Arterial and venous thrombosis in systemic and monogenic vasculitis.

Systemic vasculitis, common forms of which include anti-neutrophil cytoplasmic antibody-associated small-vessel vasculitis, large-vessel vasculitis and Behçet syndrome, are frequently complicated by arterial or venous thrombotic events (AVTEs). Newly identified entities such as DADA2 (deficiency of adenosine deaminase 2) and VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome, which are driven by genetic mutations, also exhibit vasculitic features and are associated with a high risk of AVTEs. AVTEs in systemic vasculitis, including monogenic forms of vasculitis, are due to the complex interaction of inflammation and coagulation.

Myocardial infarction in ANCA-associated vasculitis: a population-based cohort study.

Objectives: To determine the incidence rate (IR) and predictors of myocardial infarction (MI) in patients with antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) as well as to estimate the IR ratio (IRR) of MI in AAV versus the background population.

Methods: 325 patients diagnosed with AAV 1997-2016 in Skåne, Sweden were included. Data were collected from the time of AAV diagnosis, and each patient was grouped with 10 age-matched and sex-matched reference subjects from the background population.

Mepolizumab versus benralizumab for eosinophilic granulomatosis with polyangiitis (EGPA): A European real-life retrospective comparative study.

Background: Following the results of the MANDARA trial, this real-life study aimed at comparing the effectiveness and safety profile of mepolizumab versus benralizumab in a European EGPA cohort.

Methods: We conducted a retrospective observational comparative study including EGPA patients, who received mepolizumab or benralizumab at the asthma dose. Patients were matched 1:1 by sex, age, BVAS and oral corticosteroid (OCS) dosage at the treatment initiation (T0).

In-Depth Analysis of Disease Manifestations in Antineutrophil Cytoplasmic Antibody-Associated Vasculitides Identifies Distinct Clinical Phenotypes.

Objective: The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides are heterogeneous disorders. The aim of this study was to identify and characterize subgroups of patients based on sex, ANCA, age at diagnosis, and organ involvement.

Methods: In total, 1,167 patients with granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA) were retrospectively recruited to the study.

Temporal artery biopsy in giant cell arteritis: clinical perspectives and histological patterns.

Although its role has been debated, temporal artery biopsy (TAB) remains the gold standard for the diagnosis of cranial giant cell arteritis (GCA). The specificity of TAB is excellent and the sensitivity, albeit lower, is comparable with other diagnostic modalities used for the diagnosis of GCA. This outpatient procedure has a low rate of complications and is well integrated in the majority of healthcare systems.

The use of intravenous methylprednisolone in giant cell arteritis: a population-based study.

Objectives: To determine clinical characteristics, outcome and occurrence of comorbidities in patients with biopsy-confirmed giant cell arteritis (GCA) treated with intravenous methylprednisolone (IVMP) vs those receiving oral glucocorticoids (OGC) only.

Methods: A retrospective study included patients with GCA diagnosed from 2004 through 2019. Clinical and laboratory characteristics, and cumulative GC dose were compared in patients receiving IVMP vs OGCs.

Data-driven subclassification of ANCA-associated vasculitis: model-based clustering of a federated international cohort.

Background: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a heterogenous autoimmune disease. While traditionally stratified into two conditions, granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), the subclassification of ANCA-associated vasculitis is subject to continued debate. Here we aim to identify phenotypically distinct subgroups and develop a data-driven subclassification of ANCA-associated vasculitis, using a large real-world dataset.

Giant cells in temporal artery biopsies and the risk of large-vessel involvement in patients with giant cell arteritis.

Objectives: Giant cell arteritis (GCA) is a systemic disease with variable vascular involvement. The objective was to investigate predictors of time-dependent large vessel involvement (LVI) in a population-based cohort of patients with GCA.

Methods: GCA patients with positive temporal artery biopsies (TAB) between 1997- 2010 were identified through a regional pathology register.

Classification Criteria for ANCA Associated Vasculitis - Ready for Prime Time?

Purpose Of Review: This review aims to summarize the evolution and recent developments in the classification of ANCA associated vasculitis (AAV) and to summarize evaluations of the 2022 ACR/EULAR classification criteria of AAV in several cohorts.

Recent Findings: The classification of AAV has been a field of controversy for some time. The parallel existence of classification criteria and disease definitions produced some overlap in classification, leading to challenges when comparing different cohorts.

Thyroid disease in ANCA-associated vasculitis: a clinical and epidemiological study.

Objectives: To describe clinical and laboratory characteristics and outcomes in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and thyroid disease (TD). We also aimed to calculate incidence and identify predictors of TD in two large cohorts of patients with AAV.

Methods: The study comprised 644 patients with AAV in a population-based cohort from southern Sweden (n=325) and a cohort from a specialised vasculitis centre in Cambridge, UK (n=319).

Myocardial infarction in a population-based cohort of patients with biopsy-confirmed giant cell arteritis in southern Sweden.

Objectives: To determine the incidence rate (IR) of myocardial infarction (MI), relative risk of MI, and impact of incident MI on mortality in individuals with biopsy-confirmed giant cell arteritis (GCA).

Methods: MIs in individuals diagnosed with GCA 1998-2016 in Skåne, Sweden were identified by searching the SWEDEHEART register, a record of all patients receiving care for MI in a coronary care unit (CCU). The regional diagnosis database, with subsequent case review, identified GCA patients receiving care for MI outside of a CCU.

