IntroductionDue to the rarity of the condition, optimal assessment and therapeutic strategies to manage coronary arteritis in Takayasu’s arteritis (TAK) have not been well defined.MethodsCases of coronary arteritis were identified within an ongoing single-center prospective observational cohort study in TAK. Patients underwent standardized clinical, imaging, and laboratory assessment per protocol with centralized review of data. Imaging assessment included non-invasive angiography of the aorta and branch vessels, cardiac computed tomographic angiography, cardiovascular magnetic resonance imaging, and positron emission tomography (PET). Cardiac involvement was defined based on demonstration of at least one vasculitic lesion within a coronary artery by an appropriate imaging study.ResultsThe prevalence of coronary arteritis was 13 (9%) out of 137 patients with TAK. Patients with and without coronary arteritis were similar in terms of demographics, angiographic pattern of disease, and non-cardiac clinical symptoms. Vasculitic lesions typically were stenosing and involved the proximal coronary arteries. Active vasculitis by PET in the ascending aorta was associated with active coronary arteritis (sensitivity 100%, specificity 67%). Favorable clinical outcomes were generally achievable but often required medical therapy and vascular intervention. Anti-cytokine medical therapies were likely more effective than cytotoxic therapies. Fifty percent of patients had complications from vascular grafts or stents, respectively, often prompting additional vascular procedures.DiscussionCoronary arteritis is an uncommon complication in TAK. Multimodal imaging can be useful to diagnose, monitor, and manage coronary arteritis. While medical therapy is preferred, vascular intervention may be necessary, and complications from attempts at vascular reperfusion are common.

Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a monogenic disease of adulthood characterised by treatment-refractory systemic inflammation and progressive bone marrow failure. VEXAS syndrome is caused by acquired mutations in the UBA1 gene that are restricted to haematopoietic cells. Men aged 50 years or older are particularly susceptible to VEXAS syndrome, with prevalence estimates of approximately one in 4000 men. Perturbation of UBA1, the master enzyme of cellular ubiquitination, promotes myeloid-driven inflammation that is difficult to control with medications other than glucocorticoids. Cytokine-directed therapies (ie, IL-6 and JAK inhibitors) might temporise symptoms and allow glucocorticoid reduction. Hypomethylating agents (ie, azacytidine) can induce clinical and molecular remission in some patients, but are associated with substantial toxicities. Haematopoietic cell transplant might be effective treatment in patients who are suitable candidates. The discovery of VEXAS syndrome highlights the potential role of somatic mutations in complex inflammatory diseases.

OBJECTIVE:Relapsing polychondritis (RP) is a rare disease defined by recurrent cartilaginous inflammation. Anti-collagen II (Col2) antibodies have been proposed as a diagnostic biomarker for RP, but their performance characteristics are not well defined. METHODS:In an observational cohort, anti-Col2 antibody levels were measured in patients with RP compared to other inflammatory diseases that mimic RP. In parallel, a systematic review and meta-analysis was performed to assess anti-Col2 antibody prevalence across a broad spectrum of diseases. Individual study risk ratios (RRs) and pooled disease category RRs, study bias, and interstudy heterogeneity were assessed. RESULTS:In the observational cohort, anti-Col2 antibody prevalence did not differ between RP and comparators. The performance characteristics of anti-Col2 to diagnose RP were poor (sensitivity = 18%; specificity = 72%). Anti-Col2 antibody titers did not correlate with disease activity in RP (r = 0.08, P = 0.44). In the systematic review, 71 of 2,443 reviewed articles were included. Anti-Col2 antibodies were not associated with RP across five pooled studies (RR: 2.09; 95% confidence interval [CI]: 0.05-81.80; P = 0.69). Anti-Col2 antibodies were significantly associated with a composite group of inflammatory diseases with cartilaginous involvement (RR: 2.99; 95% CI: 1.29-6.91; P = 0.01). Studies using healthy controls reported increased effect sizes compared to studies that used disease controls (β-estimate = 1.14, I2 = 17.08%; P = 0.0004). CONCLUSION:Anti-Col2 antibodies are neither sensitive nor specific for RP, are detected in the minority of patients with RP, and are detected at similar prevalences across a spectrum of inflammatory diseases with cartilage inflammation. Use of these antibodies to diagnose or monitor RP is not advisable.

