-Associated Vasculitis and Goodpasture Syndrome

• Authors: Evangelia Fouka, Fotios Drakopanagiotakis, P. Steiropoulos
• Year: 2024
• Venue: International Journal of Molecular Sciences
• URL: https://www.semanticscholar.org/paper/c56c403ca46e674e2b1606e0c93cf116f65f9713
• DOI: 10.3390/ijms25105278
• PMID: 38791316
• PMCID: 11121030
• Citations: 15
• Summary: The underlying pathogenesis of pulmonary involvement in vasculitis, encompassing various forms such as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and anti-GBM disease is explored.
• Evidence snippets:
• Snippet 1 (score: 0.597) > Pulmonary manifestations of vasculitis are associated with significant morbidity and mortality in affected individuals. They result from a complex interplay between immune dysregulation, which leads to vascular inflammation and tissue damage. This review explored the underlying pathogenesis of pulmonary involvement in vasculitis, encompassing various forms such as granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and anti-GBM disease. Mechanisms involving ANCA and anti-GBM autoantibodies, neutrophil activation, and neutrophil extracellular trap (NETs) formation are discussed, along with the role of the complement system in inducing pulmonary injury. Furthermore, the impact of genetic predisposition and environmental factors on disease susceptibility and severity was considered, and the current treatment options were presented. Understanding the mechanisms involved in the pathogenesis of pulmonary vasculitis is crucial for developing targeted therapies and improving clinical outcomes in affected individuals.

[6] Unmet needs and evidence gaps in hypereosinophilic syndrome and eosinophilic granulomatosis with polyangiitis.

• Authors: M. Wechsler, B. Hellmich, M. Cid, D. Jayne, X. Tian et al.
• Year: 2023
• Venue: The Journal of allergy and clinical immunology
• URL: https://www.semanticscholar.org/paper/2262c7717070288ce68dfff7a898c00942328bd5
• DOI: 10.1016/j.jaci.2023.03.011
• PMID: 37086239
• Citations: 34
• Summary: The limitations of the current understanding of HES and EGPA are outlined and areas for future work are highlighted, which ultimately should help improve patient management and outcomes.
• Evidence snippets:
• Snippet 1 (score: 0.589) > Hypereosinophilic syndrome (HES) and eosinophilic granulomatosis with polyangiitis (EGPA) are rare systemic inflammatory disorders with overlapping symptoms, elevated eosinophil counts, and heterogenous clinical presentations. Although progress has been made in recent years, there are substantial gaps in our understanding of the pathologic mechanisms involved in these diseases, as well as numerous unmet needs relating to both diagnosis and patient management. For example, in most cases of HES, the underlying cause of hypereosinophilia is unknown, while in EGPA, although a polygenic genetic susceptibility has been found, understanding of the pathogenic mechanisms remains largely elusive. Delineating differences between certain disease variants may be challenging, and there are no reliable predictive markers of disease course. In addition, the current diagnostic criteria for HES and classification criteria for EGPA are not easy to implement in a nonspecialist setting, and specialist referral pathways need to be signposted more clearly. Furthermore, disease-specific activity scores need to be developed to aid the assessment of treatment effects, and improved biomarkers are needed to aid with treatment stratification. In this review, we outline the limitations of our current understanding of HES and EGPA and highlight areas for future work, which ultimately should help improve patient management and outcomes.

[7] Effect of anti-interleukin-5 antibody on development of vasculitis in an ovalbumin-induced eosinophilic vasculitis mouse model

