Abstracts tagged "Vasculitis"

Abstract Number: 0425 • ACR Convergence 2020
MAGIC Syndrome in a Cohort of Patients with Relapsing Polychondritis
Yiming Luo¹, Emily Rose², Keith Sikora³, Kaitlin Quinn⁴, Kristina Wells⁵, Emily Rominger⁵, Wendy Goodspeed⁶, Peter C. Grayson⁷ and Marcela Ferrada⁶, ¹Rheumatology Fellowship and Training Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, ²Pediatric Translational Research Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethsda, ³Pediatric Translational Research Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, ⁴Systemic Autoimmunity Branch, National Institutes of Health, NIAMS, Bethesda, ⁵Systemic Autoimmunity Branch, Vasculitis Translational Research Program, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, ⁶Systemic Autoimmunity Branch, Vasculitis Translational Research Program, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ⁷Systemic Autoimmunity Branch, National Institutes of Health, NIAMS, Bethesda, MD
Background/Purpose: Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome is an extremely rare condition that encompasses symptoms of relapsing polychondritis (RP) and Behcet’s disease…
Abstract Number: 1423 • ACR Convergence 2020
Declining In-Hospital Mortality in Vasculitis: A 17-year U.S. National Study
Jasvinder Singh¹ and John Cleveland¹, ¹University of Alabama at Birmingham, Birmingham, AL
Background/Purpose: To our knowledge, only a few population-based studies for vasculitis mortality exist; and most are limited to vasculitis sub-types. Therefore, our study objective was…
Abstract Number: 1690 • ACR Convergence 2020
Henoch-Schönlein Purpura Nephritis: Different Histological Classifications, but Which One Is Most Strongly Associated to the Outcome of the Disease? Pilot Study of the Paediatric Rheumatology European Society Vasculitis Working Party
Martina Held¹, Mario Sestan¹, Marijana Coric¹, Stela Bulimbasic¹, Teresa Giani², Neil Martin³, Nastasia Cekada¹, Sasa Srsen⁴, Ana Gudelj Gracanin⁵, Domagoj Kifer⁶, Merav Heshin⁷, Angelo Ravelli⁸, Rolando Cimaz⁹, Seza Ozen¹⁰, Alenka Gagro¹¹, Marijan Frkovic¹ and Marija Jelusic¹, ¹University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia, Zagreb, Grad Zagreb, Croatia, ²Anna Meyer Children’s Hospital, Florence, Italy, Florence, Toscana, Italy, ³Royal Hospital for Children, Glasgow, United Kingdom, Glasgow, Scotland, United Kingdom, ⁴University Hospital Centre Split, University of Split School of Medicine, Split, Croatia, Split, Splitsko-Dalmatinska, Croatia, ⁵University Hospital Dubrava, University of Zagreb School of Medicine, Zagreb, Croatia, Zagreb, Grad Zagreb, Croatia, ⁶Faculty of Pharmacy and Biochemistry, Zagreb, Croatia, Zagreb, Grad Zagreb, Croatia, ⁷Dana Dwek Children's Hospital, Tel Aviv, Israel, Tel Aviv, Tel Aviv, Israel, ⁸Università degli Studi di Genova, Genoa, Italy, ⁹ASST Gaetano Pini-CTO Institute and Università degli Studi di Milano, Milan, Italy, Milan, Italy, ¹⁰Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Ankara, Turkey, ¹¹Children's Hospital Zagreb, Josip Juraj Strossmayer University of Osijek, Medical Faculty Osijek, Zagreb, Croatia, Zagreb, Grad Zagreb, Croatia
Background/Purpose: Henoch-Schönlein purpura nephritis (HSPN) is the main and almost the only cause of morbidity and mortality among children suffering from this most common vasculitis…
Abstract Number: 1953 • ACR Convergence 2020
Somatic Mutations in a Single Residue of UBA1 Cause VEXAS, a Severe Adult-Onset Rheumatic Disease Presenting as Relapsing Polychondritis, Polyarteritis Nodosa, or Giant Cell Arteritis
