Abstracts tagged "Vasculitis"

Abstract Number: 1680 • 2019 ACR/ARP Annual Meeting
Design of a Phase 2, Multicenter, Randomized, Double-Blind, Placebo-Controlled Trial of 2 Different Dose Regimens of IFX-1, a C5a Inhibitor, as an Add-On Therapy for Granulomatosis with Polyangiitis or Microscopic Polyangiitis
Peter Merkel¹, Bernhard Hellmich ², David Jayne ³, Simon Rückinger ⁴, Zsuzsanna Tamas ⁵, Claus Thielert ⁵ and Othmar Zenker ⁶, ¹University of Pennsylvania, Philadelphia, PA, ²Department of Internal Medicine, Rheumatology and Immunology, Vasculitis-Center Tübingen-Kirchheim, Medius Klinik Kirchheim, University of Tübingen, Kirchheim-Teck, Germany, Kirchheim, Germany, ³Vasculitis and Lupus Clinic, Addenbrooke’s Hospital, University of Cambridge, UK, Cambridge, United Kingdom, ⁴Metronomia Clinical Research GmbH, Munich, Germany, ⁵InflaRx GmbH, Planegg, Germany, ⁶InflaRx GmbH, Jena, Germany
Background/Purpose: Granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) are life-threatening rare autoimmune diseases to anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Standard of care (SOC)…
Abstract Number: 2630 • 2019 ACR/ARP Annual Meeting
A Dutch Consensus Statement on the Diagnosis and Treatment of ANCA Associated Vasculitis
Ebru Dirikgil¹, Peter Verhoeven ², Sander Tas ³, Abraham Rutgers ⁴, Hein Bernelot Moens ⁵, Jacob van Laar ⁶, Willem Jan Bos ⁷ and Onno Teng ¹, ¹LUMC, Leiden, Netherlands, ²The Dutch Vasculitis Foundation, Silvolde, Netherlands, ³Amsterdam Rheumatology & immunology Center, Amsterdam UMC/University of Amsterdam, Amsterdam, Netherlands, ⁴University Medical Center Groningen, Groningen, Netherlands, ⁵Ziekenhuisgroep Twente, Almelo, Netherlands, ⁶UMC Utrecht, Department of Rheumatology & Clinical Immunology, Utrecht, The Netherlands, Utrecht, Netherlands, ⁷St. Antoniusziekenhuis, Nieuwegein, Netherlands
Background/Purpose: Several guidelines have been published on the diagnosis and treatment of anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis (AAV). These guidelines provide an evidence-based approach…
Abstract Number: 810 • 2019 ACR/ARP Annual Meeting
Small Vessel Vasculitis Syndrome with Autoinflammation Caused by De Novo Mutations in LYN Kinase
Adriana de Jesus¹, Gina Montealegre Sanchez ², Helen Freeman ³, Neil Martin ⁴, Ebun Omoyinmi ⁵, Katherine Calvo ⁶, Richard Chyi-chia Lee ⁷, Murray Passo ⁸, Natasha Ruth ⁸, David Kleiner ⁷, Yan Huang ⁹, Nirali Shah ¹⁰, Paul Brogan ¹¹, SuJin Hwang ¹², HyeSun Kuehn ¹², Sergio Rosenzweig ¹², Zuoming Deng ¹³, Anna Huttenlocher ¹⁴, Susan Moir ¹⁵, Douglas Kuhns ¹⁶ and Raphaela Goldbach-Mansky ¹⁷, ¹Translation Autoinflammatory Diseases Section/NIAID/NIH, Silver Spring, MD, ²Translational Autoinflammatory Disease Section/NIAID/NIH, Bethesda, ³Raigmore Hospital, Inverness, United Kingdom, ⁴Royal Hospital for Children, Glasgow, United Kingdom, ⁵University College London Institute of Child Health, London, United Kingdom, ⁶Hematology Service/Department of Laboratory Medicine/NIH, Bethesda, MD, ⁷Laboratory of Pathology/NCI/NIH, Bethesda, MD, ⁸Medical University of South Carolina, Charleston, SC, ⁹Translational Autoinflammatory Disease Section/NIAID/NIH, Bethesda, MD, ¹⁰Pediatric Oncology Branch/NCI/NIH, Bethesda, MD, ¹¹Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, ¹²Immunology Service/Department of Laboratory Medicine/NIH, Bethesda, MD, ¹³Biomining and Discovery Section/NIAMS/NIH, Bethesda, MD, ¹⁴Department of Pediatrics/University of Wisconsin, Madison, WI, ¹⁵Immunopathogenesis Section/NIAID/NIH, Bethesda, MD, ¹⁶Collaborative Clinical Research Branch/NIAID/NIH, Bethesda, MD, ¹⁷Translational Autoinflammatory Diseases Section/NIAID/NIH, Bethesda, MD