• Authors: Kiyoto Kageyama, Eri Kikuchi, N. Hoshino
• Year: 2025
• Venue: Frontiers in Pharmacology
• URL: https://www.semanticscholar.org/paper/81f2d78dd73ea59f7543ca4b6ef67edb6201a302
• DOI: 10.3389/fphar.2025.1546785
• PMID: 40176907
• PMCID: 11961647
• Citations: 1
• Summary: The OVA-induced eosinophilic vasculitis mouse model is used to evaluate and characterize its usefulness and may be useful for drug discovery targeting both eosinophilic and non-eosinophilic aspects of EGPA pathogenesis.
• Evidence snippets:
• Snippet 1 (score: 0.576) > Eosinophilic granulomatosis with polyangiitis (EGPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, distinct from other major forms of ANCA-associated vasculitis, such as granulomatosis with polyangiitis and microscopic polyangiitis. EGPA is a rare and intractable chronic disease characterized by a marked increase in peripheral blood eosinophil counts, often occurring against a background of allergic diseases like bronchial asthma. Its pathophysiological characteristics generally include eosinophilic inflammation in systemic organs and granulomatous necrotizing vasculitis in small-to-medium-sized blood vessels. > The eosinophilic inflammation and ANCA-mediated inflammation involving immune cells other than eosinophils contribute to the development of EGPA pathology. The clinical symptoms and progression of the disease may vary depending on genetic background and environmental factors. ANCA-negative patients are genetically closer to asthma and tend to frequently exhibit heart failure. On the other hand, ANCA-positive patients share genetic characteristics with other ANCA-associated vasculitis, and tend to frequently exhibit glomerulonephritis, alveolar hemorrhage, and neuropathy (Furuta et al., 2019). Furthermore, several cytokines are involved in the pathogenesis and disease activity of EGPA (Vaglio et al., 2013;Rodriguez-Pla et al., 2020). > Pharmacotherapy for EGPA includes high-dose glucocorticoids used to induce remission in combination with or without immunosuppressive drugs. With time, the dosage of glucocorticoids is gradually reduced (Chung et al., 2021;Hellmich et al., 2024). Mepolizumab (NUCALA ® ) is an antibody targeting interleukin (IL)-5, a cytokine that regulates eosinophils, which has been approved for the treatment of EGPA.

[8] Cardiac Involvement in Eosinophilic Granulomatosis with Polyangiitis

• Authors: Mukunthan Srikantharajah, Deepa Gopalan, Harold Wilson-Morkeh, Salman Siddiqui, Stephen McAdoo et al.
• Year: 2025
• Venue: Current Cardiology Reports
• URL: https://www.semanticscholar.org/paper/7f032d2a7e0c694c05b8ede36397f9e3ac7b8e5a
• DOI: 10.1007/s11886-025-02258-z
• PMID: 40632386
• PMCID: 12241301
• Citations: 4
• Influential citations: 2
• Summary: A diagnostic approach to facilitate early identification of affected patients with Eosinophilic granulomatosis with polyangiitis is outlined and including CMR in the baseline evaluation of all EGPA patients at diagnosis is recommended.
• Evidence snippets:
• Snippet 1 (score: 0.567) > This review highlights recent advances in the pathophysiology, diagnosis, and treatment of cardiac disease in patients with Eosinophilic granulomatosis with polyangiitis (EGPA). We outline a diagnostic approach to facilitate early identification of affected patients. Recent advancements in diagnostic techniques including cardiac magnetic resonance (CMR) have improved recognition of cardiac disease in patients with EGPA. CMR has demonstrated a high prevalence of cardiac abnormalities, even in the absence of clinical symptoms, electrocardiographic or echocardiographic findings. Cardiac disease is a major cause of mortality in patients with EGPA, accounting for around 50% of disease-related deaths. However, due to the lack of standardised screening and diagnostic criteria, the true incidence remains unknown. Systemic immunosuppressive therapy is warranted to prevent acute complications as well as mitigate the long-term impact of chronic inflammation and tissue damage. Given the challenges in early detection and the prognostic significance of cardiac involvement, we recommend including CMR in the baseline evaluation of all EGPA patients at diagnosis.

[9] Successful Treatment With Mepolizumab for Eosinophilic Granulomatosis With Polyangiitis: A Case Report

