Search Results (71)

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare vasculitis. A key bio-logical hallmark is peripheral blood eosinophilia while antineutrophil cytoplasmic an-tibody (ANCA) positivity is not always present. Therefore, an early phase suspicion of EGPA leading to a correct diagnosis can be challenging. We performed an observa-tional cross-sectional study limited to baseline data on 38 patients between December 2019 and December 2025 at the Allergy and Clinical Immunology Unit, Messina, Italy. Data collection included eosinophil, neutrophil, and lymphocyte counts; ANCAs and antinuclear antibodies (ANAs); erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) determination; and the Birmingham vasculitis activity score (BVAS) questionnaire: Median eosinophil counts were 685 cells/µL, the neutro-phil-to-lymphocyte ratio (NLR) was 2.29, ESR median values were 24 mm/h, and CRPs were 1.99 mg/L. ANCAs were positive in five patients, and ANAs in 20. The trend in ANA+ patients was of lower median eosinophil counts and higher ESR and CRP me-dian values and median BVASs. General, nervous system, and ENT BVAS domains had higher scores.: Disease activity in EGPA at diagnosis can be difficult to recognize. We defined it as chameleon-like and could not be captured by specific immunological sig-natures. Therefore, an early diagnosis needs multidimensional assessment, integrating laboratory parameters with clinical scores. In our EGPA cohort, ANAs were detected in just over half the patients, suggesting that rather than acting as bystanders, these could help clinicians, together with eosinophil counts, NLRs, and BVASs, to suspect EGPA at an early stage for referral to specialized medical centers. Full article

Background: IL-5 inhibitors are effective at inducing remission in EGPA but the comparative benefit of specific drugs has been poorly studied. Methods: A comprehensive search of PubMed and EMBASE was conducted on 10 October 2025 to identify all studies that directly compare mepolizumab with benralizumab in the treatment of EGPA. Risk of bias was assessed using the Cochrane Risk of Bias 2.0 and ROBINS-I V2 tools. Data extraction and statistical analysis were performed using RevMan software to calculate Odds’ Ratios and assess heterogeneity with the I² statistic. Results: Three studies including a total of 365 participants were analyzed. All patients failed prior treatment, and most patients had a BVAS > 2 and glucocorticoid doses of 10 mg or higher. There was no difference in induction of clinical remission with mepolizumab as compared to benralizumab (OR 0.66 [95% CI 0.43–1.01], I² = 0%). There was decreased odds of glucocorticoid discontinuation with mepolizumab (OR 0.61 [95% CI: 0.38–0.99], I² = 0%). There was no difference in achieving glucocorticoid dose less than 5 mg (OR 0.73 [95% CI: 0.18–2.91], I² = 26%), adverse events (OR 1.26 [95% CI: 0.51–3.12], I² = 8%), or adverse events requiring discontinuation of therapy (OR 0.62 [95% CI: 0.21–1.85], I² = 62%). Conclusions: Both benralizumab and mepolizumab effectively induced remission in EGPA. There was a reduced odds of glucocorticoid discontinuation with mepolizumab thus there is a need for prospective head-to-head trials powered to detect a relative glucocorticoid-sparing effect to further assess this finding. Full article

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare and heterogeneous autoimmune disease involving multiple organs and systems, characterized by asthma, eosinophilia, and granulomatous or vasculitic involvement of several organs. Most of the initial symptoms include rhinitis, sinusitis, and asthma. The primary consultation departments are typically allergy, otolaryngology, and respiratory. EGPA lacks effective biomarkers for early diagnosis, and most patients have experienced serious multiple organ damage at the time of diagnosis, which makes it more difficult to treat. Therefore, as an allergist, improving the understanding of EGPA, especially the early identification of EGPA in the rhinitis, asthma, or sinusitis stage, will effectively shorten the overall course of EGPA and reduce the mortality and disability rates. Based on this, the author compiled a diagnosis and treatment pathway to outline the clinical manifestations; examination, diagnosis, and treatment pathways; differential diagnosis; disease management; and other aspects of EGPA in the allergic department, aiming to promote early detection, identification, and diagnosis by allergists. Full article