Background/Purpose: Lyn kinase is a member of the Src family of non-receptor tyrosine-protein kinases that modifies signals from various cell surface receptors and regulates innate…
Abstract Number: 1681 • 2019 ACR/ARP Annual Meeting
The Association of Reduced Low-Density Lipoprotein (LDL) Cholesterol Levels with ANCA-Associated Vasculitis (AAV)
Zachary Wallace¹, Xiaoqing Fu ¹, Yuqing Zhang ², John Stone ³ and Hyon K. Choi ², ¹Massachusetts General Hospital, Boston, ²Massachusetts General Hospital, Boston, MA, ³Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Boston, MA
Background/Purpose: ANCA-associated vasculitis (AAV) is a small vessel vasculitis associated with an intense inflammatory state. AAV patients are at a 2-fold higher risk of cardiovascular…
Abstract Number: 2632 • 2019 ACR/ARP Annual Meeting
Changing Trends in the Management of ANCA-associated Vasculitis at an Academic Medical Center
Sana Afroz¹ and Najia Shakoor ², ¹RUMC, Chicago, IL, ²Rush University Medical Center, Chicago, IL
Background/Purpose: For years, cyclophosphamide (CYC) has been the primary treatment for severe anca-associated vasculitides (AAV) and has significantly improved disease related mortality. In addition, maintenance…
Abstract Number: 869 • 2019 ACR/ARP Annual Meeting
Clinical Manifestations of Patients with Eosinophilic Granulomatosis with Polyangiitis in a Large North American Cohort
Irena Doubelt¹, David Cuthbertson ², Simon Carette ¹, Nader A. Khalidi ³, Curry L. Koening ⁴, Carol Langford ⁵, Carol A. McAlear ⁶, Larry W. Moreland ⁷, Paul Monach ⁸, Philip Seo ⁹, Ulrich Specks ¹⁰, Antoine Sreih ¹¹, Steven Ytterberg ¹⁰, Peter A. Merkel ¹², Christian Pagnoux ¹ and VCRC Vasculitis Clinical Research Consortium ¹³, ¹Mount Sinai Hospital and University Health Network, Toronto, ON, Canada, ²University of South Florida, Tampa, FL, ³McMaster University, Hamilton, ON, Canada, ⁴University of Utah Hospital, Salt Lake City, UT, ⁵Cleveland Clinic, Cleveland, OH, ⁶University of Pennsylvania - VCRC Project Manager, Philadelphia, PA, ⁷University of Pittsburgh, Pittsburgh, PA, ⁸Brigham and Women's Hospital, Boston, MA, ⁹Johns Hopkins Medicine, Baltimore, MD, ¹⁰Mayo Clinic College of Medicine, Rochester, MN, ¹¹University of Pennsylvania, Philadelphia, PA, ¹²Univeristy of Pennsylvania, Philadelphia, PA, ¹³University of Pennsylvania, Division of Rheumatology, philadelphia
Background/Purpose: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, systemic necrotizing small-vessel vasculitis, with only a few published North American series. This project aimed to…
Abstract Number: 1687 • 2019 ACR/ARP Annual Meeting
Dramatic but Suspensive Effect of interleukin-1 Inhibitors on Persistent Urticarial Vasculitis