Based on the Chapel Hill consensus criteria, the primary forms of vasculitis are classified by the predominant size of the affected blood vessels into large, medium, or small vessels. The two main forms of large vessel vasculitis (LVV) are giant cell arteritis and Takayasu arteritis, both of which are more prevalent in women while showing distinct geographic patterns of prevalence. The pathogenesis of LVV is complex and multifactorial, involving a combination of genetic predisposition, environmental and geographic triggers, immune dysregulation, and aging or (premature) senescence of the immune system and blood vessels. Diagnosis based on clinical presentation alone can be challenging because of the wide variety and often nonspecific symptoms that are associated with LVV. This has prompted the development of novel diagnostic tools to aid patient management, in particular advanced vascular imaging approaches. New therapies targeting specific immune pathways are now becoming available to improve outcomes while limiting the side effects associated with traditional glucocorticoid treatment. Advances in molecular imaging techniques may also enhance our ability to objectively monitor disease and treatment response in patients with LVV. Ongoing research aims to better understand the underlying mechanisms and to develop better targeted therapies for LVV, and to improve patient assessment and life time management. This narrative review will provide an in-depth update on current challenges and future trends in LVV, enhancing our understanding of its pathogenesis, diagnostic features, and management strategies, including disease- and treatment-related cardiovascular complications.

Inflammatory bowel disease (IBD) causes chronic suffering from gastrointestinal inflammation and dysfunction that can progress to colon cancer1,2. The prevalence of the disease is increasing, and there is an urgent need to better understand its pathogenic mechanisms to improve treatment. We show that GPR15-a G-protein-coupled receptor expressed in immune cells and described previously as an entry co-factor for human and simian immunodeficiency viruses3-is a marker and homing receptor for a subset of intramucosal GPR15-guided regulatory CD8+ T lymphocytes (CD8+ TIGR cells). Deleterious GPR15 gene variants in humans cause defective homing of CD8+ TIGR cells and are associated with severe early-onset IBD. Moreover, CD8+ TIGR cells are reduced in the intestinal mucosa of individuals with sporadic IBD. In mice, GPR15 deficiency impairs colonic homing of CD8+ TIGR cells, leading to accumulation of inflammatory macrophages and increased susceptibility to colitis. CD8+ TIGR cells potently kill macrophages activated by intestinal damage or disease using Fas ligand and TNF-related weak inducer of apoptosis (TWEAK). The identification of CD8+ TIGR cells yields new insights into organ-specific immune regulation and potential therapeutics for IBD.

Objective : To identify the most important and relevant items to consider when developing criteria to measure response to treatment in giant cell arteritis (GCA). Methods : As part of an ongoing project to develop response criteria for GCA, a 4-round web-based Delphi exercise was conducted. Participants included patients with GCA and health professionals with expertise in GCA. Participants rated the importance (1=lowest, 9=highest) of 51 items selected based on a systematic literature review, grouped into six domains, and could suggest additional items. Items scored 7-9 by at least 70% of health professionals and 70% of patients were considered critically important and to have reached consensus. In the last round of the Delphi, participants ranked the top 10 most relevant items. A task force (n=32 members) meeting followed to review the Delphi results, discuss rankings, and finalize the selection of items. Results : One hundred eighty-seven physicians and 85 patients, from 38 countries, participated in the Delphi exercise. Twenty-four (75%) task force members participated in the virtual meeting. Thirteen new items were proposed in round 1. Twenty-four items were rated as critically important. No items were excluded during any round, and consensus was not reached for 40 items. The top 3 highest rated items by both patients and physicians were headache (ranked highest by 52%), amaurosis fugax (highest=10%), and jaw claudication (highest=7%). Twelve items were selected for the next phase of the project. Conclusion : Experts and patients identified 12 items considered important for measuring response to treatment in GCA.