• Authors: Andrés Toscano Peña, A. Ali Munive, Y. Arevalo
• Year: 2023
• Venue: Cureus
• URL: https://www.semanticscholar.org/paper/f5b5b3b8bd63a90769b4c6b87f094040e850186d
• DOI: 10.7759/cureus.38797
• PMID: 37303316
• PMCID: 10250110
• Citations: 2
• Summary: These cases describe two patients with EGPA whose initial presentation was severe asthma and who appeared to have extrapulmonary end-organ damage, and Mepolizumab was used in both cases with a successful response.
• Evidence snippets:
• Snippet 1 (score: 0.552) > Eosinophilic granulomatosis with polyangiitis (EGPA) treatment represents a challenge among physicians. Understanding the pathophysiologic mechanisms involved in the development of the disease has allowed alternative treatment options with different mechanisms of action and impact in the inflammatory cascade to emerge. Understanding the role of eosinophils and their mediators has supported the use of Mepolizumab as the only anti-IL5 approved agent for the treatment of EGPA given its efficacy and safety profile in clinical trials. Mortality reduction with minimum adverse effects with Mepolizumab represents significant progress in the treatment of ANCA vasculitis. However, further research is required to define the adequate starting time and the optimal duration of treatment with these drugs.

[10] Targeting the Interleukin-5 Pathway for Treatment of Eosinophilic Conditions Other than Asthma

• Authors: F. Roufosse
• Year: 2018
• Venue: Frontiers in Medicine
• URL: https://www.semanticscholar.org/paper/ec6055289c375f1bb0dfe7f70b82c7590ac000f3
• DOI: 10.3389/fmed.2018.00049
• PMID: 29682504
• PMCID: 5897501
• Citations: 170
• Influential citations: 3
• Summary: Results of clinical trials evaluating the efficacy and safety of these new antibodies in diseases with prominent tissue eosinophilia are reviewed, followed by safety considerations and potential future applications.
• Evidence snippets:
• Snippet 1 (score: 0.550) > Eosinophilic granulomatosis with polyangiitis is a complex disorder combining peripheral blood hypereosinophilia, severe asthma, eosinophil-rich and granulomatous inflammation in lungs and other organs, and small/medium-vessel necrotizing vasculitis (81,82). Classically, disease develops in three consecutive stages: (1) progressive adult-onset asthma, often associated with chronic rhinosinusitis, (2) peripheral blood hypereosinophilia with eosinophilic infiltrates in lungs and possibly other organs, and (3) vasculitis. The disease is heterogeneous, with underlying pathogenic mechanisms that presumably differ in patient subgroups. Patients with positive ANCA serology for example are more likely to develop purpura, glomerulonephritis, pulmonary hemorrhage, and mononeuritis multiplex than ANCA-negative subjects. An operational approach to diagnosis requires presence of asthma, sinusitis and/or rhinitis, pathological confirmation of vasculitis or clinical surrogates highly evocative of vasculitis in at least two organs, and blood eosinophilia above 1.5 G/L. The vasculitic component often responds durably to immunosuppressive drugs such as cyclophosphamide, rituximab, and azathioprine, but the majority of patients remain OCS-dependent because of asthma exacerbations and chronic rhinosinusitis. Methotrexate or azathioprine may be required as maintenance therapy for CS-sparing purposes. > Although it is unclear whether eosinophils contribute direc tly to the vasculitic features of EGPA, they do infiltrate lungs and account for dyspnea in stage 2 disease. At this stage, ANCAnegative EGPA is often indistinguishable from chronic eosinophilic pneumonia (single-organ HES) or idiopathic HES (83,84) (when organs other than lungs are affected as well).

[11] Long-term mepolizumab treatment reduces relapse rates in super-responders with eosinophilic granulomatosis with polyangiitis

