A granulomatose eosinofílica com poliangiite (EGPA), anteriormente conhecida como síndrome de Churg-Strauss, é uma vasculite sistêmica de vasos de pequeno a médio porte, caracterizada por asma, infiltrados pulmonares transitórios e hipereosinofilia.
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A granulomatose eosinofílica com poliangiite (EGPA), anteriormente conhecida como síndrome de Churg-Strauss, é uma vasculite sistêmica de vasos de pequeno a médio porte, caracterizada por asma, infiltrados pulmonares transitórios e hipereosinofilia.
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Mostrando amostra de 200 publicações de um total de 1.781
[Successful early use of mepolizumab in eosinophilic granulomatosis with polyangiitis presenting with peripheral neuropathy and gastrointestinal bleeding].
We report a case of a 55-year-old male with eosinophilic granulomatosis with polyangiitis (EGPA) presenting with peripheral neuropathy and gastrointestinal (GI) bleeding. Despite initial treatment with high-dose intravenous methylprednisolone and intravenous immunoglobulin, the patient's symptoms persisted, and GI bleeding worsened. Capsule endoscopy revealed multiple ulcers and erosions throughout the small intestine, and biopsies from the ileum, cecum, and skin showed marked eosinophilic infiltration. Although peripheral blood eosinophil counts were normalized, tissue eosinophilia remained evident. Early administration of mepolizumab, an anti-IL-5 monoclonal antibody, led to rapid improvement in both GI bleeding and neuropathic symptoms. This case highlights the potential role of tissue-infiltrating eosinophils in EGPA pathogenesis and suggests that mepolizumab may be effective even when blood eosinophil levels are not elevated. Early intervention with mepolizumab may be beneficial in severe or refractory EGPA cases with GI involvement, which are associated with poor prognosis and high relapse rates. Careful monitoring and timely therapeutic decisions are essential for improving outcomes in such patients.
Clinical and Pathological Features of Vasculitic Neuropathy: A Single-Center Study in China.
Vasculitic neuropathy (VN) is a disease in which vessel inflammation happens and injures peripheral nerves. Despite increasing awareness, features of VN in mainland China are still understudied. To characterize the clinical, pathological features and outcomes of VN in mainland China, and evaluate clinicopathological correlations: METHODS: We retrospectively reviewed records of VN patients diagnosed pathologically between June 1999 and December 2024, including demographic data, clinical manifestations, biopsy features, and outcomes. 112 patients were totally included. All presented with axonal sensorimotor neuropathy, most commonly involving the tibial (90.63%) and peroneal (87.50%) nerves. Systemic VN (SVN) showed more frequent transmural inflammatory cell infiltration (ICI) than non-systemic VN (NSVN) (p = 0.046). Eosinophilic granulomatosis with polyangiitis (EGPA) was more common in VN without ICI than VN with ICI (p = 0.008). In SVN, the ICI-positive exhibited more severe distal upper limb weakness (p = 0.042) and higher thrombosis rates (p = 0.001) than ICI-negative. Of the 63 patients followed, 62 received glucocorticoids with or without immunosuppressants. Thirteen died from multi-organ complications (12 SVN and 1 NSVN), while others achieved remission. The 5-year all-survival rate was 80.23% (95% CI 66.83%- 88.66%). This first large cohort of VN in mainland China delineates its clinical-pathological features. EGPA showed a lower diagnostic yield on biopsy, suggesting diverse mechanisms of vascular injury. Nerve biopsy remains the diagnostic gold standard. The overall prognosis of VN is relatively favorable, emphasizing the need for early recognition and treatment.
Leukotriene receptor antagonists and eosinophilic granulomatosis with polyangiitis: a disproportionality analysis from FAERS, JADER, CVAR databases integrated with network pharmacology.
The relationship between leukotriene receptor antagonists (LTRAs) usage and the subsequent occurrence of eosinophilic granulomatosis with polyangiitis (EGPA) remained highly polarizing and controversial in previous studies. We aimed to investigate the risk of EGPA caused by LTRAs and the potential toxicological mechanisms of LTRAs-related EGPA. In this real-world pharmacovigilance study, we collected adverse event (AE) reports of EGPA associated with LTRAs use from the U.S. FDA Adverse Event Reporting System (FAERS), Japanese Adverse Drug Event Reporting (JADER), and Canadian Vigilance Adverse Reaction (CVAR) databases. The reporting odds ratio (ROR), proportional reporting ratio (PRR), information component (IC), and empirical Bayesian geometric mean (EBGM) were calculated to quantify the strength of the association between LTRAs and EGPA. The Weibull shape parameter (WSP) test was applied to analyze time-to-onset profiles of EGPA toxicity. Network pharmacology analysis was subsequently performed to identify the central genes to determine the potential mechanisms underlying LTRAs-induced EGPA. LTRAs, including montelukast, zafirlukast, and pranlukast, exhibited a strong association with EGPA in three databases (the lower limit of 95% confidence interval (CI) for ROR > 1, PRR > 2 with χ2 values ≥4, EBGM05 > 2, and IC025 > 0). After excluding corticosteroids as concomitant medication, montelukast remained significantly associated with EGPA in the FAERS database. The median time-to-onset of EGPA associated with LTRAs was 233 (range: 76-660) days, and the WSP test indicated LTRAs had early failure-type profiles. We isolated 81 interactive target genes linking LTRAs to EGPA. Several central genes, including SRC, PTGS2, EDN1, HMOX1, KDR, and OCLN, were revealed via protein-protein interactions analysis and molecular complex detection (MCODE) algorithm. Our study revealed LTRAs could increase the risk of EGPA, and initially explored potential genes and mechanisms of LTRAs-induced EGPA. It is helpful for clinicians to be alerted to the risk of EGPA during LTRAs administration.