OBJECTIVE:Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome (VEXAS) syndrome is characterized by a complex spectrum of inflammatory and hematologic manifestations. Clinical research to identify effective therapies is urgently needed but is hindered by the lack of validated outcome measures. A VEXAS-specific disease activity index (DAI) is an essential tool for reliably capturing changes in disease activity over time, thereby supporting clinical research and informing patient care in this emerging disease. This study aimed to develop a comprehensive DAI to assess disease-related inflammation across affected organ systems in patients with VEXAS syndrome. METHODS:Inflammatory manifestations of VEXAS were previously identified through a systematic literature review and expert input. A multinational expert advisory committee developed the VEXAS-DAI using modified Delphi methodology until consensus (defined as ≥75% concurrence) was reached. Item grading was adapted from the Common Terminology Criteria for Adverse Events. Scoring criteria were developed based on 158 test cases using the Physician Global Assessment of Inflammation. RESULTS:Consensus on the VEXAS-DAI was achieved after four Delphi rounds. The index assesses inflammatory activity at the time of evaluation across 12 domains: inflammatory rash, chondritis, periorbital, ophthalmologic, joint, pulmonary, cardiovascular, genitourinary, neurologic, oral and gastrointestinal, renal, and constitutional symptoms. The total score ranges from 0 to 40. CONCLUSION:The VEXAS-DAI is a novel instrument designed to quantify active inflammation over time in patients with VEXAS. Prospective validation within a clinical trial setting is ongoing.

OBJECTIVE:To compare fluorodeoxyglucose (FDG)-positron emission tomography (PET) imaging to clinical- and laboratory-based assessments of disease activity in a randomized controlled trial (RCT) in giant cell arteritis (GCA). METHODS:Patients with new-onset or relapsing GCA were randomized to guselkumab or placebo plus tapered glucocorticoids in an international, multicenter RCT. FDG-PET was performed at the baseline visit before randomization and repeated at disease flare or the week-52 visit (clinical remission off glucocorticoids). FDG-PET scans were interpreted as active or inactive by two readers, and the PET Vascular Activity Score was calculated to quantify arterial FDG uptake. FDG-PET findings were compared to clinical assessment, acute phase reactants, and glucocorticoid use at each visit. RESULTS:Baseline FDG-PET scans were interpreted as active vasculitis in 28 (55%) of 51 patients. FDG-PET activity at enrollment was not associated with clinical symptoms, acute phase reactants, or risk of flare. Younger age (70 vs 76 years; P = 0.03) and female sex (89% vs 48%, P < 0.01) were significantly associated with increased FDG-PET activity. There were no differences in prior glucocorticoid dose (median 780 mg) or duration (median 23 days) between patients with baseline active versus inactive FDG-PET scans. FDG-PET scans were active in 17 of 21 (81%) patients during clinical flare and in 14 of 20 (70%) patients at week 52. Subsets of patients had persistently active (n = 21) or inactive (n = 9) PET scans at all time points, independent of clinical assessment. CONCLUSION:Vascular FDG-PET activity may be discordant with clinical assessment, particularly in subsets of patients with GCA. Imaging-based outcome measures should remain exploratory in future therapeutic trials.