Background: Eosinophilic granulomatosis with polyangiitis (EGPA), formerly Churg–Strauss syndrome, is a rare necrotizing vasculitis characterized by asthma, eosinophilia, and systemic granulomatosis vasculitis. Perioperative risk is primarily driven by airway hyperreactivity, potential cardiac disease, chronic immunosuppressive therapy, and reported alterations in plasma cholinesterase activity. Evidence specifically addressing anesthetic management remains scarce and largely limited to case-based reports. Methods: A focused narrative review was conducted by searching MEDLINE (via PubMed), Scopus, and Embase from inception to January 2026 for publications reporting perioperative anesthetic management in patients with EGPA/Churg–Strauss syndrome. Case reports and case-based descriptions providing explicit anesthetic details were qualitatively synthesized. Results: Available evidence consists predominantly of isolated case reports across heterogeneous surgical settings, including ENT, abdominal, orthopedic, ambulatory, pediatric, and rare cardiac procedures. Recurring perioperative principles include optimization of bronchial disease and continuation of inhaled therapy; minimization of airway stimulation and avoidance of histamine-releasing drugs; selection of induction agents preserving hemodynamic stability in the presence of myocardial involvement; preference for non-depolarizing neuromuscular blockade with quantitative monitoring (and consideration for sugammadex when appropriate); individualized corticosteroid management and multimodal, opioid-sparing analgesia, often supported by regional techniques. Conclusions: In the absence of dedicated perioperative guidelines, anesthetic care for EGPA should be individualized based on clinical phenotype and organ involvement. A structured approach targeting airway protection, cardiovascular stability, safe neuromuscular management, and opioid-sparing analgesia may represent a pragmatic risk-mitigation framework. These considerations are illustrated by an institutional experience in mitral valve surgery. Full article

Background/Objectives: Dupilumab is a fully human IgG4 monoclonal antibody targeting the interleukin-4 receptor α subunit, inhibiting interleukin-4 and interleukin-13 signalling, and suppressing type 2 inflammation. It is approved for several eosinophilic and type 2 inflammatory diseases, including chronic rhinosinusitis with nasal polyps, asthma, atopic dermatitis, eosinophilic oesophagitis, and, more recently, eosinophilic chronic obstructive pulmonary disease. Although generally well tolerated, dupilumab has been associated with peripheral eosinophilia and, rarely, eosinophil-mediated complications. This study aims to describe cases of eosinophilic granulomatosis with polyangiitis (EGPA) occurring after dupilumab initiation and to review available evidence on this association. Methods: We describe two cases of new-onset EGPA developing after the introduction of dupilumab therapy, analysing clinical features, laboratory findings, management, and outcomes. A narrative review of published case reports and literature addressing dupilumab-associated eosinophilia and EGPA was also performed. Results: Both patients developed EGPA after starting dupilumab, presenting with marked peripheral eosinophilia and systemic manifestations consistent with the disease. Clinical improvement was observed following dupilumab discontinuation and initiation of appropriate immunosuppressive treatment. The literature review identified a small number of similar reports describing EGPA onset or unmasking in temporal association with dupilumab, mainly in patients with underlying type 2 inflammatory disorders. Conclusions: While a causal relationship between dupilumab and EGPA remains unproven, these findings highlight the importance of clinical awareness. Dupilumab remains an effective therapy for severe type 2 inflammatory diseases; careful monitoring may allow early recognition and management of rare eosinophilic complications. Full article