The HLA region in ANCA-associated vasculitis: characterisation of genetic associations in a Scandinavian patient population.

Objective: The antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are inflammatory disorders with ANCA autoantibodies recognising either proteinase 3 (PR3-AAV) or myeloperoxidase (MPO-AAV). PR3-AAV and MPO-AAV have been associated with distinct loci in the human leucocyte antigen (HLA) region. While the association between MPO-AAV and HLA has been well characterised in East Asian populations where MPO-AAV is more common, studies in populations of European descent are limited.

Epidemiology and clinical characteristics of biopsy-confirmed adult-onset IgA vasculitis in southern Sweden.

Objective: Immunoglobulin A vasculitis (IgAV) is the most prevalent primary childhood vasculitis in Sweden, but is considerably rarer in adults. This study aims to describe the epidemiology, clinical characteristics and renal outcome of adult-onset IgAV in Skåne, Sweden.

Methods: The study area consisted of Skåne, the southernmost region of Sweden, with a population ≥18 years of 990 464 on 31 December 2010.

Associations between plasma metabolism-associated proteins and future development of giant cell arteritis: results from a prospective study.

Objective: The aim of this study was to investigate the relationship between biomarkers associated with metabolism and subsequent development of GCA.

Method: Participants in the population-based Malmö Diet Cancer Study (MDCS; N = 30 447) who were subsequently diagnosed with GCA were identified in a structured process. Matched GCA-free controls were selected from the study cohort.

Apolipoproteins and the risk of giant cell arteritis-a nested case-control study.

Background: The etiology of giant cell arteritis (GCA) and its predictors are incompletely understood. Previous studies have indicated reduced risk of future development of GCA in individuals with obesity and/or diabetes mellitus. There is limited information on blood lipids before the onset of GCA.

Evaluation of the ACR/EULAR 2022 criteria for classification of ANCA-associated vasculitis in a population-based cohort from Sweden.

Objective: To evaluate the ACR/EULAR 2022 criteria for ANCA-associated vasculitides (AAV) classification and compare them with the European Medicines Agency (EMA) algorithm and with classification based only on ANCA serology.

Methods: In the analysis, 374 cases (47% female) were classified according to the EMA algorithm, ANCA serology and ACR/EULAR criteria. The agreement rate was calculated using the kappa (κ) statistic.

Can active sun exposure decrease the risk of giant cell arteritis and polymyalgia rheumatica in women?

Objectives: To study if active sun exposure among women affects the risk of developing GCA or PMR in a prospective cohort study with restricted latitudinal variability.

Methods: We linked the response to questions relating to sun exposure from the Melanoma Inquiry in Southern Sweden (MISS) prospective cohort study in women to the risk of developing GCA or PMR. Healthcare data were gathered from the Skåne Healthcare Register (SHR), covering all public healthcare consultations.

Overcoming challenges in rare disease registry integration using the semantic web - a clinical research perspective.

The growing number of disease-specific patient registries for rare diseases has highlighted the need for registry interoperability and data linkage, leading to large-scale rare disease data integration projects using Semantic Web based solutions. These technologies may be difficult to grasp for rare disease experts, leading to limited involvement by domain expertise in the data integration process. Here, we propose a data integration framework starting from the perspective of the clinical researcher, allowing for purposeful rare disease registry integration driven by clinical research questions.

Stratified genetic analysis reveals sex differences in MPO-ANCA-associated vasculitis.

Objective: To identify and genetically characterize subgroups of patients with ANCA-associated vasculitides (AAV) based on sex and ANCA subtype.

Methods: A previously established SNP dataset derived from DNA sequencing of 1853 genes and genotyping of 1088 Scandinavian cases with AAV and 1589 controls was stratified for sex and ANCA subtype and analysed for association with five top AAV SNPs. rs9274619, a lead variant at the HLA-DQB1/HLA-DQA2 locus previously associated with AAV positive for myeloperoxidase (MPO)-ANCA, was analysed for association with the cumulative disease involvement of ten different organ systems.

EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update.

Background: Since the publication of the EULAR recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in 2016, several randomised clinical trials have been published that have the potential to change clinical care and support the need for an update.

Methods: Using EULAR standardised operating procedures, the EULAR task force undertook a systematic literature review and sought opinion from 20 experts from 16 countries. We modified existing recommendations and created new recommendations.

Stable incidence but increase in prevalence of ANCA-associated vasculitis in southern Sweden: a 23-year study.

Objective: To update the epidemiology of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) in a defined geographical area of southern Sweden.

Methods: The study area comprised 14 municipalities with a combined adult population (≥18 years) of 623 872 in 2019. All cases diagnosed with AAV in 1997-2019 in the study area were included in the estimate of incidence.

Traditional and Disease-Specific Risk Factors for Cardiovascular Events in Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: A Multinational Retrospective Study.

Objective: To investigate the occurrence of cardiovascular events (CVEs) in a large cohort of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) across the European Union, China, Turkey, Russia, the United Kingdom, and the USA.

Methods: Patients with a definite diagnosis of AAV who were followed for ≥ 3 months and had sufficient documentation were included. Data on myocardial infarction (MI) and stroke were collected retrospectively from tertiary vasculitis centers.