OBJECTIVES:Guselkumab, a monoclonal antibody, selectively targets the p19 subunit of interleukin-23. This randomised, double-blind, placebo-controlled, phase 2 study evaluated guselkumab vs placebo for the treatment of giant cell arteritis (GCA). METHODS:Patients ≥50 years of age with new-onset or relapsing GCA were randomised 2:1 to guselkumab or placebo. Both arms received background glucocorticoid (GC) therapy, with a protocol-defined taper through week 26. The primary endpoint was the proportion of patients achieving GC-free remission at week 28. RESULTS:Thirty-five patients were randomised to receive guselkumab and 18 to receive placebo. All patients were White, 70% were female, and the mean age was 71.5 years; 60% had new-onset and 40% had relapsing GCA. At week 28, 40% (14/35) and 33% (6/18) of patients in the guselkumab and placebo groups, respectively, achieved GC-free remission (P = .64), whereas 31% (11/35) and 39% (7/18) had experienced a GCA flare or discontinued due to worsening GCA. Median time to first GCA flare through week 28 was not estimable (NE) in the guselkumab group (90% CI: 27.7-NE) and 29.7 weeks (90% CI: 20.1-NE) in the placebo group (P = .64). Through week 60, 97% (34/35) and 94% (17/18) of patients in the guselkumab and placebo groups, respectively, had adverse events (AEs); the most common AEs, aside from worsening of GCA (49% and 56%, respectively), were COVID-19 infection (23% and 28%) and headache (17% and 39%). CONCLUSIONS:The study's primary endpoint (GC-free remission) was not met; results do not support the use of guselkumab in the treatment of GCA.

Objectives: Takayasu arteritis is a rare vasculitis characterized by inflammation of large arteries. Given the robust genetic association with HLA-B*52:01, it was recently proposed to classify Takayasu arteritis within the spectrum of MHC-I-opathies. Although genetic associations in ERAP1 and ERAP2 have been described in several MHC-I-opathies, their role in Takayasu arteritis remains unclear. This study investigated the genetic interaction between ERAP1/ERAP2 and HLA-B*52:01 in Takayasu arteritis. Methods: Using data from a large multi-ancestral GWAS, we examined the genetic association between ERAP1 and ERAP2 polymorphisms with Takayasu arteritis. Next, we conducted genetic interaction analyses between ERAP1/ERAP2 polymorphisms and HLA-B*52:01, followed by a meta-analysis across populations. In addition, we conducted a genetic association test for ERAP1/ERAP2 polymorphisms restricted to HLA-B*52:01-positive individuals. Results: Our analyses suggested no significant genetic interactions between ERAP1 or ERAP2 and HLA-B*52:01 in Takayasu arteritis, which contrasts with the findings observed in other MHC-I-opathies. In addition, no significant associations between ERAP1/ERAP2 and Takayasu arteritis were detected, including analyses under dominant or recessive models, or after stratifying by HLA-B*52:01 status Conclusion: These results suggest that the genetic profile of Takayasu arteritis differs from other MHC-I-opathies, supporting its classification as a distinct subtype within this group of immune-mediated diseases. These finding are hypothesis-generating, and validation in larger cohorts is warranted.

OBJECTIVE:The efficacy of nucleic acid-based vaccines against SARS-CoV-2 varies across individuals, partly due to genetic factors influencing neutralizing antibody production. In patients with systemic autoimmune diseases (SADs), this response may be further altered by immune dysregulation. METHODS:We conducted a genome-wide association study (GWAS) to identify genetic variants associated with postvaccination anti-SARS-CoV-2 IgG antibody levels and to assess whether these associations differ between patients with SAD and healthy individuals. RESULTS:The study included 165 participants (138 with SADs, 27 healthy controls), all of whom received nucleic acid-based vaccines. Antibody levels targeting the spike protein receptor-binding domain (RBD) and nucleocapsid were measured between 1 and 12 months after vaccination. GWAS results were metaanalyzed with data from a previously published GWAS with 1076 healthy individuals. We identified a novel association near RACGAP1 (rs706785; βmeta = -0.30, P meta = 3.85 × 10-8) and replicated a known association at HLA-DRB1 position 71 (βmeta = -0.23, P meta = 1.94 × 10-11). No significant interactions were observed between genotype and disease status. CONCLUSION:This study highlights both MHC and non-MHC genetic contributions to SARS-CoV-2 vaccine responses and suggests these effects are consistent across patients with SADs and healthy individuals, supporting standard vaccination strategies for individuals with systemic autoimmune conditions.

