[This corrects the article DOI: 10.1016/j.ero.2025.11.014.].

OBJECTIVES:Real-world practice patterns of eosinophilic granulomatosis with polyangiitis (EGPA) remain poorly defined. This study aimed to describe current diagnostic and therapeutic approaches across experienced European centres, identifying areas of convergence and variability to inform future standardization of care. METHODS:We distributed a 44-item online survey covering diagnostic evaluation, treatment strategies, patient-reported outcome measures (PROMs) and the role of patient advocacy groups. The survey was reviewed by an expert panel and disseminated within the European EGPA Study Group. Responses were collected anonymously between April and August 2025 for statistical analysis. RESULTS:Fifty-four experts from six countries participated, most with long-standing experience and substantial EGPA caseloads. Multidisciplinary care and screening for cardiac and renal involvement were widely adopted; histological confirmation was reported in fewer than 25% of cases. Treatment strategies varied considerably: over half of respondents initiated anti-IL-5 therapy at diagnosis, and the combination of glucocorticoids, rituximab and mepolizumab was the preferred induction regimen in severe disease. CS tapering protocols differed, with most clinicians targeting withdrawal within 12 months. PROMs and disease-specific questionnaires were used inconsistently, despite broad recognition of their value. Advocacy groups were viewed as crucial, particularly for patient education and referral. CONCLUSION:This first multinational survey reveals substantial heterogeneity in real-world diagnostic and therapeutic practice, reflecting gaps in validated criteria, standardized activity measures and treatment algorithms. These findings highlight the need for coordinated prospective research and harmonized evidence-based guidance to optimize outcomes for patients with EGPA.

OBJECTIVES:ANCA are a key biomarker for ANCA-associated vasculitis (AAV), particularly microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA). Although indirect immunofluorescence (IIF) has traditionally been the reference technique, its diagnostic value in contemporary real-world practice remains uncertain. The aim of this study was to evaluate the diagnostic performance of IIF ANCA testing in routine clinical practice. METHODS:We conducted a retrospective study of all patients with an ANCA request at a tertiary hospital over a 4-year period. All IIF-positive sera were subsequently tested for anti-PR3 and anti-MPO antibodies by chemiluminescent immunoassay (CLIA). Clinical data, test indication and final diagnoses were retrieved from electronic medical records. RESULTS:We included 5157 patients and IIF was positive in 653 (12.7%): perinuclear ANCA (P-ANCA) in 17.9%, cytoplasmic ANCA (C-ANCA) in 13.3% and atypical ANCA (A-ANCA) in 68.8%. CLIA was negative in 97.3% of A-ANCA. AAV was diagnosed in 47 patients, and 42 (89.4%) had positive IIF. For GPA and MPA, IIF showed a sensitivity of 93% and specificity of 88%, with a very high negative predictive value (NPV) (99.9%) but low positive predictive value (PPV) (6.1%). Specificity improved to 96.8% when restricted to typical patterns (C-ANCA or P-ANCA) and to 99.6% when combined with positive CLIA results >20 IU/ml. Almost all AAV cases were diagnosed in patients with high pre-test probability (such as renal disease, lung infiltrates or peripheral neuropathies) and interstitial lung disease was the most frequent non-AAV diagnosis in IIF-positive patients. CONCLUSIONS:ANCA IIF retains good diagnostic efficiency and a very high NPV for GPA and MPA, but has low PPV, particularly when tested for nonspecific symptoms.