ANCA-associated vasculitis following sotatercept initiation in a patient with heritable pulmonary arterial hypertension and previously silent eosinophilic granulomatosis with polyangiitis: a case report.
Pulmonary arterial hypertension (PAH) is a progressive disease characterized by increased pulmonary vascular resistance and right ventricular failure. Sotatercept, a novel activin receptor ligand trap, has demonstrated promising haemodynamic benefits in PAH treatment. Eosinophilic granulomatosis with polyangiitis (EGPA) is an extremely rare, Th2-driven, small-vessel vasculitis, and its overlap with PAH is scarcely reported. We report the case of a woman with heritable PAH initially stabilized with oral therapy. In 2024, after further clinical decline, subcutaneous treprostinil was initiated. Subsequently, sotatercept was added, resulting in brief clinical improvement. Within weeks, however, the patient developed severe eosinophilia and exhibited laboratory and histopathological evidence of p-ANCA-positive necrotizing vasculitis, accompanied by renal and hepatocellular dysfunction. This case suggests that sotatercept's modulation of the TGF-β pathway may unmask latent autoimmune diseases such as EGPA in predisposed individuals. Although the temporal relationship between sotatercept initiation and the onset of vasculitis is compelling, both causality and underlying molecular mechanisms remain to be elucidated. Further studies are necessary to understand the potential immunomodulatory mechanisms of sotatercept.
Natural History of Patients With Histologically Proven Acute Eosinophilic Myocarditis.
No large registries of patients with acute eosinophilic myocarditis (EM) are available. However, EM is perceived as a cardiac disease with high mortality, affecting mainly young and middle-aged adults according to small series and case reports. Awareness of the clinical presentation, associated systemic conditions, treatments, and outcomes of this uncommon condition is an unmet need. In this international, multicenter, retrospective cohort study, 53 centers screened 193 patients with histologically proven acute EM between 1992 and 2023. After the exclusion of patients with insufficient data (n=10), symptoms lasting >30 days (n=19), or histological diagnosis not confirmed after review (n=8), 156 patients were included. Median age at presentation was 48 years (first to third quartile, 34-59 years) with male predominance (67.3%), and only 2 were pediatric cases (≤16 years of age; 1.3%). The main signs and symptoms at presentation were dyspnea (75.6%), fever (61.3%), and chest pain (53.2%). Unexpectedly, peripheral eosinophilia was reported in only 57.4% of cases, with a median cell count of 630 eosinophils/μL. The median left ventricular ejection fraction at presentation was 32% (first to third quartile, 25%-48%). The disorders most frequently associated with EM were eosinophilic granulomatosis with polyangiitis (22.4% of cases) and hypersensitivity forms (14.1%). Idiopathic/undefined forms accounted for 44.9% of cases, and miscellaneous causes accounted for 18.6%. In-hospital death or need for heart transplantation (HTx) occurred in 23 patients (14.7%; 22 deaths and 1 HTx), despite 43.6% being treated with temporary mechanical circulatory support and 92.9% being treated with immunosuppressive agents. Estimated rates of death or HTx at 1 and 3 years were 19.0% and 23.8%. Increased age, decreased left ventricular ejection fraction on admission, and no immunosuppressive therapy during hospitalization were independent predictors of death or HTx. A nonsignificant higher occurrence of deaths or HTx was observed in the hypersensitivity form (46.1%) compared with the eosinophilic granulomatosis with polyangiitis-associated form (13.1%) at 3 years (P=0.15). Acute EM can often present without peripheral eosinophilia, and rates of in-hospital and midterm mortality or HTx are high. Endomyocardial biopsy is required to reach the final diagnosis of EM because relying on peripheral eosinophilia can lead to missing diagnosis. In-hospital immunosuppression is associated with HTx-free survival, although tailored immunosuppressive therapies are needed to improve outcomes. URL: https://www.clinicaltrials.gov; Unique identifier: NCT06447935.
Publicações recentes
Antineutrophil Cytoplasmic Antibody-Negative Eosinophilic Granulomatosis With Polyangiitis in a Pediatric Patient.
Latent Class Analysis to Explore Subtypes of EGPA: Focusing on Respiratory Involvement and Inflammation Markers.
🥇 Ensaio randomizado[DIAGNOSIS OF ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-NEGATIVE EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS BASED ON RENAL PATHOLOGICAL FINDINGS WITHOUT ASTHMA].
Ischemic stroke in eosinophilic granulomatosis with polyangiitis: a single-center retrospective cohort study.
Successful treatment of eosinophilic granulomatosis with polyangiitis and Crohn's disease with combined mepolizumab and adalimumab.
📚 EuropePMC1.091 artigos no totalmostrando 197
Benralizumab: An effective alternative in patients with eosinophilic granulomatosis with polyangiitis refractory to mepolizumab.