Large vessel vasculitis (LVV) comprises inflammatory disorders that primarily affect large arteries, most notably Takayasu arteritis (TAK) and giant cell arteritis (GCA). GCA frequently coexists with polymyalgia rheumatica (PMR), reflecting a shared disease spectrum. [18F]FDG-PET/CT is an established imaging modality and has become integral to the evaluation of these conditions. When performed alone or combined with CT angiography ([18F]FDG-PET/CT(A)), it enables visualization of metabolic activity in inflamed arterial walls as well as characteristic periarticular involvement. Since publication of the previous procedural recommendations, substantial new evidence has emerged, prompting an update of guidance. This joint guideline, developed by EANM, SNMMI, ASNC, CANM, CSNM and ANZSNM, provides updated evidence-based recommendations and expert consensus on the use of [18F]FDG-PET/CT(A) in LVV and PMR. It addresses patient preparation, tracer administration, image acquisition, interpretation, reporting, and radiation considerations, with a strong emphasis on standardization to improve diagnostic accuracy and reproducibility. The guideline highlights the impact of glucocorticoid therapy on PET sensitivity and provides disease-specific interpretation criteria for large-vessel (lvGCA) and cranial GCA (cGCA), TAK, and PMR, including validated visual grading systems and composite scores. Systematic reviews and meta-analyses demonstrate moderate to high diagnostic accuracy of [18F]FDG-PET/CT in LVV and PMR. Current evidence does not support routine PET imaging for treatment monitoring or relapse prediction. However, [18F]FDG-PET/CT is valuable for identifying vascular territories at risk of future structural damage. The guideline also reviews emerging developments, including PET/MRI, long axial field-of-view PET/CT systems, and novel radiotracers, and underscores the importance of training and harmonized practice. Overall, these updated procedural recommendations aim to strengthen international harmonization and establish robust standards for [18F]FDG-PET/CT(A) imaging in LVV and PMR, supporting clinical decision-making and guiding future research.

BACKGROUND:Prior studies identified subsets of patients with Takayasu arteritis (TAK) based on angiographic patterns of disease in cohorts from India and North America. This study aimed to validate these patterns in a TAK cohort from Turkey and determine the role of genetics in arterial patterns. METHODS:421 Turkish patients with TAK underwent angiography of the aorta and branch vessels, with disease involvement characterized in 13 arterial territories. K-means cluster analysis identified angiographically-based subgroups. 282 patients with TAK from Turkey and 115 European-American patients from North America were genotyped. Approximately 6.5 million SNPs were evaluated in a meta-analysis of both cohorts. Logistic regressions identified genetic associations with angiographic clusters (threshold for association: p-value<1x10-5). Associated variants were functionally annotated. RESULTS:Three clusters were identified in Turkish patients, validating the previously-identified cluster pattern. Genome-wide meta-analyses revealed a locus in the solute carrier family gene SLC24A2 in Cluster One as the most significant association (rs2891138, OR = 3.34, p-value = 2.32x10-7). Several genetic loci were associated with Cluster Two, including in LGALSL (rs883021, OR = 0.43, p-value = 1.00X10-5), AK4P3 (rs1072778, OR = 0.39, p-value = 4.07X10-6), and TMEM132B (rs4765045, OR = 3.05, p-value = 6.18X10-6). The most significant locus associated with Cluster Three was in FRMD6 (rs55692665, OR = 2.79, p-value = 1.11X10-7). Genetic effects were consistent between the cohorts. Several loci were associated with levels of mRNA expression in arterial tissues. A Cluster Two-associated variant in APBB2 (rs2465578, OR = 0.35, p-value = 6.69x10-6) showed evidence for chromatin interaction with the NSUN7 promoter and increased aortic NSUN7 expression. CONCLUSIONS:Genetic factors are associated with distinct subsets of TAK defined by angiographic pattern of disease.