OBJECTIVES:permanent visual loss (PVL) is the most frequent ischemic complication of GCA. We aimed to evaluate whether clinical signs, symptoms, and blood test abnormalities at GCA diagnosis can predict PVL. PATIENTS AND METHODS:retrospective, multicenter study of patients with biopsy-proven GCA. The whole cohort was randomly split into a derivation and a validation dataset. Multivariable logistic regression (MVLR) was used to develop a prediction model and a predictive score. The model's performance was determined through the area under the curve (AUC). RESULTS:620 patients were included, 397 in the derivation cohort. PVL occurred in 20.3%. MVLR showed that amaurosis fugax (OR 5.86, 95%CI 3.41-10.07, p<0.001), jaw claudication (OR 2.48, 95%CI 1.51-4.07, p<0.001), and increasing age (OR 1.09, 95%CI 1.05-1.14, p<0.001) were independently associated to PVL. Fever was the only independent protective factor (OR 0.45, 95%CI 0.25-0.84, p=0.01). The optimum cut-off for age as a PVL predictor was 78 years (OR 2.4, 95%CI 1.62-3.59, p<0.001). No laboratory variables were independently associated with PVL. Our model showed an AUC 0.80 (95%CI 0.76-0.85) and good internal validity (AUC 0.82, 95%CI 0.76-0.87). A predictive risk score with a sensitivity of 64.9%, a specificity of 82.0%, and positive and negative predictive values of 46.4% and 90.01% is proposed. CONCLUSIONS:The risk of developing PVL at GCA diagnosis may be estimated upon a detailed patient evaluation, including temporal arteries. Amaurosis fugax, jaw claudication, and older age can predict PVL. Fever is a protective factor. Inflammatory markers do not differentiate patients at risk of developing PVL.

Tocilizumab (TCZ) has demonstrated potential efficacy in managing large-vessel (LV) vasculitis such as giant-cell arteritis (GCA) and Takayasu arteritis (TAK). Despite the shared characteristics between the LV-GCA phenotype and TAK, there are differences between both entities that may affect therapeutic responses to TCZ. We aim to assess and compare the effectiveness and safety of TCZ in patients with LV-GCA and TAK. Multicenter, observational study on 70 LV-GCA patients and 57 TAK patients treated with TCZ. Outcomes were assessed at baseline and at 1, 3, 6 and 12 months post-treatment initiation. The variables analyzed included the following: (a) the achievement of clinical remission and improvement in laboratory markers; (b) imaging-based disease activity; (c) a glucocorticoid (GC)-sparing effect; and (d) side events and a safety profile. At the treatment initiation, TAK patients were younger, exhibited longer disease duration, had received more prior biologics, and were on higher doses of prednisone compared to LV-GCA patients. While TAK patients showed a slower initial clinical response, remission rates at 12 months were comparable between groups (74.5% for LV-GCA vs. 76.9% for TAK). Both groups experienced rapid laboratory marker improvement and a significant GC-sparing effect. However, complete imaging resolution was observed in only 18.9% of LV-GCA patients and 21.1% of TAK patients. The safety profile was similar in both groups, with severe infections leading to TCZ discontinuation in four LV-GCA and three TAK patients. In clinical practice, TCZ demonstrates similar efficacy in promoting remission and reducing GC dependency in both LV-GCA and TAK patients. Nonetheless, discrepancies between clinical outcomes and imaging improvement highlight the need for further investigation into disease monitoring and management strategies.

Objectives:Diagnosing eosinophilic granulomatosis with polyangiitis (EGPA) is challenging due to its rarity, multisystem involvement, and varied clinical presentation, often resulting in delayed diagnoses and associated complications. EGPAware project aimed to establish a first European Delphi consensus on red flags for raising suspicion of EGPA to assist the prompt identification, referral, and diagnosis of these patients. Methods:EGPAware was a European Delphi study led by a multidisciplinary committee that developed the survey based on the red flags from a previously published Spanish systematic review and focused on clinical suspicion indicators. Data analysis used descriptive statistics, with consensus defined as ≥70% agreement. Statements with <70% consensus in the first round were revisited again in round 2. In the second round, panellists additionally ranked the 10 most relevant red flags by clinical importance and frequency of observation. Results:A multidisciplinary European group of 53 physicians with expertise in EGPA completed the 2-round Delphi survey. Consensus was reached on 25 red flags. Mononeuritis multiplex, lung infiltrates/nodules, vasculitis on biopsy, and myeloperoxidase-antineutrophil cytoplasmic antibodies positivity were the highest-ranked signs based on clinical relevance, whereas lung infiltrates/nodules, nasal polyposis, and mononeuritis multiplex were most frequently observed in panellists' experience. Conclusions:The EGPAware study provides a useful checklist of consensual red flags to be used in clinical practice to help clinicians identify patients with EGPA early. The study supports a multidisciplinary approach to EGPA diagnosis and management. Further research is recommended to refine diagnostic tools and validate these findings.