Thomas Bettuzzi ¹, Alban Deroux ², Marie Jachiet ³, Meryem-Maud Farhat ⁴, Julien Wipff ¹, Marc Fabre ⁵, Laurence Bouillet ², Nora Kramkimel ¹, Selim Aractingi ¹, Nicolas Dupin ¹ and Benjamin Terrier⁶, ¹Cochin Hospital, Paris, France, ²CHU, Grenoble, France, ³Saint Louis Hospital, Paris, France, ⁴CHRU, Lille, France, ⁵CH, Bourgoin-Jailleu, France, ⁶National Referral Center for Rare Systemic Autoimmune Diseases Paris Cochin, Paris, France
Background/Purpose: Urticarial vasculitis (UV) is a rare disease characterized by dermal capillary inflammation responsible for long-lasting urticarial lesions. UV can be separated into 2 different…
Abstract Number: 2634 • 2019 ACR/ARP Annual Meeting
ANCA Response upon Rituximab or Cyclophosphamide in ANCA-associated Vasculitis Patients
Laura van Dam¹, Ebru Dirikgil ², Edwin Bredewold ², Argho Ray ², Ton Rabelink ², Cees van Kooten ² and Onno Teng ², ¹LUMC, Leiden, Zuid-Holland, Netherlands, ²LUMC, Leiden, Netherlands
Background/Purpose: Recent studies have demonstrated that in patients with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) with successful remission-induction (RI) after cyclophosphamide (CYC), maintenance treatment with…
Abstract Number: 821 • 2018 ACR/ARHP Annual Meeting
Comparison of Aortitis Vs Non-Inflammatory Aortic Aneurysms Among Patients Who Undergo Open Aortic Aneurysm Repair
Laarni Quimson¹, Bryan Rea² and Rennie L. Rhee³, ¹Internal Medicine, University of Pennsylvania, Philadelphia, PA, ²University of Pennsylvania, Philadelphia, PA, ³Rheumatology, Division of Rheumatology, University of Pennsylvania, Philadelphia, PA
Comparison of Aortitis vs Non-Inflammatory Aortic Aneurysms Among Patients Who Undergo Open Aortic Aneurysm RepairBackground/Purpose: Distinguishing aortitis-induced aneurysms from more common non-inflammatory aortic aneurysms is…
Abstract Number: 828 • 2018 ACR/ARHP Annual Meeting
Medium and Small-Sized Vessel Involvement in Hypereosinophilic Syndrome
Julien Rohmer¹, Matthieu Groh², Maxime Samson³, Jonathan London⁴, Marie JACHIET⁵, Diane Rouzaud⁶, Antoinette Perlat⁷, Romain Paule⁸, Felipe SUAREZ⁹, Jean-Emmanuel Kahn¹⁰, Loïc Guillevin¹¹ and Benjamin Terrier¹², ¹National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, Internal medicine, France, Paris, France, ²Internal Medicine, Foch, Suresnes, France, ³Dijon University Hospital, Dijon, France, ⁴Service de Médecine Interne, Hôpital Cochin, Centre de référence national pour les maladies systémiques autoimmunes rares d’Ile de France, DHU Authors, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France, Paris, France, ⁵Dermatology department, St Louis Hospital, Paris, France, ⁶Université Paris-Diderot, Paris, France, ⁷Internal medicine, CHU de Rennes, Rennes, France, ⁸Department of Internal Medicine, Department of Internal Medicine, Cochin University Hospital, Paris, France, ⁹Hematology Department, Necker Hospital, Paris, France, ¹⁰Service de Médecine Interne, Centre de Référence des Syndromes Hyperéosinophiliques-CEREO, Hôpital Foch, Université Versailles–Saint-Quentin-en-Yvelines, Suresnes, France, ¹¹Medecine Interne, Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hôpital Cochin, AP–HP, Université Paris Descartes, Paris, France, ¹²National Referral Center for Rare Systemic Autoimmune Diseases, Paris Cochin, France, Paris, France
Background/Purpose: Primary systemic vasculitis, especially eosinophilic granulomatosis with polyangiitis and polyarteritis nodosa, can be associated with blood and tissue eosinophilia. Conversely, eosinophilia in the context…