Giant cell arteritis (GCA) and Takayasu’s arteritis (TAK) are the two main forms of large-vessel vasculitis (LVV), defined by inflammation of the aorta and its primary branches. Use of vascular imaging, including FDG-PET, has been increasingly incorporated into the assessment of patients with LVV. FDG-PET detects metabolic activity in the walls of the large arteries as a surrogate for vascular inflammation. In this article we review the use of FDG-PET to diagnose and monitor disease activity in different forms of LVV. Use of FDG-PET to diagnose GCA by assessing vascular FDG uptake in the aorta and branch arteries is well-established. More recently, newer generation PET/CT scanners have also been used to assess metabolic activity in the cranial arteries, including the temporal arteries. In TAK, non-invasive angiography is used to assess for luminal damage at diagnosis, while FDG-PET can provide complementary information about whether active vascular inflammation is present. Recent studies have focused on the use of FDG-PET to monitor disease activity in LVV and the prognostic value of FDG-PET scans. Use FDG-PET in LVV remains an area of active ongoing research. While use at time of diagnosis in LVV has become well established, more studies are needed to evaluate the prognostic value of FDG-PET when monitoring disease activity in patients with LVV. Additional future directions for use of FDG-PET in LVV include employment of novel radiotracers, use of newer generation PET scanners, and incorporation into clinical trials to assess treatment response at the vascular level.

Many monogenic autoinflammatory diseases, including DADA2 (deficiency of adenosine deaminase 2), HA20 (haploinsufficiency of A20), SAVI (STING-associated vasculopathy with onset in infancy), COPA syndrome, LAVLI (LYN kinase-associated vasculopathy and liver fibrosis) and VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome, present predominantly with vasculitis and constitute a substantial subgroup of vasculitic conditions associated with a ‘probable aetiology’. The spectrum of monogenic vasculitis encompasses all sizes and types of blood vessel, ranging from large vessels to medium-size and small vessels, and from the arterial side to the venous side of the vasculature. Monogenic vasculitis typically starts early in life during infancy or childhood; VEXAS syndrome, which presents in late adulthood, is an exception. The activation of myeloid cells via inflammasome and nuclear factor-κB pathways, type I interferon-enhanced autoimmune mechanisms and/or dysregulated adaptive immune responses have an important role in the development of immune-mediated endothelial dysfunction and vascular damage. Genetic testing is essential for the diagnosis of underlying monogenic autoinflammatory diseases; however, the penetrance of genetic variants can vary. Increased awareness and recognition of distinctive clinical findings could facilitate earlier diagnosis and allow for more-targeted treatments. This Review discusses the clinical features, pathogenesis, diagnosis and management of monogenic forms of vasculitis. The authors emphasize that increased awareness of these rare diseases could aid earlier diagnosis and better, more-targeted treatment options for patients.