David Beck¹, Marcela Ferrada², Keith Sikora³, Amanda Ombrello⁴, Daniela Ospina Cardona⁵, Nicholas Balanda⁶, Wuhong Pei⁶, Jason Collins⁶, Robert Colbert⁷, Mariana Kaplan⁸, Massimo Gadina⁹, Sinisa Savic¹⁰, Helen Lachmann¹¹, Kyle Retterer¹², Shawn Burgess¹³, William Gahl⁶, Achim Werner⁶, Ivona Aksentijevich¹⁴, Neal S. Young⁶, Katherine R. Calvo⁶, Peter C. Grayson¹⁵ and Daniel Kastner¹⁶, ¹National Human Genome Research Institute, Bethesda, ²Systemic Autoimmunity Branch, Vasculitis Translational Research Program, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ³National Institutes of Health Clinical Center, Bethesda, MD, ⁴National Human Genome Research Institute/National Institutes of Health, Bethesda, MD, ⁵National Institute of Health, Bethesda, ⁶National Institutes of Health, Bethesda, ⁷Pediatric Clinical Trials Unit and Office of Clinical Director, NIAMS, NIH, Bethesda, MD, ⁸National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, ⁹National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), NIH, Bethesda, MD, ¹⁰University of Leeds, England, United Kingdom, ¹¹National Amyloidosis CenterRoyal Free Campus, Rowland Hill St, London, United Kingdom, ¹²GeneDX, Gaithersburg, ¹³National Institutes of Health, Bethesda, MD, ¹⁴National Human Genome Research Institute, Bethesda, MD, ¹⁵Systemic Autoimmunity Branch, National Institutes of Health, NIAMS, Bethesda, MD, ¹⁶National Human Genome Research Institute (NHGRI), NIH, Bethesda, MD
Background/Purpose: Identifying the causes of adult-onset rheumatic diseases remains a challenge, and limits diagnosis, prognosis, and targeted treatment. We hypothesized that mutations in genes regulating…
Abstract Number: 0428 • ACR Convergence 2020
Enrichment of Clinical Trial Recruitment Using Advanced Molecular Imaging in Takayasu’s Arteritis
Kaitlin Quinn¹, Mark Ahlman², Emily Rose³, Peter Merkel⁴ and Peter C. Grayson⁵, ¹Systemic Autoimmunity Branch, National Institutes of Health, NIAMS, Bethesda, ²National Institutes of Health, Clinical Center, Radiology and Imaging Sciences, Bethesda, MD, ³Systemic Autoimmunity Branch, Vasculitis Translational Research Program, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ⁴University of Pennsylvania, Philadelphia, PA, ⁵Systemic Autoimmunity Branch, National Institutes of Health, NIAMS, Bethesda, MD
Background/Purpose: Definitions of disease activity are not standardized in Takayasu’s arteritis (TAK), which can lead to difficulty in determining whether a patient should be enrolled…
Abstract Number: 1427 • ACR Convergence 2020
Anti-IL5 Therapy for Eosinophilic Granulomatosis with Polyangiitis (EGPA) – An 18 Month Follow-up Study as a Steroid Sparing Therapeutic Approach
Allyson Egan¹, Pasupathy Sivasothy², Robin Gore³, Caroline Owen³, Marcos Del Martinez Pero⁴, Rachel Jones⁴, Lisa Willcocks⁴, Rona Smith⁴, Stella Burns⁴ and David Jayne⁵, ¹Vasculitis and Lupus Clinic, Department of Medicine, Addenbrooke's Hospital, Cambridge., London, United Kingdom, ²Vasculitis and Lupus Clinic, Department of Medicine, Addenbrooke's Hospital, Cambridge., Cambridge, England, United Kingdom, ³Department of Respiratory medicine, Addenbrooke's Hospital, Cambridge, Cambridge, United Kingdom, ⁴Vasculitis and Lupus Clinic, Department of Medicine, Addenbrooke's Hospital, Cambridge., Cambridge, ⁵Department of Medicine, Vasculitis and Lupus Research Group, University of Cambridge, Cambridge, UK, Cambridge, United Kingdom
Background/Purpose: EGPA is a small vessel vasculitis characterized by the presence of tissue eosinophilia, necrotizing vasculitis and granulomatous inflammation1. In the randomized, placebo-controlled MIRRA trial…
Abstract Number: 1691 • ACR Convergence 2020
Applied Geostatistics in Pediatric Rheumatology – Spatial Clustering of IgA Vasculitis
Matej Sapina¹, Marijan Frkovic², Mario Sestan², Sasa Srsen³, Aleksandar Ovuka⁴, Mateja Batnozic Varga⁵, Nastasia Cekada², Karolina Kramaric⁶, Dario Brdaric⁷, Kresimir Milas⁵, Alenka Gagro⁸ and Marija Jelusic², ¹University Hospital Centre Osijek, Josip Juraj Strossmayer University of Osijek, Medical Faculty Osijek, Faculty of Dental Medicine and Health, Osijek, Croatia, Osijek, Osjecko-Baranjska, Croatia, ²University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia, Zagreb, Grad Zagreb, Croatia, ³University Hospital Centre Split, University of Split School of Medicine, Split, Croatia, Split, Splitsko-Dalmatinska, Croatia, ⁴University Hospital Centre Rijeka, University of Rijeka School of Medicine, Rijeka, Croatia, Rijeka, Primorsko-Goranska, Croatia, ⁵University