BackgroundAntineutrophil cytoplasmic antibody-associated vasculitis (AAV) are rare autoimmune diseases characterized by inflammation of blood vessels. This study aimed to assess the cost-utility of avacopan in combination with rituximab (RTX) or cyclophosphamide (CYC) compared with glucocorticoids (GC) for the treatment of severe, active AAV in Spain.MethodsA 9-state Markov model was designed to reflect the induction of remission and sustained remission of AAV over a lifetime horizon. Clinical data and utility values were mainly obtained from the ADVOCATE trial, and costs (euro 2022) were sourced from national databases. Quality-adjusted life years (QALYs), and incremental cost-utility ratio (ICUR) were evaluated. An annual discount rate of 3% was applied. Sensitivity analyses were performed to examine the robustness of the results.ResultsAvacopan yielded an increase in effectiveness (6.52 vs. 6.17 QALYs) and costs (euro16,009) compared to GC, resulting in an ICUR of euro45,638 per additional QALY gained. Avacopan was associated with a lower incidence of end-stage renal disease (ESRD), relapse and hospitalization-related adverse events. Sensitivity analyses suggested that the model outputs were robust and that the progression to ESRD was a driver of ICUR.ConclusionsAvacopan is a cost-effective option for patients with severe, active AAV compared to GC in Spain.

BACKGROUND:Giant cell arteritis is an age-related vasculitis that mainly affects the aorta and its branches in individuals aged 50 years and older. Current options for diagnosis and treatment are scarce, highlighting the need to better understand its underlying pathogenesis. Genome-wide association studies (GWAS) have emerged as a powerful tool for unravelling the pathogenic mechanisms involved in complex diseases. We aimed to characterise the genetic basis of giant cell arteritis by performing the largest GWAS of this vasculitis to date and to assess the functional consequences and clinical implications of identified risk loci. METHODS:We collected and meta-analysed genomic data from patients with giant cell arteritis and healthy controls of European ancestry from ten cohorts across Europe and North America. Eligible patients required confirmation of giant cell arteritis diagnosis by positive temporal artery biopsy, positive temporal artery doppler ultrasonography, or imaging techniques confirming large-vessel vasculitis. We assessed the functional consequences of loci associated with giant cell arteritis using cell enrichment analysis, fine-mapping, and causal gene prioritisation. We also performed a drug repurposing analysis and developed a polygenic risk score to explore the clinical implications of our findings. FINDINGS:We included a total of 3498 patients with giant cell arteritis and 15 550 controls. We identified three novel loci associated with risk of giant cell arteritis. Two loci, MFGE8 (rs8029053; p=4·96 × 10-8; OR 1·19 [95% CI 1·12-1·26]) and VTN (rs704; p=2·75 × 10-9; OR 0·84 [0·79-0·89]), were related to angiogenesis pathways and the third locus, CCDC25 (rs11782624; p=1·28 × 10-8; OR 1·18 [1·12-1·25]), was related to neutrophil extracellular traps (NETs). We also found an association between this vasculitis and HLA region and PLG. Variants associated with giant cell arteritis seemed to fulfil a specific regulatory role in crucial immune cell types. Furthermore, we identified several drugs that could represent promising candidates for treatment of this disease. The polygenic risk score model was able to identify individuals at increased risk of developing giant cell arteritis (90th percentile OR 2·87 [95% CI 2·15-3·82]; p=1·73 × 10-13). INTERPRETATION:We have found several additional loci associated with giant cell arteritis, highlighting the crucial role of angiogenesis in disease susceptibility. Our study represents a step forward in the translation of genomic findings to clinical practice in giant cell arteritis, proposing new treatments and a method to measure genetic predisposition to this vasculitis. FUNDING:Institute of Health Carlos III, Spanish Ministry of Science and Innovation, UK Medical Research Council, and National Institute for Health and Care Research.

Anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis is characterised by small vessel necrotising inflammatory vasculitis. Prior to immunosupressant therapy availability it usually led to a fatal outcome. Current treatment has changed ANCA-associated vasculitis into a condition with a significant response rate, although with a not negligible relapse occurrence and cumulative organ lesions, mostly due to drug-related toxicities. The use of glucocorticoids, cyclophosphamide and other immunosupressants (such as azathioprine, mychophenolate and methotrexate) was optimised in a series of clinical trials that established the treatment of reference. In recent years, a better knowledge of B lymphocyte function and the role of complement inhibition has transformed the course of this disease while minimising treatment-related adverse effects. This multidisciplinary document of recommendations is based on the consensus of three scientific societies (Internal Medicine, Nephrology and Rheumatology) and on the best available evidence on diagnosis, treatment and follow-up of patients with ANCA-associated vasculitis, including some special situations. The aim of this document is to provide updated information and well-grounded clinical recommendations to practising physicians as to how to improve the diagnosis and treatment outcome of our patients.

Eosinophil-related diseases represent a group of pathologic conditions with highly heterogeneous clinical presentation and symptoms ranging from mild to critical. Both systemic and localized forms of disease are typically treated with glucocorticoids. The approval of novel biologic therapies targeting the interleukin-5 pathway can help reduce the use of systemic glucocorticoids (SGC) in eosinophilic diseases and reduce the risk of SGC-related adverse effects (AEs). In this article, a panel of experts from different medical specialties reviewed current evidence on the use of SGC in two systemic eosinophilic diseases: Eosinophilic Granulomatosis with PolyAngiitis (EGPA) and HyperEosinophilic Syndrome (HES); and in two single-organ (respiratory) eosinophilic diseases: Chronic RhinoSinusitis with Nasal Polyps (CRSwNP) and Severe Asthma with Eosinophil Phenotype (SA-EP), and contrasted it with their experience in clinical practice. Using nominal group technique, they reached consensus on key aspects related to the dose and tapering of SGC as well as on the initiation of biologics as SGC-sparing agents. Early treatment with biologics could help prevent AEs associated with medium and long-term use of SGC.

INTRODUCTION:Ant-iTNF treatment has been broadly linked with autoantibodies and autoimmune disorders development. After the clinical observation of aPTT (activated partial thromboplastin clotting time) prolongation in our cohort of IBD patients treated with anti-TNF, we sought to determine the presence of antiphospolipid antibodies in our population, along with antiphospholipid syndrome (APS) occurrence. METHODS:We included in the study 289 patients treated with anti-TNFα antibodies. RESULTS:Twenty four of 289 patients presented a prolonged aPPT (8.3%) after starting anti-TNF treatment. We found antiphospholipid antibodies in 70.8% (17/24) of patients with aPTT prolongation. No major thrombotic events were reported although one patient met criteria for APS because of persistent antiphospolipid antibodies and two miscarriages. Another patient was diagnosed with lupus-like syndrome. CONCLUSION:Anti-TNF treatment is associated with the induction of various antibodies, among them, antiphospholipid antibodies. However, a very low number of patients develop APS. Testing for antiphospholipid antibodies patients with prolonged aPPT could identify those at risk and lead to individualized treatment. Additional prospective studies are necessary to acquire more information.

Las vasculitis asociadas a anticuerpos anticitoplasma de neutrófilo (ANCA) se caracterizan por una inflamación necrotizante de vasos pequeños. Antes de la llegada del tratamiento inmunosupresor solían tener un desenlace fatal. El tratamiento ha transformado las vasculitis en una enfermedad con una importante tasa de respuesta, pero con un porcentaje nada despreciable de recidivas y un daño orgánico acumulado, en gran parte debido a las toxicidades relacionadas con los fármacos. El uso de glucocorticoides (GC), ciclofosfamida (CFM)y otros inmunosupresores (como azatioprina [AZA], micofenolato y metotrexato [MTX]) se optimizó mediante una serie de ensayos clínicos que establecieron un tratamiento de referencia. En los últimos años, la mejora de los conocimientos sobre los linfocitos B y la inhibición del complemento ha revolucionado el curso de la enfermedad y minimizado los efectos adversos (EA) del tratamiento.El presente documento multidisciplinar de recomendaciones se ha basado en un consenso de tres especialidades (Medicina Interna, Nefrología y Reumatología) y en la mejor evidencia disponible acerca del diagnóstico, tratamiento y seguimiento del paciente con vasculitis asociada a ANCA, incluyendo situaciones especiales. El objetivo es brindar a los médicos que manejan habitualmente este tipo de enfermedades, información actualizada y recomendaciones clínicas bien fundamentadas, que mejoren el enfoque diagnóstico y terapéutico de nuestros pacientes.