Medicina clinicaEosinophilic granulomatosis with polyangiitis coexisting with multiple myeloma: independent entities or coexistence? A case report.
Frontiers in immunologyBeyond Classification: An Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Overlap Case.
Cureus[Successful early use of mepolizumab in eosinophilic granulomatosis with polyangiitis presenting with peripheral neuropathy and gastrointestinal bleeding].
Rinsho shinkeigaku = Clinical neurologyClinical and Pathological Features of Vasculitic Neuropathy: A Single-Center Study in China.
European journal of neurologyEarly benralizumab use for the successful treatment of eosinophilic granulomatosis with polyangiitis-associated peripheral neuropathy: A case report.
Internal medicine (Tokyo, Japan)Benralizumab reduces sputum ANCA in patients with eosinophilic granulomatosis with polyangiitis.
ERJ open researchEpidemiology of eosinophilic granulomatosis with polyangiitis in northern Spain: A population-based study (2000-2024) and literature review.
Autoimmunity reviewsLeukotriene receptor antagonists and eosinophilic granulomatosis with polyangiitis: a disproportionality analysis from FAERS, JADER, CVAR databases integrated with network pharmacology.
PloS oneSafety of Immune Checkpoint Inhibitors in Cancer Patients With Preexisting Autoimmune Vasculitis.
ACR open rheumatologyHepatic Lesions With Eosinophilic Granulomatosis With Polyangiitis.
International journal of rheumatic diseasesDifferential Diagnosis of Eosinophilic Lung Diseases.
The journal of allergy and clinical immunology. In practiceA Case of Eosinophilic Granulomatosis with Polyangiitis Emerging During Benralizumab Therapy: Successful Management Through a Switch to Mepolizumab Therapy.
Journal of asthma and allergyBurden of eosinophilic granulomatosis with polyangiitis by disease phase and steroid-sparing effects of biologics: a real-world retrospective study in Europe.
ERJ open researchFractional Exhaled Nitric Oxide as a Novel Biomarker of Relapse in Eosinophilic Granulomatosis with Polyangiitis.
American journal of respiratory and critical care medicineImproved long-term prognosis of eosinophilic granulomatosis with polyangiitis: retrospective analysis of 87 patients after biologic therapy introduction in Japan.
Scientific reportsEosinophilic granulomatosis with polyangiitis: recent therapeutic advances.
Current opinion in rheumatologyEffectiveness of Mepolizumab in Severe Uncontrolled Asthma Associated or Not with EGPA Based on the Exacto Scale and Separ-Remas Criteria.
Journal of asthma and allergyRole of biological therapies targeting eosinophils in eosinophilic granulomatosis with polyangiitis: current evidence and future perspectives.
Current medical research and opinionEosinophilic Granulomatosis with Polyangiitis Presenting with Intermittent Claudication.
JMA journal[RECONSIDERATION OF EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (EGPA)].
Arerugi = [Allergy]ANCA-associated vasculitis following sotatercept initiation in a patient with heritable pulmonary arterial hypertension and previously silent eosinophilic granulomatosis with polyangiitis: a case report.
European heart journal. Case reportsCase Report: Angioimmunoblastic T-cell lymphoma initially diagnosed as eosinophilic granulomatosis with polyangiitis.
Frontiers in medicinePrediction of Relapse and Glucocorticoid Dependence in Eosinophilic Granulomatosis With Polyangiitis: Findings From a Large European Cohort.
Arthritis & rheumatology (Hoboken, N.J.)Novel Diagnostic Approaches for Eosinophilic Lung Diseases.
The journal of allergy and clinical immunology. In practiceNatural History of Patients With Histologically Proven Acute Eosinophilic Myocarditis.
CirculationCatastrophic antiphospholipid syndrome with eosinophilia mimicking hypereosinophilic syndromes with disseminated intravascular coagulation: a case report.
Journal of medical case reportsFatal Eosinophilic Pulmonary Vasculitis in a Neonate - The First Documented Case in the English Literature.
Annals of clinical and laboratory scienceNS-229, a novel Janus kinase 1 inhibitor, ameliorates eosinophilic vasculitis in an ovalbumin-induced mouse model by modulating multiple cytokine signaling pathways.
The Journal of pharmacology and experimental therapeuticsANCA-negative EGPA with migratory pulmonary masses: A case report of mepolizumab response and literature review.
MedicineValidation of the 2022 ACR/EULAR classification criteria for eosinophilic granulomatosis with polyangiitis in a Chinese cohort.
Clinical rheumatologyEGPA presenting as sudden cardiac arrest: a case report and review of cardiac manifestations.
Frontiers in immunologyCase Report: Triple autoimmune overlap: rheumatoid arthritis, systemic lupus erythematosus, and hypereosinophilic asthma with systemic manifestations.
Frontiers in immunologyImmature leukocyte and plasma-induced cell death reveal subclinical immune activation in EGPA patients in remission.
Inflammation research : official journal of the European Histamine Research Society ... [et al.]Treatment of Eosinophilic Granulomatosis With Polyangiitis (EGPA): Do We Need Immunosuppressives?
The journal of allergy and clinical immunology. In practiceEffectiveness, Tolerability, and Safety of Mepolizumab Injection Spacing in Patients With Eosinophilic Granulomatosis With Polyangiitis: A Retrospective Cohort Study.
Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseasesEosinophilic granulomatosis with polyangiitis related giant coronary aneurysm leading to acute myocardial infarction: a case report.
European heart journal. Case reportsHepatic granulomas heralding eosinophilic granulomatosis with polyangiitis overlapping with Sjögren's syndrome.
Hepatology forumA Red Eye in an Elderly Patient: A Case Report Highlighting the Importance of Urgent Diagnosis.
CureusIntracerebral Hemorrhage During the Chronic Phase of Eosinophilic Granulomatosis With Polyangiitis: A Case Report Emphasizing Cerebrovascular Vulnerability.
Clinical case reportsTargeting Immunologic Pathways in Eosinophilic Granulomatosis With Polyangiitis: Translating Emerging Evidence Into Clinical Practice.
AllergyANCA-associated vasculitis in the United Arab Emirates: a 17-year case series with review from Gulf literature.
Annals of Saudi medicineMore Than Five Years of Sustained Remission With Mepolizumab in Adolescent-Onset Eosinophilic Granulomatosis With Polyangiitis: A Case Report.
CureusPlacoid Lesions in Eosinophilic Granulomatosis with Polyangiitis.
OphthalmologyDiaphragmatic paralysis in eosinophilic granulomatosis with polyangiitis.
Medicina clinicaEosinophilic Granulomatosis with Polyangiitis Complicated by Eosinophilic Chronic Rhinosinusitis Successfully Treated with Combination of Mepolizumab and Dupilumab: A Case Report.
Internal medicine (Tokyo, Japan)Effectiveness of low-dose mepolizumab in refractory eosinophilic granulomatosis with polyangiitis: systemic steroid use and remission.
The Korean journal of internal medicineUnmasking EGPA with near fatal diffuse alveolar haemorrhage in severe eosinophilic asthma under treatment with benralizumab: a case report.
BMC pulmonary medicineAirway immune profiles and therapeutic implications of IGF1 in eosinophilic granulomatosis with polyangiitis.
Nature communicationsEosinophilic Granulomatosis with Polyangiitis Presenting as Acute Abdomen: A Rare Case Report.
Journal of inflammation researchEvaluation and differential diagnosis of eosinophilia: A tertiary allergy center experience.
Tuberkuloz ve toraksEosinophilic Granulomatosis With Polyangiitis Presenting as Mononeuritis Multiplex in a Diabetic Patient: Diagnostic and Therapeutic Challenges.
CureusEosinophilic Granulomatosis With Polyangiitis Presenting as a Mononeuritis Multiplex Mimicking Guillain-Barré Syndrome.
CureusAnalyses of Final Trends and Comorbidity of Eosinophilic Granulomatosis with Polyangiitis in Patients Hospitalized in Poland in 2014-2023.
Journal of clinical medicineBenralizumab: Bringing winds of change to eosinophil-associated diseases.
The World Allergy Organization journalANCA-Negative Eosinophilic Granulomatosis With Polyangiitis Presenting With Duodenal Stenosis: A Case Report.
The American journal of case reportsEosinophilic granulomatosis with polyangiitis diagnosed during tezepelumab treatment for severe asthma: A case report.
Allergology international : official journal of the Japanese Society of AllergologyAnti-Programmed Death-Ligand 1 Antibody-Associated Eosinophilic Granulomatosis with Polyangiitis in Non-Small Cell Lung Cancer: A Case Report.
Internal medicine (Tokyo, Japan)Granulomatosis With Polyangiitis (Wegener Granulomatosis): Then and Now.
Archives of pathology & laboratory medicineUpdated treatment approaches for eosinophilic granulomatosis with polyangiitis: A systematic scoping review.
Modern rheumatologyOverview of Biologics Targeting Type 2 Inflammation in Respiratory Disease.
The journal of allergy and clinical immunology. In practiceInside GEMA 5.5: Expert Insights on the Latest Changes in Asthma Management.
Archivos de bronconeumologiaVenous Thromboembolism May Be Increased in the Early Phases of ANCA-Associated Vasculitis and Could Be Associated With a High Body Mass Index: TR-VaS Experience.
International journal of rheumatic diseasesEosinophilic Granulomatosis With Polyangiitis Presenting With Syphilitic-Like Palmar Erythema.
International journal of rheumatic diseasesNanomedicine-driven microRNA therapy: a precision approach for eosinophilic granulomatosis with polyangiitis.
Annals of medicine and surgery (2012)Bilateral Ischaemic Stroke in Eosinophilic Granulomatosis With Polyangiitis.
CureusOverlapping forms of granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis: Insights from a European multicenter study.
Journal of internal medicineA case report of eosinophilic granulomatosis with polyangiitis in children with cerebella infarction as the first symptom and literature review.
Frontiers in immunologyEffective Use of Mepolizumab in a Rare Case of Eosinophilic Granulomatosis With Polyangiitis Complicated by Pulmonary Tuberculosis.
CureusAngiolymphoid Hyperplasia With Eosinophilia Vs Kimura Disease: Continuum of the Same Disease or a Distinct Entity-A Case Report With Literature Review.
Case reports in medicineDual pathogenesis and treatment approaches for eosinophilic granulomatosis with polyangiitis: a comprehensive review.