• Authors: N. Masumoto, C. Oshikata, Ryo Nakadegawa, Yuto Motobayashi, Reeko Osada et al.
• Year: 2023
• Venue: Allergy, Asthma, and Clinical Immunology : Official Journal of the Canadian Society of Allergy and Clinical Immunology
• URL: https://www.semanticscholar.org/paper/647b75e8f50bfef2ce99595057eaba38349eb568
• DOI: 10.1186/s13223-023-00801-7
• PMID: 37179316
• PMCID: 10182616
• Citations: 7
• Summary: Mepolizumab treatment of super-responders sustainably reduced the relapse rate in patients with eosinophilic granulomatosis with polyangiitis.
• Evidence snippets:
• Snippet 1 (score: 0.538) > Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare immunological condition characterized by allergic granulomatosis and small-and medium-vessel necrotizing vasculitis associated with peripheral blood eosinophilia and tissue infiltration by eosinophils [1,2]. Eosinophils are the most important effector cells and contribute to the disease mechanism [2,3]. Interleukin (IL)-5, which regulates eosinophil proliferation, maturation, and differentiation, is produced by T-helper (Th)2 cells and type 2 innate lymphoid cells (ILC2s) [4,5]. Serum IL-5 or IL-25 levels are more highly correlated with disease activity in patients with active EGPA than in those with asthma [6,7], and in these patients they are significantly higher than in healthy controls [8]. We reported previously that the serum IL-33 concentration and peripheral blood ILC2 count increase in patients with active EGPA at diagnosis and relapse [9]. > The mainstay of treatment for EGPA is systemic corticosteroid therapy; some patients receive additional treatment with other immunosuppressive agents, such as cyclophosphamide, azathioprine, methotrexate, interferon-α [10,11], rituximab [12][13][14], intravenous immunoglobulins (IVIGs) [15][16][17], and other biologics [18,19]. Biologics include omalizumab [20,21], mepolizumab [21][22][23][24][25][26][27][28], benralizumab [29] dupilumab [30], reslizumab, and lebrikizumab; the latter three are being tested in ongoing clinical trials [31].

[12] Radioclinical Profile of Eosinophilic Lung: A Case Series

• Authors: Abir Bouhamdi, Fatima Saddouki, Badreddine Alami, Mounia Serraj, Mohamed Biaz et al.
• Year: 2024
• Venue: Cureus
• URL: https://www.semanticscholar.org/paper/9b54d6500ded3b73647a61a39c82d9425ea6bc90
• DOI: 10.7759/cureus.62579
• PMID: 39036186
• PMCID: 11258676
• Summary: Findings from an analysis of 17 patients diagnosed with eosinophilic lung disease are presented, with a majority (64.70%) being male and the main etiologies identified were chronic eosinophilic pneumonia, followed by eosinophilic granulomatosis with polyangiitis and drug-induced hypereosinophilia.
• Evidence snippets:
• Snippet 1 (score: 0.533) > Eosinophilic lung diseases are a complex group of diffuse parenchymal lung diseases characterized by eosinophilic infiltration of lung tissue [1].Although rare, these conditions are of significant clinical importance due to their diagnostic and therapeutic implications.Eosinophils, immune cells involved in allergic and inflammatory responses, are often found in excessive quantities in lung tissue, associated with alveolar eosinophilia exceeding 25%.However, blood eosinophilia can vary, sometimes being absent or temporary [2]. > These conditions share similarities in pathophysiology, radiology, and response to corticosteroid treatment, but they differ considerably in terms of etiology, clinical presentation, and disease progression.The underlying mechanisms involved in these pathologies often remain complex and poorly understood, making their diagnosis and clinical management a challenge for practitioners. > Eosinophilic lung diseases can be classified into primary and secondary categories [1].Primary eosinophilic lung diseases originate directly from eosinophilic infiltration of the lung tissue, often without a clear underlying cause.Examples include eosinophilic pneumonia and eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome).Secondary eosinophilic lung diseases arise due to underlying conditions or triggers such as parasitic infections, allergic reactions, certain medications, or other systemic diseases.These include conditions like allergic bronchopulmonary aspergillosis and hypereosinophilic syndrome.Primary forms typically have a more direct association with eosinophilic infiltration, while secondary forms are often linked to external factors or underlying diseases. > Our research aims to thoroughly investigate the epidemiological, clinical, biological, and radiological characteristics of patients with eosinophilic lung disease.By emphasizing the varied and often insidious clinical presentations of these conditions, as well as the diagnostic challenges faced by clinicians, our study aspires to contribute to improving the management of patients with eosinophilic lung disease.By providing relevant data on the different subtypes of this pathology and analyzing responses to available treatments, we aim to contribute to a better understanding of these conditions and guide therapeutic decisions in daily clinical practice.