Background: The anti-IL-5 monoclonal antibody, mepolizumab, has shown clinical efficacy and safety for the treatment of severe eosinophilic asthma (SEA), chronic rhinosinusitis with nasal polyps (CRwNP) and eosinophilic granulomatosis with polyangiitis (EGPA). We aimed to investigate the trajectories of the inflammatory cytokines at the systemic level during mepolizumab treatment, in SEA, SEA with CRwNP, and EGPA. Material and Methods: Treatment response was explored within a real-life observational prospective study. Clinical, functional and inflammatory outcomes as well as serum T2 (IL-4, IL-5 and IL-13) and non-T2 cytokine trends (including IL-5, IL-6, IL-13, IL-10) were evaluated at baseline and 6–12 months after mepolizumab initiation. Results: Overall, 45 patients were consecutively enrolled (SEA: 18; SEA with CRwNP: 9; EGPA: 18), including 27 females, with an average cohort age 60.65 years. Clinical parameters (FEV1, FeNO, SNOT 22, ACT, blood eosinophil count) improved in the different subgroups regardless of coexisting determinants of potential higher disease burden, including CRwNP and previous EGPA history. Cytokine analysis revealed heterogeneous profiles at baseline and statistically significant changes in IL-5 and IL-10 concentrations within the same disease subgroup at different time points. Conclusions: Our observations suggest the ability of mepolizumab to modulate both T2 and non-T2 immune pathways and highlight the persistence of slightly different molecular profile in different severe asthma patients depending on concomitant conditions, which is relevant for the long-term follow-up and potential association therapy combining options which address different targets. More research and larger studies are needed. Full article

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare vasculitis, and contemporary population data from Central and Eastern Europe are limited. Aim: To describe hospital-based incidence, patient characteristics and comorbidities among EGPA hospitalizations in Poland (2014–2023), including differences by age, sex and place of residence. Methods: This retrospective, population-based study used nationwide hospital discharge records with an EGPA diagnosis. First EGPA-coded hospitalizations were used to estimate annual incidence per 1,000,000 inhabitants. Demographics, duration of stay and accompanying comorbidities (cardiovascular disease, pulmonary disease, and asthma) were analyzed for all EGPA hospitalizations. Place of residence was classified as urban or rural. Group differences and temporal trends were assessed using appropriate parametric and non-parametric tests and regression models, with a two-sided p value < 0.05 considered statistically significant. Results: Between 2014 and 2023, 911 patients had a first EGPA-coded hospitalisation in Poland, corresponding to a mean annual hospital-based incidence of 2.38 per 1,000,000 inhabitants (range 1.28–3.38); incidence declined significantly from 2014 to 2019 (p < 0.001) and was disrupted during the COVID-19 period. Overall, 3524 EGPA hospitalisations were recorded, and women were more frequently hospitalised than men (54.5% vs. 45.2%; p < 0.001). Mean age at hospitalisation increased over time, with patients in 2023 being about 5–6 years older than in 2014 (p ≤ 0.009). Median length of stay was 8 days for first admissions and 5 days for all EGPA stays and shortened significantly over the study period (p < 0.001). Cardiovascular disease, pulmonary disease and asthma were present in 23.6%, 35.3% and 32.3% of patients, respectively. Cardiovascular disease was more common in men and in rural residents (both p < 0.001) and was associated with older age (p < 0.001), whereas pulmonary disease was associated with younger age (p < 0.001). Among women, the proportions with pulmonary disease and asthma decreased over time (p = 0.009 and p = 0.025). Conclusions: EGPA in Poland is rare, with hospital-based incidence comparable to other European and Asian populations. The hospitalized EGPA population is aging and cardiovascular comorbidity is increasingly prominent, especially in older and rural patients, while recorded pulmonary disease and asthma in women are decreasing. Full article