ReumatologiaRESER/NVAN: Spanish Registry of patients with ANCA- associated vasculitis: objectives and methodology.
Reumatologia clinicaAdjunctive dual biologic therapy for persistent nasal symptoms in eosinophilic granulomatosis with polyangiitis (EGPA).
American journal of otolaryngologyAcute Eosinophilic Myocarditis and Heart Failure As the First Manifestation of Eosinophilic Granulomatosis With Polyangiitis (EGPA) in an Asthmatic Patient: A Diagnostic Challenge.
CureusGlucocorticoid-free remission with simultaneous mepolizumab and rituximab in life-threatening PR3-ANCA-positive eosinophilic granulomatosis with polyangiitis: a case report and literature review.
Clinical rheumatologyEosinophilic granulomatosis with polyangiitis mimicking giant cell arteritis.
Canadian journal of ophthalmology. Journal canadien d'ophtalmologiePrediction model to identify patients with hypereosinophilic syndrome using real-world data.
The journal of allergy and clinical immunology. GlobalHypereosinophilic syndrome with pulmonary and hepatic involvement mimicking autoimmune disease.
Oxford medical case reportsMolecular Pathogenesis and Targeted Therapies in Eosinophilic Granulomatosis with Polyangiitis: An Updated Review.
International journal of molecular sciencesAbdominal pain as the sole initial manifestation of ANCA-positive EGPA with myocarditis: a case report and literature review.
BMC cardiovascular disordersHow to choose a biologic agent considering comorbidities of bronchial asthma.
Respiratory investigationANCA-negative Pauci-immune Vasculitis, an Often-missed Clinical Entity: A Case Report.
The Journal of the Association of Physicians of IndiaEarly Diagnosis and Treatment in Patients With Eosinophilic Granulomatosis With Polyangiitis.
The journal of allergy and clinical immunology. In practiceDistinct predictors of peripheral neuropathy in antineutrophil cytoplasmic antibody-associated vasculitis: KEIO-vasculitis cohort.
Seminars in arthritis and rheumatismCoronary vasospasms and other cardiac manifestations in Eosinophilic Granulomatosis with Polyangiitis: Clinical impact and frequency in a monocentre study of 103 patients.
RMD openEpidemiology of anti-neutrophil cytoplasmic antibody-associated vasculitis in Lithuania, 2012-2021: a retrospective cohort study.
BMJ openEstimation of Health Utility Values for Eosinophilic Granulomatosis With Polyangiitis.
PharmacoEconomics - openClinical, Biomarker, and Radiological Progression from Asthma to Systemic Eosinophilic Granulomatosis with Polyangiitis: A Retrospective Cohort Study.
Journal of asthma and allergyCoronary Vasospasm in Eosinophilic Granulomatosis With Polyangiitis Presenting as Acute Coronary Syndrome Treated With Anti-IL-5.
JACC. Case reportsCurrent definition of remission in eosinophilic granulomatosis with polyangiitis (EGPA) and future perspectives.
Allergology international : official journal of the Japanese Society of AllergologyModification of 2022 ACR/EULAR ANCA-associated vasculitis classification criteria in a Japanese cohort (J-CANVAS).
Clinical and experimental rheumatologyLoeffler endocarditis in hypereosinophilic syndrome, the paradox of absent peripheral eosinophilia: a case report.
European heart journal. Case reportsChronic rhinosinusitis is a risk factor for interstitial lung disease in Sjogren's syndrome.
BMC pulmonary medicineCase Report: Sequential use of tezepelumab and mepolizumab for eosinophilic otitis media in EGPA: a steroid-sparing strategy.
Frontiers in allergyBenralizumab for adults with rare and off-label eosinophilic disorders: a 52-week prospective, single-center study.
Frontiers in immunologyPre-existing diabetes mellitus and PR3-ANCA positivity as predictors of relapse in ANCA-associated vasculitis: a retrospective cohort study.
Clinical rheumatologyA presentation of recurrent cerebral ischemic stroke in eosinophilic granulomatosis with polyangiitis: a case report.
Journal of medical case reportsEosinophil Extracellular Trap Remnants as Potential Biomarkers for the Diagnosis and Activity Assessment of Otitis Media Associated With Eosinophilic Granulomatosis With Polyangiitis and Eosinophilic Otitis Media.
Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and NeurotologyAnswer: Unravelling embolic stroke in eosinophilic granulomatosis with polyangiitis: insights from cardiac imaging.
European heart journal. Acute cardiovascular careMyeloperoxidase-anti-neutrophil cytoplasmic antibody positivity and disease characteristics, treatment, and prognosis in eosinophilic granulomatosis with polyangiitis.
Scandinavian journal of rheumatologyImmune cell crosstalk between ANCA-associated vasculitis and IgG4-related disease: an unresolved pathogenic link.
Frontiers in immunologyProgressive Purpura in Microscopic Polyangiitis: A Case Report.
CureusLong-term effectiveness and safety of benralizumab in EGPA: a 3-year single-center experience.
Annals of medicineLiving with Eosinophilic Granulomatosis with Polyangiitis (EGPA): Podcast of a Patient-Physician Discussion.
Advances in therapy[A case of eosinophilic granulomatosis with polyangiitis initially presenting with allergic rhinitis and sinusitis].