[13] Clinical perspective on serum periostin in antineutrophil-cytoplasmic antibody-associated vasculitis

• Authors: T. Yoon, Jiyeol Yoon, Eunhee Ko, Yong-Beom Park, Sang-Won Lee
• Year: 2025
• Venue: The Korean Journal of Internal Medicine
• URL: https://www.semanticscholar.org/paper/dc41ecb53df461e6dcbd9549dc177391b5d3938d
• DOI: 10.3904/kjim.2024.254
• PMID: 40360226
• PMCID: 12081101
• Summary: This study is the first to demonstrate that serum periostin at diagnosis could independently reflect cross-sectional BVAS and further partially contribute to all-cause mortality prediction in patients with AAV.
• Evidence snippets:
• Snippet 1 (score: 0.531) > Periostin is an extracellular matrix protein that belongs to the Fasciclin family because of its homology to Fasciclin 1. Periostin is produced in mesenchymal lineage cells such as osteoblasts, periodontal ligament, and periosteum, and it is secreted into the extracellular space [1]. Periostin plays a role in forming and maintaining cellular structures by interacting with structural matrix proteins, including collagen [2]. Conversely, as a matricellular protein, it may also play other roles in regulating and modulating cellular functions through cross-talk with cell-surface receptors, proteases, and hormones [3,4]. The clinical utility of serum periostin was first reported in allergic diseases because of the induction of periostin gene expression by interleukin (IL)-4 and IL-13 [2,5]. Subsequently, many studies have reported the utility of periostin as a serum biomarker and therapeutic target in various chronic inflammatory and fibrotic diseases, such as liver and lung diseases [6]. In particular, periostin is involved in signalling pathways via nuclear factor kappalight-chain-enhancer of activated B cells, IL-8, extracellular signal-regulated kinase, and mitogen-activated protein kinase in the pathophysiology of chronic kidney diseases and inflammatory bowel disease [7,8]. The possibility of the clinical utility of periostin in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) with a similar pathological mechanism has been proposed [9,10]. To date, only one study that included patients with eosinophilic granulomatosis with polyangiitis (EGPA) has reported the clinical utility of serum periostin in AAV [11]. However, since the pathogenesis of EGPA is also partially based on a well-known allergic immunological mechanism, the previous study could not represent all cases of AAV with vasculitic immunological mechanisms and thus; may not provide new and additional information on pre-existing concepts [12].

[14] Molecular Pathogenesis and Targeted Therapies in Eosinophilic Granulomatosis with Polyangiitis: An Updated Review

• Authors: M. López Paraja, Grisell Starita Fajardo, Ignacio Donate Velasco, D. Lucena López, María Pilar Iranzo Alcolea et al.
• Year: 2025
• Venue: International Journal of Molecular Sciences
• URL: https://www.semanticscholar.org/paper/04a24ba69a713d052001e8020b0192cb198e84b0
• DOI: 10.3390/ijms262211141
• PMID: 41303621
• PMCID: 12652875
• Summary: An updated review of EGPA integrates molecular insights, clinical endotypes, and therapeutic innovations to outline current evidence and future precision-medicine strategies aimed at improving long-term patient outcomes.
• Evidence snippets:
• Snippet 1 (score: 0.530) > This review was conducted as a structured narrative synthesis in accordance with current recommendations for evidence-based clinical reviews. A comprehensive literature search was performed in PubMed, Scopus, and Web of Science covering the period from January 2015 to April 2025, using combinations of the following keywords: "eosinophilic granulomatosis with polyangiitis," "Churg-Strauss syndrome," "ANCA-associated vasculitis," "biomarkers," "genetics," "treatment," and "clinical phenotypes." Additional references were identified through manual screening of bibliographies and recent consensus statements. > Inclusion criteria comprised peer-reviewed human studies, meta-analyses, clinical trials, cohort studies, and major reviews addressing the pathogenesis, clinical features, biomarkers, or management of EGPA. Exclusion criteria included non-English publications, isolated case reports, and animal or in vitro studies not directly related to disease mechanisms. > The available evidence was qualitatively appraised according to study design and level of evidence, prioritizing randomized controlled trials, population-based cohorts, and expert consensus. Where applicable, international guidelines (EULAR, ACR, and EUVAS) were referenced to contextualize therapeutic recommendations. > This review integrates genetic, immunopathogenic, and clinical perspectives to provide an updated synthesis of the current understanding and emerging therapeutic approaches in EGPA.

