Characterizing infection in anti-neutrophil cytoplasmic antibody-associated vasculitis:results from a longitudinal, matched-cohort data linkage study

We wish to thank Information Division Services Scotland for assisting with data linkage and data access in the National Safe Haven. Information presented in this article was previously presented as a poster at the American College of Rheumatology Annual Conference 2018, Chicago, IL, USA. The study was conceived by S.H.S., A.M., C.B. and N.B. All authors contributed to the study design and data collection. Data analysis and interpretation and drafting of the manuscript were conducted by all authors. C.B. and N.B. were joint senior authors. All authors critically reviewed the manuscript and approved the final version. Funding: S.H.S. and the study were funded by the Aberdeen Development Trust and the Farr Institute of Health Informatics Research. The Farr Institute is supported by a 10-funder consortium: Arthritis Research UK, the British Heart Foundation, Cancer Research UK, the Economic and Social Research Council, the Engineering and Physical Sciences Research Council, the Medical Research Council, the National Institute of Health Research, the National Institute for Social Care and Health Research (Welsh Assembly Government), the Chief Scientist Office (Scottish Government Health Directorates) and the Wellcome Trust (Scotland MR/K007017/1). Disclosure statement: L.E. is a GlaxoSmithKline employee. The other authors have declared no conflicts of interest.Peer reviewedPublisher PD

ImmPort, toward repurposing of open access immunological assay data for translational and clinical research

Immunology researchers are beginning to explore the possibilities of reproducibility, reuse and secondary analyses of immunology data. Open-access datasets are being applied in the validation of the methods used in the original studies, leveraging studies for meta-analysis, or generating new hypotheses. To promote these goals, the ImmPort data repository was created for the broader research community to explore the wide spectrum of clinical and basic research data and associated findings. The ImmPort ecosystem consists of four components–Private Data, Shared Data, Data Analysis, and Resources—for data archiving, dissemination, analyses, and reuse. To date, more than 300 studies have been made freely available through the ImmPort Shared Data portal , which allows research data to be repurposed to accelerate the translation of new insights into discoveries

Outcomes of COVID-19 in patients with primary systemic vasculitis or polymyalgia rheumatica from the COVID-19 Global Rheumatology Alliance physician registry: a retrospective cohort study

BACKGROUND: Patients with primary systemic vasculitis or polymyalgia rheumatica might be at a high risk for poor COVID-19 outcomes due to the treatments used, the potential organ damage cause by primary systemic vasculitis, and the demographic factors associated with these conditions. We therefore aimed to investigate factors associated with COVID-19 outcomes in patients with primary systemic vasculitis or polymyalgia rheumatica. METHODS: In this retrospective cohort study, adult patients (aged ≥18 years) diagnosed with COVID-19 between March 12, 2020, and April 12, 2021, who had a history of primary systemic vasculitis (antineutrophil cytoplasmic antibody [ANCA]-associated vasculitis, giant cell arteritis, Behçet's syndrome, or other vasculitis) or polymyalgia rheumatica, and were reported to the COVID-19 Global Rheumatology Alliance registry were included. To assess COVID-19 outcomes in patients, we used an ordinal COVID-19 severity scale, defined as: (1) no hospitalisation; (2) hospitalisation without supplemental oxygen; (3) hospitalisation with any supplemental oxygen or ventilation; or (4) death. Multivariable ordinal logistic regression analyses were used to estimate odds ratios (ORs), adjusting for age, sex, time period, number of comorbidities, smoking status, obesity, glucocorticoid use, disease activity, region, and medication category. Analyses were also stratified by type of rheumatic disease. FINDINGS: Of 1202 eligible patients identified in the registry, 733 (61·0%) were women and 469 (39·0%) were men, and their mean age was 63·8 years (SD 17·1). A total of 374 (31·1%) patients had polymyalgia rheumatica, 353 (29·4%) had ANCA-associated vasculitis, 183 (15·2%) had giant cell arteritis, 112 (9·3%) had Behçet's syndrome, and 180 (15·0%) had other vasculitis. Of 1020 (84·9%) patients with outcome data, 512 (50·2%) were not hospitalised, 114 (11·2%) were hospitalised and did not receive supplemental oxygen, 239 (23·4%) were hospitalised and received ventilation or supplemental oxygen, and 155 (15·2%) died. A higher odds of poor COVID-19 outcomes were observed in patients who were older (per each additional decade of life OR 1·44 [95% CI 1·31–1·57]), were male compared with female (1·38 [1·05–1·80]), had more comorbidities (per each additional comorbidity 1·39 [1·23–1·58]), were taking 10 mg/day or more of prednisolone compared with none (2·14 [1·50–3·04]), or had moderate, or high or severe disease activity compared with those who had disease remission or low disease activity (2·12 [1·49–3·02]). Risk factors varied among different disease subtypes. INTERPRETATION: Among patients with primary systemic vasculitis and polymyalgia rheumatica, severe COVID-19 outcomes were associated with variable and largely unmodifiable risk factors, such as age, sex, and number of comorbidities, as well as treatments, including high-dose glucocorticoids. Our results could be used to inform mitigation strategies for patients with these diseases. FUNDING: American College of Rheumatology and the European Alliance of Associations for Rheumatology