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis characterized by asthma, eosinophilia, and necrotizing inflammation of small- to medium-sized vessels. Accumulating evidence indicates that EGPA is a polygenic and heterogeneous disorder comprising distinct antineutrophil cytoplasmic antibody (ANCA)–defined endotypes with divergent genetic backgrounds, immune pathways, and clinical phenotypes. Its pathogenesis reflects the convergence of epithelial–alarmin signaling, type 2 inflammation, eosinophil effector mechanisms, and B-cell/autoantibody responses, with myeloperoxidase (MPO)-ANCA serving as a hallmark of the vasculitic subset. Recent advances in genomics, immunology, and multi-omics profiling have uncovered biomarkers and molecular circuits sustaining disease activity and guiding therapeutic stratification. The identification of the interleukin (IL)-5–eosinophil axis, epithelial-derived alarmins, and B-cell/IgG4 networks as central pathogenic nodes has enabled the development of targeted biologic therapies that are redefining treatment paradigms. Benralizumab (anti-IL-5Rα) has recently been approved for EGPA following the phase 3 head-to-head MANDARA trial, which demonstrated non-inferiority to mepolizumab in achieving remission (BVAS = 0 with ≤4 mg/day prednisone equivalent) at weeks 36 and 48. These results, together with the established efficacy of mepolizumab, inform practical selection between IL-5 and IL-5Rα blockade and support glucocorticoid-sparing approaches. A structured literature search (2015–2025) was conducted in PubMed, Scopus, and Web of Science to identify recent advances in epidemiology, genetics, biomarkers, and targeted therapies for EGPA. This updated review integrates molecular insights, clinical endotypes, and therapeutic innovations to outline current evidence and future precision-medicine strategies aimed at improving long-term patient outcomes. Full article

Background/Objectives: Eosinophilic myocarditis (EM) is a rare, life-threatening inflammatory cardiomyopathy driven by eosinophil cytotoxicity and extracellular trap formation. Interleukin-5 (IL-5) inhibition may disrupt this pathogenic cascade. We reviewed contemporary evidence on IL-5 blockade in EM and contextualized it with an illustrative case. Methods: We searched PubMed through May 2025 for reports of EM treated with mepolizumab or benralizumab. Inclusion criteria were consistent with prior cohorts: acute cardiac symptoms with biomarker elevation plus abnormalities on transthoracic echocardiography and/or cardiac magnetic resonance imaging (CMR), along with documented IL-5-targeted therapy. We extracted clinical, imaging, biopsy, treatment-timing, and outcome data and included one institutional case. Results: Twenty-one episodes were analyzed (median age, 45 years; 10 men). Underlying conditions included eosinophilic granulomatosis with polyangiitis (10 cases; 48%), hypereosinophilic syndrome (5 cases; 24%), drug reaction with eosinophilia and systemic symptoms (DRESS, 3 cases; 14%), and eosinophilic asthma (3 cases; 14%). Treatments involved mepolizumab in 17 cases (81%) and benralizumab in 4 (19%); 4 patients received “early-start” therapy within 14 days of EM diagnosis. Among the 11 episodes with reported left ventricular ejection fraction (LVEF) at baseline and follow-up, the median baseline LVEF was 40% (range, 30–62), with 10 of 11 (91%) <50%. On follow-up, all 11 patients improved: 4 normalized (≥50%) and 7 improved to 40–49%. CMR (n = 18) demonstrated late gadolinium enhancement in 14 cases (78%), edema in 9 (50%), and intracardiac thrombus in 4 (22%). Endomyocardial biopsy confirmed eosinophilic infiltration in 13 of 15 cases (87%). Outcomes included one death (fulminant DRESS), one recovery following veno-arterial extracorporeal membrane oxygenation, and one successful heart transplantation. Illustrative case: A 24-year-old man on a steroid taper received mepolizumab 300 mg on Day 4. His LVEF improved from 47% to 59% by Day 15, accompanied by biomarker decline and successful steroid tapering. Conclusions: Across published cases and our institutional experience, IL-5–targeted therapy appears safe, steroid-sparing, and associated with rapid ventricular recovery, particularly when initiated early. Although limited, these findings support the need for prospective trials to define the optimal agent, dosing, timing, and integration with standard immunosuppression and anticoagulation. Full article