RATIONALE AND OBJECTIVES:Cardiovascular disease (CVD) is closely associated with aortoiliac plaque burden, yet current research on its automated detection and segmentation has largely focused on plaque burden analysis using CT angiography. In this study, we present an automated method for aortoiliac plaque detection and segmentation that enables accurate quantification of calcified plaque burden on both non-contrast and contrast-enhanced CT scans. MATERIALS AND METHODS:The training data included 119 non-contrast whole-body PET-CT scans and 23 contrast-enhanced abdominopelvic CT urography scans, all obtained from our institution. The testing data comprised 99 contrast-enhanced thoracoabdominopelvic CT scans from the sarcopenia dataset; 93 from the prostate cancer dataset; 1214 paired non-contrast and contrast-enhanced abdominal CT scans from a renal donor cohort; 9199 non-contrast abdominal CT colonography scans from a second institution; and 1446 non-contrast chest CT scans from a third institution. The nnU-Net was used to train a model for aortoiliac plaque detection and segmentation. Detection accuracy was evaluated on non-contrast chest CT scans. Segmentation accuracy was assessed on CT scans with manually labeled plaque regions from the sarcopenia, prostate, renal donor, and CT colonography datasets. The correlation between Agatston scores on paired non-contrast and contrast-enhanced scans was evaluated in the renal donor cohort. Correlations between whole-torso calcified plaque burden (Agatston scores), demographics, and diseases were analyzed using multivariable analysis on the CT colonography dataset. RESULTS:Aortoiliac plaques were detected with 88.1% precision, 99.5% recall, and a 93.4% F1 score. Segmentation achieved Dice scores of 64.3-83.7% across two internal contrast-enhanced and two external non-contrast CT datasets, outperforming baseline methods by over 10% (p < 0.001). Agatston scores from paired CT scans showed strong correlation (R2 = 0.99). Multivariate analysis showed calcified plaque burden assessment correlated with sex, age, BMI, and smoking (all p < 0.001), as well as alcohol abuse (p = 0.01). The calcified burden assessment was also correlated with CVD, heart failure, myocardial infarction (all p < 0.001), and type 2 diabetes (p = 0.03), but showed no correlation with cancer (p = 0.14) or femoral neck fracture (p = 0.61). CONCLUSION:Automated aortoiliac plaque detection enables accurate whole-torso atherosclerotic calcified burden assessment, offering a potential pathway for improved CVD diagnosis and treatment.

Infections are increasingly recognised as a major cause of morbidity and mortality in patients with vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome. We conducted a systematic review to characterise the infectious burden of VEXAS syndrome and propose preventive strategies. We included 57 studies (813 patients) showing that infections in patients with VEXAS syndrome were frequent, severe in 40-60% of cases, and fatal in 6-15% of cases. Pulmonary infections were most common, followed by cutaneous infections and bacteraemia. Opportunistic pathogens, such as Pneumocystis jirovecii, Legionella pneumophila, non-tuberculous mycobacteria, and varicella zoster virus, were frequently reported, even in patients not receiving immunosuppressive therapy, which suggests intrinsic immune dysfunction. Prophylaxis with co-trimoxazole (or other Pneumocystis prophylaxis, such as atovaquone or pentamidine) and valaciclovir should particularly be considered for patients at high risk of infection, including those receiving immunosuppressive therapy and those with lymphopenia, pMet41Val mutation, or previous severe or recurrent infections. Posaconazole might be appropriate in patients with neutropenia who are taking azacitidine. Vaccination against Streptococcus pneumoniae, varicella zoster virus, influenza, and SARS-CoV-2 is recommended. These data highlight the need to integrate infectious risk into VEXAS syndrome management and to evaluate preventive strategies in prospective studies.

Atherosclerosis, characterized by plaque buildup in arterial walls, is often studied in coronary arteries. However, it is less studied in the aorta and iliac arteries, despite their links with diseases like ischemic heart disease, stroke, and periph-eral vascular conditions. This work develops an automated method to segment and label plaques in the aorta, left, and right iliac arteries. Using an nnU-Net-based segmentation framework, plaques, arteries, and organs that potentially in-duce false plaque detections are first segmented. Plaques are then labeled according to the arteries in which they are located. Agatston scores are calculated on labeled plaques to assess atherosclerosis burden. The proposed method was validated using 50 internal contrast-enhanced and 60 external non-contrast CT scans. For both internal and external datasets, the plaque burdens computed from the automated segmentation were found to be strongly correlated with those computed from manual annotations $(R^{2} > 0.8$, except for the left iliac artery in the external dataset). These results sug-gest the potential of automated plaque labeling method for atherosclerotic plaque burden assessment in clinical usage.