Outcomes of COVID-19 in patients with primary systemic vasculitis or polymyalgia rheumatica from the COVID-19 Global Rheumatology Alliance physician registry : a retrospective cohort study

Funding Information: This study was funded by the American College of Rheumatology and the European League Against Rheumatism. The views expressed herein are those of the authors and participating members of the COVID-19 Global Rheumatology Alliance and do not necessarily represent the views of the American College of Rheumatology, the European Alliance of Associations for Rheumatology, the UK National Health Service, the NIHR, the UK Department of Health, or any other organisation. Patient research partners (KB and LN) were involved in the design, conduct, reporting and interpretation of the results of this study. Patient partners have participated in the development of this manuscript and are listed as coauthors. Funding Information: SES reports funding from a Vasculitis Clinical Research Consortium (VCRC)–Vasculitis Foundation Fellowship (the VCRC is part of the Rare Diseases Clinical Research Network, an initiative of the Office of Rare Diseases Research, National Center for Advancing Translational Science [NCATS], and is funded by a collaboration between NCATS and the National Institute of Arthritis and Musculoskeletal and Skin Diseases [NIAMS; U54 AR057319]). RC reports speaker's fees from Janssen, Roche, Sanofi, and Abbvie outside the submitted work. CH received funding under a sponsored research agreement from Vifor Pharmaceuticals, outside the submitted work. SLM has received consulting fees from AbbVie; consulting fees from AstraZeneca; other from Roche-Chugai; consulting fees from Sanofi; and non-financial support from Roche, all outside the submitted work; and is a patron of the patient charity PMRGCAuk. PM is a Medical Research Council-GlaxoSmithKline (MRC-GSK) EMINENT clinical training fellow, who has received project funding from this organisation, outside the submitted work; has received funding from the National Institute for Health Research (NIHR) University College London Hospitals Biomedical Research Centre (UCLH BRC); reports grants from MRC-GSK; reports personal fees from Swedish Orphan Biovitrum and Lilly; and reports consultancy fees from Abbvie and Pfizer, all outside the submitted work. LN reports being a trustee of the charity PMR-GRA Scotland. JSA reports grants from the National Institute of Health (NIH) and NIAMS, the Rheumatology Research Foundation, the Brigham Research Institute, the R Bruce and Joan M Mickey Research Scholar Fund, and Amgen; grants and personal fees from Bristol-Myers Squibb; and personal fees from Gilead, Inova, Janssen, Optum, and Pfizer, all outside the submitted work. AD-G is supported by the US Centers for Disease Control and Prevention, the Rheumatology Research Foundation Scientist Development Award, the Robert D and Patricia E Kern Center for the Science of Health Care Delivery, and the Women's Health Career Enhancement Award outside the submitted work. KLH reports receiving speaker's fees from Abbvie; grant income from Bristol-Myers Squibb, UCB Pharma, and Pfizer, all outside the submitted work; and is supported by the NIHR Manchester Biomedical Research Centre outside the submitted work. RG reports non-financial support from Pfizer Australia and Janssen Australia; and personal fees from Pfizer Australia, Cornerstones, Janssen New Zealand, and Novartis, all outside the submitted work. UM-L is supported by grants from the German Ministry of Research and Education and the German Research Foundation outside the submitted work. MAG reports funding from the NIH and the NIAMS. PCR reports personal fees from Abbvie and Gilead; grants and personal fees from Janssen, Novartis, UCB Pharma, and Pfizer; non-financial support from Bristol-Myers Squibb and Pfizer; and personal fees from Lilly and Roche, all outside the submitted work. JY reports no competing interests related to this work; is supported by grants from NIH (K24 AR074534 and P30 AR070155); and reports consulting fees from Eli Lilly, Pfizer, Aurinia, and AstraZeneca, all outside the submitted work. PMM has received consulting or speaker's fees from Abbvie, Bristol-Myers Squibb, Celgene, Eli Lilly, Galapagos, Janssen, Merck Sharp & Dohme, Novartis, Pfizer, Roche, and UCB Pharma, all outside the submitted work (all <$10 000); and is supported by the NIHR UCLH BRC outside the submitted work. ES is a board member of the Canadian Arthritis Patient Alliance, which is a patient-run, volunteer-based organisation, whose activities are largely supported by independent grants from pharmaceutical companies. JWL reports grants from Pfizer, outside the submitted work. JSH reports no competing interests related to this work; is supported by grants from the Rheumatology Research Foundation; receives salary support from the Childhood Arthritis and Rheumatology Research Alliance; and reports consulting fees for Novartis, Swedish Orphan Biovitrum, and Biogen, all outside the submitted work (<$10 000). PS reports no competing interests related to this work, but reports receiving honorarium for editing social media for the American College of Rheumatology journals (<$10 000). SBh reports receiving non-branded consulting fees from AbbVie, Amgen, Horizon, Novartis, and Pfizer (<$10 000 from each)outside the submitted work. ZSW reports receiving grant support from Bristol-Myers Squibb and Principia-Sanofi; has