Central nervous system (CNS) involvement is an extremely rare manifestation in eosinophilic granulomatosis with polyangiitis (EGPA), associated with a poor prognosis. Here we present a case of 50-year-old female patient with long-term asthma treatment who presented initially with extreme eosinophilia (56%) and severe progressive ascending paresis, similar to Guillain–Barré syndrome, leading to tetraplegia. After navigating through diagnostic mazes, the diagnosis of EGPA was established based on eosinophilia, myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) positivity, asthma, eosinophil granulomatosis in the gastrointestinal tract, and severe peripheral nervous system involvement, complicated with rare central nervous granulomas and ischemia. With combined immunosuppressive and immunomodulatory treatment including high-dose corticosteroids, rituximab and intravenous immunoglobulin along with symptomatic treatment and planned rehabilitation over 6 months, our patient recovered gradually from tetraplegia and adverse events such as severe infections and osteoporotic fractures. Now, from a 2-year perspective, we can conclude a successful treatment leading to decrease in all of her symptoms. Due to persistent eosinophilia after steroid tapering, she was switched to mepolizumab maintenance treatment and demonstrated continuous improvement of motor and sensory functions. Thanks to periodically repeated rehabilitation, she became self-sufficient and returned to her previous job. Our case highlights that EGPA patients should be treated in a center of expertise due to the rarity of the disease and complexity of diagnosis and treatment. Careful multidisciplinary cooperation, the huge effort of the patient, and a supportive environment can show a way back from immune-mediated tetraplegia. Full article

Eosinophilic Granulomatosis with Polyangiitis (EGPA) is a rare systemic vasculitis with eosinophilic inflammation and variable clinical presentations. Although skin manifestations are frequent, current classification criteria do not include them, which may underestimate their diagnostic value. This prospective observational study aimed to assess systemic and skin involvement as well as eosinophilia, anti-neutrophil cytoplasmic antibody (ANCA), and Anti-nuclear antibodies (ANA) serum levels in 20 EGPA patients followed for one year at the University Hospital of Messina, Italy, before starting Mepolizumab, 300 mg. Eosinophilia, ANCA status, systemic and skin involvement were also evaluated at 6 and 12 months; a literature review on these data supplements our findings. Skin involvement was present in 55% of patients, including purpura, urticarial vasculitis, angioedema, maculopapular rash, and nodules, mostly in ANCA-negative patients, though purpura was more frequent in ANCA-positive cases but without any statistically significant correlation. ANAs were present in 50% of patients, together with ANCA in two subjects and without in eight. Mepolizumab significantly reduced eosinophil levels, BVASs, and corticosteroid dependence, with notable improvement in skin symptoms. In conclusion, skin manifestations are common in EGPA and may represent useful indicators of disease activity. Their integration with ANCA status, eosinophil counts, and positivity to other autoantibodies could enhance diagnostic and monitoring strategies identifying different clusters of EGPA patients even if the small sample size limits the generalizability of the findings. Full article

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), represent a spectrum of systemic disorders characterized by necrotizing inflammation of small- to medium-sized vessels. Nailfold videocapillaroscopy (NVC) is a validated, non-invasive technique routinely employed in the assessment of microvascular involvement in systemic sclerosis and in the differential diagnosis of Raynaud’s phenomenon; its application in the context of AAV, particularly EGPA, has not been investigated yet. The present study aims to assess the presence and the possible pattern of microcirculatory abnormalities detected by NVC in EGPA patients, and to explore potential correlations between capillaroscopic findings and disease activity status. Methods: A total of 29 patients with EGPA (19 women and 10 men), aged between 51 and 73 years, and 29 age- and sex-matched healthy controls were retrospectively enrolled between October 2023 and April 2025, after providing informed consent and meeting the inclusion and exclusion criteria. NVC was conducted in both groups to assess various morphological parameters, and mean capillary density was also calculated. Results: This study observed the presence of capillaroscopic alterations in the EGPA group, including decreased capillary density (38%), neoangiogenesis (72%), rolling (100%), pericapillary stippling (66%), and inverted capillary apex (52%). Overall, when comparing healthy controls with EGPA patients, microcirculatory abnormalities were significantly more prevalent in the latter. Specifically, scores for neoangiogenesis, capillary rolling, pericapillary stippling, and inverted capillary apex showed p-values < 0.001. Conclusions: Our study demonstrates a higher prevalence of four nailfold videocapillaroscopic abnormalities in patients with EGPA compared to healthy controls. However, the identification of these capillaroscopic alterations as specific to EGPA requires further confirmation. Ongoing studies aim to explore the potential role of NVC as a diagnostic marker and to investigate its correlation with the clinical manifestations of EGPA. Full article