[15] Novel Targets for Drug Use in Eosinophilic Granulomatosis With Polyangiitis

• Authors: M. Uzzo, F. Regola, B. Trezzi, P. Toniati, F. Franceschini et al.
• Year: 2021
• Venue: Frontiers in Medicine
• URL: https://www.semanticscholar.org/paper/e5e6ac5470ea91a44d9ff29104915f9a92b43c5b
• DOI: 10.3389/fmed.2021.754434
• PMID: 34796188
• PMCID: 8593004
• Citations: 14
• Influential citations: 1
• Summary: A review of the emerging therapies used in EGPA, focusing on the most recent studies on biologics and analyzing their efficacy and safety, finds that new insights into EGPA pathogenesis allow the discovery of new targets.
• Evidence snippets:
• Snippet 1 (score: 0.526) > As classified by the 2012-revised Chapel Hill consensus conference, Eosinophilic Granulomatosis with Polyangiitis (EGPA, formerly Churg Strauss syndrome) is a rare systemic necrotizing vasculitis of small and medium size vessels, characterized by asthma and blood and tissue eosinophilia; among all kind of vasculitis, EGPA can have an impressive heart involvement (1). It is a rare disease with a prevalence ranging between 7.3 and 17.8 per million and an annual incidence of 0.9-2.4 per million (2)(3)(4). > EGPA belongs to the anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis group (AAV), including granulomatosis with polyangiitis (GPA), and microscopic polyangiitis (MPA). However, only 30-40% of patients are ANCA positive and ∼70% with myeloperoxidase (MPO) specificity: the ANCA positive disease is characterized by vasculitic features and shares pathogenetic mechanisms with the other AAV; the ANCA negative disease seems to share the pathogenetic mechanisms observed in eosinophilic syndromes instead (5). A recent genome-wide association study confirmed the existence of those two phenotypes from a genetical point of view: the former is linked with HLA-DQ, the latter with genes involved in mucosal responses (6). The natural history of the disease involves three steps: asthma or allergy related symptoms, followed by eosinophilia and lung infiltrates and finally, after a mean of 9.3 years, vasculitic manifestations (7).

[16] Genetic and Non-Genetic Contributions to Eosinophilic Granulomatosis with Polyangiitis: Current Knowledge and Future Perspectives

• Authors: M. Treccani, L. Veschetti, C. Patuzzo, Giovanni Malerba, Augusto Vaglio et al.
• Year: 2024
• Venue: Current Issues in Molecular Biology
• URL: https://www.semanticscholar.org/paper/806ca0698298207cdab806ef6afe874dc24b95aa
• DOI: 10.3390/cimb46070446
• PMID: 39057087
• PMCID: 11275403
• Citations: 5
• Summary: A comprehensive overview of the genetic and non-genetic complexity of eosinophilic granulomatosis with polyangiitis (EGPA), a rare complex systemic disease that occurs in people presenting with severe asthma and high eosinophilia.
• Evidence snippets:
• Snippet 1 (score: 0.526) > In this work, we present a comprehensive overview of the genetic and non-genetic complexity of eosinophilic granulomatosis with polyangiitis (EGPA). EGPA is a rare complex systemic disease that occurs in people presenting with severe asthma and high eosinophilia. After briefly introducing EGPA and its relationship with the anti-neutrophil cytoplasmic autoantibodies (ANCA)-associated vasculitis (AAVs), we delve into the complexity of this disease. At first, the two main biological actors, ANCA and eosinophils, are presented. Biological and clinical phenotypes related to ANCA positivity or negativity are explained, as well as the role of eosinophils and their pathological subtypes, pointing out their intricate relations with EGPA. Then, the genetics of EGPA are described, providing an overview of the research effort to unravel them. Candidate gene studies have investigated biologically relevant candidate genes; the more recent genome-wide association studies and meta-analyses, able to analyze the whole genome, have confirmed previous associations and discovered novel risk loci; in the end, family-based studies have dissected the contribution of rare variants and the heritability of EGPA. Then, we briefly present the environmental contribution to EGPA, reporting seasonal events and pollutants as triggering factors. In the end, the latest omic research is discussed and the most recent epigenomic, transcriptomic and microbiome studies are presented, highlighting the current challenges, open questions and suggesting approaches to unraveling this complex disease.