consulted for Viela Bio and MedPace; and is supported by grants from the National Institutes of Health, all outside the submitted work. AS reports personal fees for lectures from AbbVie, Celltrion, Lilly, Merck Sharp & Dohme, Roche, Bristol-Myers Squibb, and Pfizer outside the submitted work. EFM has received grants from Abbvie, Novartis, Lilly Portugal, Amgen Biofarmacêutica, Grünenthal SA, Merck Sharp & Dohme, Medac, and A Menarini Portugal-Farmacêutica SA; grants and non-financial support from Pfizer; and non-financial support from Grünenthal, all outside the submitted work. LG reports research grants from Amgen, Galapagos, Janssen, Lilly, Pfizer, Sandoz, and Sanofi; consulting fees from AbbVie, Amgen, Bristol-Myers Squibb, Biogen, Celgene, Galapagos, Gilead, Janssen, Lilly, Novartis, Pfizer, Samsung Bioepis, Sanofi-Aventis, and UCB Pharma, all outside the submitted work. LC declares no competing interests related to this study, but her institute works by contract for laboratories among other institutions, such as Abbvie Spain, Eisai, Gebro Pharma, Merck Sharp & Dohme España SA, Novartis Farmaceutica, Pfizer, Roche Farma, Sanofi Aventis, Astellas Pharma, Actelion Pharmaceuticals España, Grünenthal, and UCB Pharma. NJP reports grants from NIH during the conduct of the study. MU-G reports grants from Pfizer and Janssen, outside the submitted work. SBa reports grants and personal fees from Alexion Pharma, outside the submitted work. RV reports grants from Novartis, Pfizer, and Bristol-Myers Squibb, outside the submitted work. All other authors declare no competing interests. Funding Information: This study was funded by the American College of Rheumatology and the European League Against Rheumatism. The views expressed herein are those of the authors and participating members of the COVID-19 Global Rheumatology Alliance and do not necessarily represent the views of the American College of Rheumatology, the European Alliance of Associations for Rheumatology, the UK National Health Service, the NIHR, the UK Department of Health, or any other organisation. Patient research partners (KB and LN) were involved in the design, conduct, reporting and interpretation of the results of this study. Patient partners have participated in the development of this manuscript and are listed as coauthors. Publisher Copyright: © 2021 Elsevier LtdBackground: Patients with primary systemic vasculitis or polymyalgia rheumatica might be at a high risk for poor COVID-19 outcomes due to the treatments used, the potential organ damage cause by primary systemic vasculitis, and the demographic factors associated with these conditions. We therefore aimed to investigate factors associated with COVID-19 outcomes in patients with primary systemic vasculitis or polymyalgia rheumatica. Methods: In this retrospective cohort study, adult patients (aged ≥18 years) diagnosed with COVID-19 between March 12, 2020, and April 12, 2021, who had a history of primary systemic vasculitis (antineutrophil cytoplasmic antibody [ANCA]-associated vasculitis, giant cell arteritis, Behçet's syndrome, or other vasculitis) or polymyalgia rheumatica, and were reported to the COVID-19 Global Rheumatology Alliance registry were included. To assess COVID-19 outcomes in patients, we used an ordinal COVID-19 severity scale, defined as: (1) no hospitalisation; (2) hospitalisation without supplemental oxygen; (3) hospitalisation with any supplemental oxygen or ventilation; or (4) death. Multivariable ordinal logistic regression analyses were used to estimate odds ratios (ORs), adjusting for age, sex, time period, number of comorbidities, smoking status, obesity, glucocorticoid use, disease activity, region, and medication category. Analyses were also stratified by type of rheumatic disease. Findings: Of 1202 eligible patients identified in the registry, 733 (61·0%) were women and 469 (39·0%) were men, and their mean age was 63·8 years (SD 17·1). A total of 374 (31·1%) patients had polymyalgia rheumatica, 353 (29·4%) had ANCA-associated vasculitis, 183 (15·2%) had giant cell arteritis, 112 (9·3%) had Behçet's syndrome, and 180 (15·0%) had other vasculitis. Of 1020 (84·9%) patients with outcome data, 512 (50·2%) were not hospitalised, 114 (11·2%) were hospitalised and did not receive supplemental oxygen, 239 (23·4%) were hospitalised and received ventilation or supplemental oxygen, and 155 (15·2%) died. A higher odds of poor COVID-19 outcomes were observed in patients who were older (per each additional decade of life OR 1·44 [95% CI 1·31–1·57]), were male compared with female (1·38 [1·05–1·80]), had more comorbidities (per each additional comorbidity 1·39 [1·23–1·58]), were taking 10 mg/day or more of prednisolone compared with none (2·14 [1·50–3·04]), or had moderate, or high or severe disease activity compared with those who had disease remission or low disease activity (2·12 [1·49–3·02]). Risk factors varied among different disease subtypes. Interpretation: Among patients with primary systemic vasculitis and polymyalgia rheumatica, severe COVID-19 outcomes were associated with variable and largely unmodifiable risk factors, such as age, sex, and number of comorbidities, as well as treatments, including high-dose glucocorticoids. Our results could be used to inform mitigation strategies for patients with these diseases. Funding: American College of Rheumatology and the European Alliance of Associations for Rheumatology.publishersversionPeer reviewe