Zhonghua er bi yan hou tou jing wai ke za zhi = Chinese journal of otorhinolaryngology head and neck surgeryVasculitis syndromes: large vessel vasculitides and ANCA-associated vasculitides from a neuroradiologist's perspective.
Japanese journal of radiologyNavigating Anesthesia for Thoracoscopic Biopsy in a Six-Year-Old Child With Eosinophilic Granulomatosis With Polyangiitis, Stroke Sequelae, and Pulmonary Collapse.
CureusScleritis Secondary to Eosinophilic Granulomatosis With Polyangiitis.
Journal of vitreoretinal diseasesQuestion: Unravelling embolic stroke in eosinophilic granulomatosis with polyangiitis: insights from cardiac imaging.
European heart journal. Acute cardiovascular careClinical management of dupilumab-induced blood eosinophilia in CRSwNP: a practical algorithm.
RhinologyPulmonary Involvement in Eosinophilic Granulomatosis with Polyangiitis.
Journal of the Belgian Society of Radiology[Recent developments in the treatment of ANCA-associated vasculitides].
La Revue du praticien[Epidemiology and risk factors of ANCAassociated vasculitides].
La Revue du praticien[Special features of ANCA-associated vasculitides].
La Revue du praticienTwo-year Outcomes of Remission Induction Therapy with Mepolizumab for Eosinophilic Granulomatosis with Polyangiitis: A Case Series.
Internal medicine (Tokyo, Japan)Kimura Disease With Eosinophilic Granulomatosis With Polyangiitis Successfully Treated by Mepolizumab.
Clinical case reportsPanuveitis Associated With Idiopathic Hypereosinophilic Syndrome.
Journal of vitreoretinal diseasesEosinophilic Lung Diseases.
Clinics in chest medicineHealthcare Resource Utilisation and Costs in Patients with Eosinophilic Granulomatosis with Polyangiitis: A Retrospective Analysis of German Insurance Claims Data.
Rheumatology and therapyChallenges in the diagnosis, classification and prognosis of ANCA-associated vasculitis.
Nature reviews. RheumatologyCauda equina neuritis in eosinophilic granulomatosis with polyangiitis.
Rheumatology (Oxford, England)Interleukin-5, Eosinophils, and Interleukin-5 Pathway Inhibitors in Eosinophilic Granulomatosis With Polyangiitis.
Arthritis & rheumatology (Hoboken, N.J.)Eosinophilic Myocarditis Treated with IL-5 Blockade: An Integrated Case Report and Literature Review.
Journal of clinical medicine[Ear, nose and throat manifestations in ANCA-associated vasculitis].
La Revue de medecine interneCentral Retinal Artery Occlusion Induced by Dupilumab: A Unique Manifestation of Eosinophilic Granulomatosis with Polyangiitis.
Internal medicine (Tokyo, Japan)Real-world safety and effectiveness of mepolizumab for patients with eosinophilic granulomatosis with polyangiitis in Japan: A subgroup analysis of the MARS study.
Modern rheumatologyAntineutrophil cytoplasmic antibody related vasculitis with a unique imaging presentation of organizing pneumonia. The key role of lung ultrasound.
Multidisciplinary respiratory medicineLoeffler's Endocarditis Unveils Underlying Eosinophilic Granulomatosis With Polyangiitis.
Anatolian journal of cardiologyBiologic Therapies in Severe Asthma and Eosinophilic Granulomatosis with Polyangiitis: Targeted Therapy and Personalized Care.
Tuberculosis and respiratory diseases[TWO CASES OF MPO-ANCA-POSITIVE EGPA WITH CHRONIC RHINOSINUSITIS SHOWING DIFFERENT RESPONSES TO MEPOLIZUMAB].
Arerugi = [Allergy]Antineutrophil Cytoplasmic Antibody Negative Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss) Presenting as Acute Decompensated Heart Failure.
JACC. Case reportsDivergent Risk Patterns of Upper Airway Inflammation in Autoimmune Diseases: A Nationwide Retrospective Analysis.
American journal of rhinology & allergyDupilumab-associated anti-glomerular basement membrane nephritis in a patient with eosinophilic granulomatosis with polyangiitis: a case report.
BMC nephrologyProteinase 3-Antineutrophil Cytoplasmic Antibody (PR3-ANCA)-Associated Vasculitis: A Case Report.
CureusResidual Eosinophilic Inflammation of Sinonasal Mucosa Under the Maintenance Phase of Eosinophilic Granulomatosis With Polyangiitis.
CureusDupilumab-induced eosinophilic granulomatosis with polyangiitis mimicking giant cell arteritis.
Canadian journal of ophthalmology. Journal canadien d'ophtalmologie[Successful benralizumab therapy for severe asthma secondary to EGPA refractory to mepolizumab].
Revista alergia Mexico (Tecamachalco, Puebla, Mexico : 1993)Psoriasis Increases the Risk of ANCA Associated Vasculitis: Insights from A Propensity Score-Matched Study.
ImmunoTargets and therapyEosinophilic myocarditis: case series and review of the literature.
Minerva cardiology and angiologyEosinophilic granulomatosis with polyangiitis treated with Mepolizumab and Rituximab combination therapy - a case report.