[17] Antineutrophil cytoplasmic antibody-associated vasculitis, update on molecular pathogenesis, diagnosis, and treatment

• Authors: Farid Arman, Marina Barsoum, U. Selamet, Hania Shakeri, O. Wassef et al.
• Year: 2018
• Venue: International Journal of Nephrology and Renovascular Disease
• URL: https://www.semanticscholar.org/paper/88cc5eb3c0db8495256aa171fc3ae3231af98f78
• DOI: 10.2147/IJNRD.S162071
• PMID: 30538527
• PMCID: 6255047
• Citations: 13
• Influential citations: 2
• Summary: Treatment updates are provided with regard to new studies and the now standard use of anti-CD-20 monoclonal antibodies as first-line therapy in all but the most aggressive presentations of this disease.
• Evidence snippets:
• Snippet 1 (score: 0.525) > Circulating antineutrophil cytoplasmic antibodies (ANCAs) are the central pathogenic mechanism for a group of systemic and renal syndromes called the ANCA-associated vasculitis (AAV). The nomenclature has changed from eponymous labeling to granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis. These syndromes predominantly affect the pulmonary and renal systems. We also review the molecular pathology behind ANCAs and associated antigens and infections. Various clinical presentations, the multiple target organs affected, and diagnostic challenges involved in identifying these diseases are discussed. Treatment updates are also provided with regard to new studies and the now standard use of anti-CD-20 monoclonal antibodies as first-line therapy in all but the most aggressive presentations of this disease. Maintenance regimens and monitoring strategies for relapse of vasculitis and associated systemic complications are discussed.

[18] Eosinophilic Granulomatosis with Polyangiitis: An Overview

• Authors: A. Gioffredi, F. Maritati, E. Oliva, C. Buzio
• Year: 2014
• Venue: Frontiers in Immunology
• URL: https://www.semanticscholar.org/paper/1c069da28c301b26344c82ee3bbaa27170d7ad21
• DOI: 10.3389/fimmu.2014.00549
• PMID: 25404930
• PMCID: 4217511
• Citations: 173
• Influential citations: 7
• Summary: Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic disorder, belonging to the small vessel anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, defined as an eOSinophil-rich and necrotizing granulOMatous inflammation often involving the respiratory tract, and Necrotizing vasculopathy predominantly affecting small to medium-sized vessels.
• Evidence snippets:
• Snippet 1 (score: 0.524) > Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic disorder, belonging to the small vessel anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, defined as an eosinophil-rich and necrotizing granulomatous inflammation often involving the respiratory tract, and necrotizing vasculitis predominantly affecting small to medium-sized vessels, associated with asthma and eosinophilia. EGPA pathogenesis is not well known: HLA-DRB104 and 07, HLA-DRB4 and IL10.2 haplotype of the IL-10 promoter gene are the most studied genetic determinants. Among the acquired pathogenetic factors, the exposure to different allergens, infections, vaccinations, drugs, and silica exposure have been involved. Eosinophils are the most characteristic cells in EGPA and different studies have demonstrated their role as effector and immunoregulatory cells. EGPA is considered as a disease with a prevalent activation of the Th-2 cellular-mediated inflammatory response and also humoral immunity plays an important role. A link between B and T inflammatory responses may explain different disease features. EGPA typically develops into three sequential phases: the allergic phase, distinguished by the occurrence of asthma, allergic rhinitis, and sinusitis, the eosinophilic phase, in which the main pathological finding is the eosinophilic organ infiltrations (e.g., lungs, heart, and gastrointestinal system), and the vasculitic phase, characterized by purpura, peripheral neuropathy, and constitutional symptoms. ANCA (especially pANCA anti-myeloperoxidase) are present in 40–60% of the patients. An elevation of IgG4 is frequently found. Corticosteroids and cyclophosphamide are classically used for remission induction, while azathioprine and methotrexate are the therapeutic options for remission maintenance. B-cell depletion with rituximab has shown promising results for remission induction.

Notes

• This provider combines search_papers_by_relevance with snippet_search.
• No synthesis or second-stage model call is performed.