Patient-powered research networks: building capacity for conducting patient-centered clinical outcomes research.

The Patient-Centered Outcomes Research Institute (PCORI) recently launched PCORnet to establish a single inter-operable multicenter data research network that will support observational research and randomized clinical trials. This paper provides an overview of the patient-powered research networks (PPRNs), networks of patient organizations focused on a particular health condition that are interested in sharing health information and engaging in research. PPRNs will build on their foundation of trust within the patient communities and draw on their expertise, working with participants to identify true patient-centered outcomes and direct a patient-centered research agenda. The PPRNs will overcome common challenges including enrolling a diverse and representative patient population; engaging patients in governance; designing the data infrastructure; sharing data securely while protecting privacy; prioritizing research questions; scaling small networks into a larger network; and identifying pathways to sustainability. PCORnet will be the first distributed research network to bring PCOR to national scale

What are the informational needs of patients with ANCA Associated Vasculitis? a Mixed Methods Study

Background: The ANCA –associated vasculitides (AAVs) are a group of rare, potentially life-threatening conditions which if untreated can be fatal. Little is known about the information needs of people with AAV. Objectives: To explore what it is like to be diagnosed with AAV and to find out the informational needs of this group. Study design: A mixed methods approach using focus groups and one-to-one interviews, then a questionnaire surveying the membership of Vasculitis UK (VUK) and the Vasculitis Clinical Research Consortium (VCRC). Results: Emergent themes from the first phase were: reaction to diagnosis, need for information on disease management and access to knowledgeable practitioners. There were 314 VUK, 273 VCRC respondents. Respondents rated information on diagnosis, prognosis, investigations, treatment, and side effects as extremely important. Information on patient support groups and psychosocial care was less important. There was no difference in the ratings of information needs based on group, sex, age, disease duration, disease, or method of questionnaire delivery. Conclusion: Receiving the diagnosis of a rare, potentially life-threatening disease causes anxiety and fear and can impede information retention and recall. People with AAV seek specific information concerning their disease, treatment regimes and side effects, and the results of investigations. Individuals preferred to receive this information from a doctor. Recommendations: Patients with AAV should be treated in a similar manner to patients with other chronic illnesses in which patient education is a fundamental part of care

Global epidemiology of vasculitis.