ARP rheumatologyMedication adherence and real-world factors of first line induction treatment selection in ANCA-associated vasculitis.
Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOGMononeuritis Multiplex as a Diagnostic Challenge in Eosinophilic Granulomatosis With Polyangiitis: Implications of Early Versus Delayed Recognition.
CureusThe Effect of Benralizumab and Mepolizumab on Use of Oral Glucocorticoids in Patients With Eosinophilic Granulomatosis With Polyangiitis.
Arthritis & rheumatology (Hoboken, N.J.)Sudden visual loss and hypereosinophilia: A case of eosinophilic granulomatosis with polyangiitis.
Modern rheumatology case reportsThe hypereosinophilia dilemma: what's beyond cardiac involvement?
Romanian journal of internal medicine = Revue roumaine de medecine interne"A Case of Eosinophilic Granulomatosis With Polyangiitis Presenting as Quadriplegia": A Clinical and Diagnostic Report.
Journal of investigative medicine high impact case reports[Distribution of eosinophilic granulocytes in the gastrointestinal tract in EGPA].
Zeitschrift fur RheumatologieMepolizumab in patients with eosinophilic granulomatosis with polyangiitis reduced glucocorticoid dose and improved residual symptoms compared to conventional immunosuppressants: a retrospective observational study.
Rheumatology internationalPulmonary nontuberculous mycobacteria disease and anti-neutrophil cytoplasmic antibody positivity: a retrospective analysis of long-term clinical outcomes including vasculitis onset.
Clinical and experimental rheumatologyClinical value at baseline and follow-up of myeloperoxidase-antibodies in ANCA-associated vasculitis.
Frontiers in immunologyEosinophilic granulomatosis with polyangiitis complicated with bullous pemphigoid.
BMJ case reportsOrbital Presentation of Eosinophilic Granulomatosis with Polyangiitis: An Interventional Case Report and Literature Review.
Journal of ophthalmic & vision research[Biologicals in Type-2-Inflammation - from Chronic Rhinosinusitis with Nasal Polyps to Hypereosinophilic Syndrome].
Laryngo- rhino- otologieSystematic Literature Review of Real-World Outcomes of Benralizumab in Eosinophilic Granulomatosis With Polyangiitis.
The journal of allergy and clinical immunology. In practiceBenralizumab in a Patient With Refractory Eosinophilic Endocarditis.
Journal of medical casesPotential masking of new-onset or relapsed eosinophilic granulomatosis with polyangiitis during benralizumab treatment: A case series.
The journal of allergy and clinical immunology. GlobalA case report of fulminant eosinophilic myo-pericarditis related to checkpoint inhibitors complicated by acute heart failure and cardiac tamponade.
European heart journal. Case reportsThe woman with a swollen tongue : A rare case of an ANCA-negative localized eosinophil-rich granulomatous inflammation affecting solely the tongue.
HNODiscontinuation of Oral Glucocorticoids and Achievement of Remission in Patients With Eosinophilic Granulomatosis With Polyangiitis Treated With Benralizumab or Mepolizumab.
ACR open rheumatologyAssociated factors to health-related quality of life in anti-neutrophil cytoplasmic antibodies-associated vasculitis patients: Data from the Almenara Vasculitis Cohort.
Reumatologia clinicaThe Way Back from Tetraplegia or, Rare Neurological Manifestations of Eosinophil Granulomatosis with Polyangiitis.
Journal of clinical medicine[Pulmonary manifestations of ANCA-associated vasculitis in imaging].
Radiologie (Heidelberg, Germany)Exact power and sample size in clinical trials with two co-primary binary endpoints.
Statistical methods in medical researchCardiac disease in patients with vasculitis.
Clinical research in cardiology : official journal of the German Cardiac SocietyExploiting pleiotropy to enhance variant discovery with functional false discovery rates.
Nature computational scienceUpadacitinib in Eosinophilic Granulomatosis With Polyangiitis: A Clinical Case Study.
International journal of dermatologyCardiac magnetic resonance imaging analysis of cardiac involvement in eosinophilic granulomatosis with polyangiitis.
Modern rheumatologyEosinophilic Granulomatosis With Polyangiitis (EGPA) Hidden in Acute Appendicitis: A Case Revealed Through Histopathological Examination.
CureusTofacitinib in combination with glucocorticoids in the treatment of eosinophilic granulomatosis with polyangiitis: a pilot study of 11 cases.
Rheumatology (Oxford, England)Clinical value of biomarkers in relation to artery size in eosinophilic granulomatosis with polyangiitis: findings from an inception cohort at a Japanese City Hospital.
Clinical rheumatologyDiagnostic Salivary Gland Biopsy in Pediatric Eosinophilic Granuolmatosis with Polyangiitis.
OTO openInitial Hemorrhagic Pericardial Effusion Evolving Into Perimyocarditis: An Atypical Early Presentation of Eosinophilic Granulomatosis With Polyangiitis.
CureusAn Unusual Case of Pulmonary Embolism Leading to the Diagnosis of Eosinophilic Granulomatosis With Polyangiitis.
JACC. Case reportsUnique case of mononeuritis multiplex in a patient of Eosinophilic granulomatosis with polyangiitis.
Journal of family medicine and primary careEsophageal Involvement in Eosinophilic Granulomatosis With Polyangiitis.
Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological AssociationEvaluation of Nailfold Capillaroscopy as a Novel Tool in the Assessment of Eosinophilic Granulomatosis with Polyangiitis.
Journal of clinical medicineImmunological Markers Associated with Skin Manifestations of EGPA.
International journal of molecular sciencesTwo-year efficacy and safety of anti-interleukin-5/receptor therapy for eosinophilic granulomatosis with polyangiitis.
Annals of the rheumatic diseasesSeptic Shock Caused by Small Bowel Ischemia in Eosinophilic Granulomatosis With Polyangiitis.
United European gastroenterology journalClinical Burden and Costs of Anti-Neutrophil Cytoplasmic Antibody-ANCA-Associated Vasculitis: Main Findings from REDCap Registry of a University Hospital in Spain.
ClinicoEconomics and outcomes research : CEORAnti-IL5/IL-5 receptor therapies for eosinophilic granulomatosis with polyangiitis: an updated Systematic Review.
Frontiers in immunologyMepolizumab treatment for eosinophilic granulomatosis with polyangiitis (EGPA) with cardiac involvement.
Rheumatology (Oxford, England)Gastrointestinal Presentation of Eosinophilic Granulomatosis with Polyangiitis, Formerly Churg-Strauss Syndrome: A Case Report.
Case reports in gastroenterologyEosinophilic Granulomatosis With Polyangiitis Presenting as Isolated Coronary Vasculitis.
JACC. Case reportsEosinophilic granulomatosis with polyangiitis and facial swelling.
European journal of dermatology : EJDIn Response to Churg-Strauss Syndrome: A Case of Laryngeal Presentation.
The LaryngoscopeAssociation Between Hearing Loss and Systemic Small-Vessel Vasculitis: Audiological Aspects Across Disease Types.
Medicina (Kaunas, Lithuania)A case of cardiac arrest and spontaneous renal hemorrhage in a male patient with persistent eosinophilia: highlighting the importance of early diagnosis of eosinophilic granulomatosis with polyangiitis.
Journal of Zhejiang University. Science. BRituximab Versus Conventional Therapy for Remission Induction in Eosinophilic Granulomatosis With Polyangiitis : A Randomized Controlled Trial.
Annals of internal medicineA Rare Presentation of Bilateral Foot Drop in Anti-neutrophil Cytoplasmic Antibody (ANCA)-Negative Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome): A Diagnostic Challenge.
CureusHypereosinophilic syndrome with elevated IgG4 and T-cell clonality: A report of two cases.
Open life sciencesEndomyocarditis in Eosinophilic Granulomatosis With Polyangiitis: Cardiac Magnetic Resonance Imaging Highlights Treatment Success.
JACC. Case reportsRefractory Coronary Vasospasms in a Patient With Eosinophilic Granulomatosis With Polyangiitis.
JACC. Case reportsDupilumab-induced Eosinophilic Granulomatosis with Polyangiitis Complicated by Peripheral Neuropathic Pain: a Case Report and Literature Review.
Journal of clinical immunologyCase Report: Coadministration of TNF-α blockade as an effective adjunctive strategy in the treatment of ANCA-negative EGPA with refractory peripheral neuropathy.
Frontiers in pharmacologyGiant Cell Arteritis With Atypical Features: Acute Vision Loss and Marked Eosinophilia Mimicking Systemic Vasculitis.
CureusMyositis Associated With Eosinophilic Granulomatosis With Polyangiitis Demonstrated by FDG PET/CT.
Clinical nuclear medicineAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
Associação brasileira dedicada a Imunodeficiências primárias.
Pioneiro da triagem neonatal na América Latina (1976). 17 milhões de bebês triados.
Comunidades
Grupos ativos de quem convive com esta doença aqui no Raras
Ainda não existe comunidade no Raras para Granulomatose eosinofílica com poliangeíte
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- [Successful early use of mepolizumab in eosinophilic granulomatosis with polyangiitis presenting with peripheral neuropathy and gastrointestinal bleeding].
- Clinical and Pathological Features of Vasculitic Neuropathy: A Single-Center Study in China.
- Leukotriene receptor antagonists and eosinophilic granulomatosis with polyangiitis: a disproportionality analysis from FAERS, JADER, CVAR databases integrated with network pharmacology.
- ANCA-associated vasculitis following sotatercept initiation in a patient with heritable pulmonary arterial hypertension and previously silent eosinophilic granulomatosis with polyangiitis: a case report.
- Natural History of Patients With Histologically Proven Acute Eosinophilic Myocarditis.
- Antineutrophil Cytoplasmic Antibody-Negative Eosinophilic Granulomatosis With Polyangiitis in a Pediatric Patient.
- Latent Class Analysis to Explore Subtypes of EGPA: Focusing on Respiratory Involvement and Inflammation Markers.
- [DIAGNOSIS OF ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-NEGATIVE EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS BASED ON RENAL PATHOLOGICAL FINDINGS WITHOUT ASTHMA].
- Ischemic stroke in eosinophilic granulomatosis with polyangiitis: a single-center retrospective cohort study.
- Successful treatment of eosinophilic granulomatosis with polyangiitis and Crohn's disease with combined mepolizumab and adalimumab.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:183(Orphanet)
- MONDO:0015943(MONDO)
- GARD:6111(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q32811(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