Hospital Centre Osijek, Josip Juraj Strossmayer University of Osijek, Medical Faculty Osijek, Croatia, Osijek, Osjecko-Baranjska, Croatia, ⁶University Hospital Centre Osijek, Josip Juraj Strossmayer University of Osijek, Faculty of Dental Medicine and Health Osijek, Croatia, Osijek, Osjecko-Baranjska, Croatia, ⁷Josip Juraj Strossmayer University of Osijek, Faculty of Dental Medicine and Health, Institute of Public Health for the Osijek-Baranja County, Osijek, Croatia, Osijek, Osjecko-Baranjska, Croatia, ⁸Children's Hospital Zagreb, Josip Juraj Strossmayer University of Osijek, Medical Faculty Osijek, Zagreb, Croatia, Zagreb, Grad Zagreb, Croatia
Background/Purpose: IgA vasculitis (IgAV) is the most common childhood vasculitis, which etiology seems to be related to the combination of genetic as well as environmental…
Abstract Number: 1959 • ACR Convergence 2020
Epidemiology of Hospitalizations and Associated Mortality in Vasculitis: A National Study
Jasvinder Singh¹ and John Cleveland¹, ¹University of Alabama at Birmingham, Birmingham, AL
Background/Purpose: To assess whether comorbid diseases frequently associated with primary hospitalizations for non-vasculitis causes in people with vasculitis are changing over time in people with…
Abstract Number: 0452 • ACR Convergence 2020
Recombinant Zoster Vaccine in Patients with Rheumatic Diseases: A Retrospective Study of 622 Patients
Tiphaine Lenfant¹, Elizabeth Kirchner², Rula Hajj-ali³, Leonard Calabrese³ and Cassandra Calabrese³, ¹Cleveland Clinic Foundation, Rocky River, OH, ²Cleveland Clinic Foundation, Cleveland, ³Cleveland Clinic, Cleveland, OH
Background/Purpose: The recombinant herpes zoster vaccine (RZV) was FDA-approved in 2017 but patients with rheumatic diseases were excluded from initial pivotal trials because of theoretical…
Abstract Number: 1428 • ACR Convergence 2020
The Efficacy and Safety of Tocilizumab in Patients with Giant Cell Arteritis: A Systematic Review and Meta-Analysis
Matthew J. Koster¹, Kenneth Warrington¹, Jian Han² and Shalini V. Mohan², ¹Mayo Clinic, Rochester, MN, ²Genentech, South San Francisco, CA
Background/Purpose: Tocilizumab (TCZ) has been proven to be safe and effective for the treatment of giant cell arteritis (GCA) in 2 randomized controlled trials; however,…
Abstract Number: 1919 • ACR Convergence 2020
Efficacy & Safety of Tocilizumab in Giant Cell Arteritis: A Single Centre NHS Experience Using Imaging (Ultrasound and PET CT) as a Diagnostic and Monitoring Tool
Alwin Sebastian¹, Abdul Kayani¹, Diana Prieto-Peña², Alessandro Tomelleri³, Madeline Whitlock¹, Kornelis S. M. van der Geest⁴ and Bhaskar Dasgupta¹, ¹Rheumatology, Mid and South Essex University Hospital NHS Foundation Trust, Southend University Hospital, United Kingdom, Westcliff on sea, England, United Kingdom, ²Research group on genetic epidemiology and atherosclerosis in systemic diseases and in metabolic bone diseases of the musculoskeletal system, IDIVAL; and Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ³Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy, Milan, Italy, ⁴Rheumatology and Clinical Immunology, University Medical Center Groningen, Netherlands, Groningen, Netherlands
Background/Purpose: Tocilizumab (TCZ), an IL-6 receptor blocker is approved to treat relapsing, refractory Giant cell arteritis (GCA) We here report our real-life clinical experience with…
Abstract Number: 2048 • ACR Convergence 2020
Comparison of Two Rituximab Regimens for Induction of Remission in Antineutrophil Cytoplasm Antibody-associated Vasculitis: Systematic Review and Meta-analysis