The many forms of vasculitis are characterized by inflammation of blood vessels, leading to potentially long-term sequelae including vision loss, aneurysm formation and kidney failure. Accurate estimation of the incidence and prevalence has been hampered by the absence of reliable diagnostic criteria and the rarity of these conditions; however, much progress has been made over the past two decades, although data are still lacking from many parts of the world including the Indian subcontinent, China, Africa and South America. Giant cell arteritis occurs in those aged 50 years and over and seems to mainly affect persons of northern European ancestry, whereas Takayasu arteritis occurs mainly in those aged under 40 years. By contrast, Kawasaki disease mainly occurs in children aged under 5 years and is most common in children of Asian ancestry, and IgA vasculitis occurs in children and adolescents. Although much less common than giant cell arteritis, the different forms of antineutrophil cytoplasmic antibody-associated vasculitis are being increasingly recognized in most populations and occur more frequently with increasing age. Behçet syndrome occurs most commonly along the ancient silk road between Europe and China. Much work needs to be done to better understand the influence of ethnicity, geographical location, environment and social factors on the development of vasculitis

The European Vasculitis Society 2016 Meeting Report.

The 2016 European Vasculitis Society (EUVAS) meeting, held in Leiden, the Netherlands, was centered around phenotypic subtyping in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). There were parallel meetings of the EUVAS petals, which here report on disease assessment; database; and long-term follow-up, registries, genetics, histology, biomarker studies, and clinical trials. Studies currently conducted will improve our ability to discriminate between different forms of vasculitis. In a project that involves the 10-year follow-up of AAV patients, we are working on retrieving data on patient and renal survival, relapse rate, the cumulative incidence of malignancies, and comorbidities. Across Europe, several vasculitis registries were developed covering over 10,000 registered patients. In the near future, these registries will facilitate clinical research in AAV on a scale hitherto unknown. Current studies on the genetic background of AAV will explore the potential prognostic significance of genetic markers and further refine genetic associations with distinct disease subsets. The histopathological classification of ANCA-associated glomerulonephritis is currently evaluated in light of data coming out of a large international validation study. In our continuous search for biomarkers to predict clinical outcome, promising new markers are important subjects of current research. Over the last 2 decades, a host of clinical trials have provided evidence for refinement of therapeutic regimens. We give an overview of clinical trials currently under development, and consider refractory vasculitis in detail. The goal of EUVAS is to stimulate ongoing research in clinical, serological, and histological management and techniques for patients with systemic vasculitis, with an outlook on the applicability for clinical trials

Autoimmune Diseases and COVID-19 as Risk Factors for Poor Outcomes: Data on 13,940 Hospitalized Patients from the Spanish Nationwide SEMI-COVID-19 Registry

(1) Objectives: To describe the clinical characteristics and clinical course of hospitalized patients with COVID-19 and autoimmune diseases (ADs) compared to the general population. (2) Methods: We used information available in the nationwide Spanish SEMI-COVID-19 Registry, which retrospectively compiles data from the first admission of adult patients with COVID-19. We selected all patients with ADs included in the registry and compared them to the remaining patients. The primary outcome was all-cause mortality during admission, readmission, and subsequent admissions, and secondary outcomes were a composite outcome including the need for intensive care unit (ICU) admission, invasive and non-invasive mechanical ventilation (MV), or death, as well as in-hospital complications. (3) Results: A total of 13,940 patients diagnosed with COVID-19 were included, of which 362 (2.6%) had an AD. Patients with ADs were older, more likely to be female, and had greater comorbidity. On the multivariate logistic regression analysis, which involved the inverse propensity score weighting method, AD as a whole was not associated with an increased risk of any of the outcome variables. Habitual treatment with corticosteroids (CSs), age, Barthel Index score, and comorbidity were associated with poor outcomes. Biological disease-modifying anti-rheumatic drugs (bDMARDs) were associated with a decrease in mortality in patients with AD. (4) Conclusions: The analysis of the SEMI-COVID-19 Registry shows that ADs do not lead to a different prognosis, measured by mortality, complications, or the composite outcome. Considered individually, it seems that some diseases entail a different prognosis than that of the general population. Immunosuppressive/immunoregulatory treatments (IST) prior to admission had variable effects