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) constitutes a group of rare diseases characterized by autoimmune-associated inflammation and vessel damage. Based on the clinical manifestations and involvement of immune components, three disease syndromes are distinguished: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). In this review, we present the current data on the epidemiology, the clinical manifestations of each syndrome, and the most up-to-date classification criteria. The role of the underlying genetic and epigenetic abnormalities, as well as their interplay, is described. The immunological diversification of AAV is also described, with a focus on the immune cell dysfunctions detected in patients. In conclusion, we emphasize the urgent need to unravel the sophisticated mechanisms of this disease, which would enable the development of new, effective therapeutic strategies. Full article

Background and Objectives: Systemic small-vessel vasculitis (SV) represents a group of rare autoimmune disorders with varied etiologies and clinical manifestations. Audiovestibular involvement in SV may present with a broad spectrum of symptoms, often complicating diagnosis and management. This study aimed to evaluate auditory function and speech perception in individuals diagnosed with SV and to investigate associations with disease-specific clinical parameters. Materials and Methods: A total of 40 patients diagnosed with SV (mean age: 48.9 years; range: 28–65 years) were recruited for comprehensive audiological assessment. The evaluation protocol included otoscopic examination, tympanometry, pure-tone audiometry, and speech audiometry. Statistical analysis was conducted using R software (version 4.3.1), and significance was set at p < 0.05. Results: Diagnoses included granulomatosis with polyangiitis (52.5%), eosinophilic granulomatosis with polyangiitis (27.5%), necrotizing vasculopathy (12.5%), and microscopic polyangiitis (7.5%). Mean disease duration was 4.14 years. Hearing complaints were reported by 77.5%; in 20%, they were the initial symptoms. Audiometry identified hearing loss in 50% of patients—predominantly sensorineural (33.8%), followed by mixed (13.7%) and conductive (2.5%) types. Hearing loss was most frequent in necrotizing vasculopathy (60%) and among ANCA-positive individuals (53.7%). Conclusions: Sensorineural hearing loss is common in SV, particularly in ANCA-positive patients, highlighting the need for routine hearing assessment in SV management. Full article

Objectives: To analyze variations in patient characteristics, treatment patterns, clinical manifestations, clinical outcomes (i.e., response, flares and flare-free survival in HES; remission, relapses and relapse-free survival in EGPA; and overall survival), and healthcare resource utilization (HCRU) in patients with hypereosinophilic syndrome (HES) and eosinophilic granulomatosis with polyangiitis (EGPA) across five European countries. Methods: In two parallel, retrospective, non-interventional, longitudinal chart review studies (GSK ID: 214661 [EGPA] and 214657 [HES]), physicians collected data of patients they treated in France, Germany, Italy, Spain, and the UK from January 2015 to December 2019, with follow-up until August 2021. Country-level results are presented for each study; HES and EGPA data were pooled in a post hoc exploratory analysis. Results: Per-country, 22–26 HES- and 38–45 EGPA-treating physicians collected data from 52–62 (total 280) patients with HES and 80–85 (total 407) with EGPA. Patient sex and age at diagnosis differed across countries. Pooled HES/EGPA data revealed high oral corticosteroid (OCS) use in all countries (94.9% of patients; median [IQR] duration 20.7 [9.0, 33.8] months); immunosuppressive treatments and biologics use varied between countries (43.7–61.5% and 25.6–59.8%, respectively). The most frequent clinical manifestations were constitutional (51.6–78.8%) and lung (43.5–55.8%) in HES, and lung (41.3–67.9) and ENT (43.8–61.2) in EGPA. Pooled HCRU data showed country-level variation; 70.7–91.2% of patients had disease-related outpatient visits and 36.1–52.6% had ER visits or hospitalizations. Conclusions: Results demonstrate substantial disease burden, including high HCRU and extensive OCS use among patients with HES and EGPA in five European countries. The findings highlight the need for improved treatment strategies such as optimizing use of biologics to mitigate the reliance on corticosteroids. Full article