Valerie Benard¹, Cynthia Farhat², Melissa Zarandi-Nowroozi², Madeleine Durand³, Christian Pagnoux⁴, Pierre Charles⁵, Xavier Puechal⁶, Loïc Guillevin⁷ and Jean-Paul Makhzoum¹, ¹Vasculitis Clinic, Canadian Network for Research on Vasculitides (CanVasc), Department of Internal Medicine, Hopital du Sacre-Coeur de Montreal, University of Montreal, Montreal, QC, Canada, ²Department of Medecine, University of Montreal, Montreal, QC, Canada, ³Department of Internal Medicine, Centre Hospitalier de l’Universite de Montreal (CHUM) and Centre de Recherche du Centre Hospitalier de l’Universite de Montreal (CRCHUM), Montreal, QC, Canada, ⁴Vasculitis Clinic, Canadian Network for Research on Vasculitides (CanVasc), Department of Rheumatology, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada, ⁵Department of Internal Medicine, Institut Mutualiste Montsouris, Paris, France, ⁶National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris-Descartes University, Paris, France, ⁷Department of Internal Medecine, National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris-Descartes University, Paris, France
Background/Purpose: Organ or life-threatening granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA), two of the antineutrophil cytoplasm antibody-associated vasculitis (AAV), are treated with cyclophosphamide or…
Abstract Number: 0509 • ACR Convergence 2020
Classification of Patients with Relapsing Polychondritis Based on Somatic Mutations in UBA1
Marcela Ferrada¹, Keith Sikora², Sinisa Savic³, Yiming Luo⁴, Kristina Wells⁵, Emily Rose¹, Kaitlin Quinn⁶, Wendy Goodspeed¹, Anne Jones⁷, Mimi Le⁸, Amanda Ombrello⁹, Zuoming Deng¹⁰, Massimo Gadina¹¹, Wanxia Tsai¹², Ivona Aksentijevich⁷, Daniel Kastner¹³, David Beck¹⁴ and Peter C. Grayson¹⁵, ¹Systemic Autoimmunity Branch, Vasculitis Translational Research Program, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ²National Institutes of Health Clinical Center, Bethesda, MD, ³University of Leeds, England, United Kingdom, ⁴Rheumatology Fellowship and Training Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, ⁵Systemic Autoimmunity Branch, Vasculitis Translational Research Program, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, ⁶Systemic Autoimmunity Branch, National Institutes of Health, NIAMS, Washington, DC, ⁷National Human Genome Research Institute, Bethesda, MD, ⁸Oregon Health & Science University, Portland, ⁹National Institutes of Health, Bethesda, ¹⁰National Institute of Arthritis Musculoskeletal and Skin diseases, Bethesda, ¹¹National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), NIH, Bethesda, MD, ¹²National Institute of Arthritis Musculoskeletal and Skin diseases, Bethesda, MD, ¹³National Human Genome Research Institute (NHGRI), NIH, Bethesda, MD, ¹⁴National Human Genome Research Institute, Bethesda, ¹⁵Systemic Autoimmunity Branch, National Institutes of Health, NIAMS, Bethesda, MD
Background/Purpose: Relapsing polychondritis (RP) is a rare, heterogenous, systemic inflammatory disease with a predilection for cartilaginous structures. Recently we discovered myeloid-restricted somatic mutations in UBA1 in…
Abstract Number: 1429 • ACR Convergence 2020
An Evaluation of Real World Use of Biologics in Rare Systemic Vasculitides During Routine Clinical Care in the US
Kathryn Starzyk¹, Kimberly Milberg¹, Ashish Deshpande¹ and Gary Curhan¹, ¹OM1, Inc, Boston, MA
Background/Purpose: The mainstay of treatment for vasculitis has been corticosteroids and other cytotoxic and immunosuppressive medications (e.g., cyclophosphamide, methotrexate) to promptly achieve and maintain remission.…
Abstract Number: 1922 • ACR Convergence 2020
Association of Leukocyte Populations in Peripheral Blood and Arterial Wall Inflammation Assessed by FDG-PET in Takayasu’s Arteritis and Giant Cell Arteritis
K. Bates Gribbons¹, Kaitlin Quinn², Mark Ahlman³, Peter Merkel⁴ and Peter C. Grayson⁵, ¹McGovern Medical School UT Houston, Houston, TX, ²Systemic Autoimmunity Branch, National Institutes of Health, NIAMS, Bethesda, ³National Institutes of Health, Clinical Center, Radiology and Imaging Sciences, Bethesda, MD, ⁴University of Pennsylvania, Philadelphia, PA, ⁵Systemic Autoimmunity Branch, National Institutes of Health, NIAMS, Bethesda, MD
Background/Purpose: Positron emission tomography (PET) is useful to demonstrate fluorodeoxyglucose (FDG) uptake in the large arteries in both Takayasu’s arteritis (TAK) and giant cell arteritis…

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