Abstract Number: 833 • 2018 ACR/ARHP Annual Meeting
11 Patients with Relapsing Polychondritis Presenting with Severe Airways Disease in One Centre in UK
Shirish Dubey¹, Grace Pink², Asad Ali², Nicholas Hart³, Patrick Murphy⁴, Joanne Shakespeare⁵, Colin Gelder⁶, Chris Taylor² and David D'Cruz⁷, ¹Rheumatology, University Hospital Coventry and Warwickshire NHS Trust, Coventry, United Kingdom, ²Respiratory medicine, University Hospital Coventry and Warwickshire NHS Trust, Coventry, United Kingdom, ³Respiratory medicine, St Thomas' Hospital, London, United Kingdom, ⁴Lane Fox Respiratory Unit, St Thomas' Hospital, London, United Kingdom, ⁵Respiratory physiology, University Hospital Coventry and Warwickshire NHS Trust, Coventry, United Kingdom, ⁶University Hospital Coventry and Warwickshire NHS Trust, Coventry, United Kingdom, ⁷Louise Coote Lupus Unit, Guy's and St. Thomas' Hospital, London, United Kingdom
Background/Purpose: Relapsing polychondritis (RP) is a multi-system disease characterised by episodes of progressive inflammation and subsequent degeneration of cartilage and connective tissue throughout the body.…
Abstract Number: 1317 • 2018 ACR/ARHP Annual Meeting
Tracheomalacia in Coventry in UK
Grace Pink¹, Shirish Dubey², Asad Ali¹, Joanna Shakespeare³, Chris Taylor¹ and Colin Gelder⁴, ¹Respiratory medicine, University Hospital Coventry and Warwickshire NHS Trust, Coventry, United Kingdom, ²Rheumatology, University Hospital Coventry and Warwickshire NHS Trust, Coventry, United Kingdom, ³Respiratory Physiology, University Hospital Coventry and Warwickshire NHS Trust, Coventry, United Kingdom, ⁴University Hospital Coventry and Warwickshire NHS Trust, Coventry, United Kingdom
Background/Purpose: Tracheomalacia (TM) is pathological diffuse or segmental narrowing of the tracheal lumen, caused by softening of the supporting cartilage and reduction in stiffness of…
Abstract Number: 3079 • 2016 ACR/ARHP Annual Meeting
The Characteristic Features of the Patients with Deficiency of Adenosine Deaminase 2 (DADA2)
Abdulsamet Erden¹, Ezgi Deniz Batu², Ekim Z. Taskiran³, Hafize Emine Sonmez², Alper Sari¹, Berkan Armagan¹, Levent Kilic¹, Zehra Serap Arıcı⁴, Yelda Bilginer⁵, Ali Akdogan¹, Omer Karadag¹, Umut Kalyoncu¹ and Seza Ozen^6,7, ¹Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey, ²Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey, ³Department of Medical Genetics, Hacettepe University Faculty of Medicine, ANKARA, Turkey, ⁴Departments of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey, ⁵Departments of Pediatric Nephrology and Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey, ⁶Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey, ⁷Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, ANKARA, Turkey
Background/Purpose: Deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessive autoinflammatory disease resulting from a loss-of-function mutation in Cat Eye Syndrome Chromosome Region Candidate…
Abstract Number: 1339 • 2015 ACR/ARHP Annual Meeting
Granulomatosis with Polyangiitis or Post Pulmonary Tuberculosis: Can CT Chest Help in Differentiating in a Tuberculosis Endemic Area?
Aman Sharma¹, Manphool Singhal², Mitali Sen³, Manish Rathi⁴, Varun Dhir¹, Kusum Sharma⁵, Ranjana Minz⁶, Surjit Singh³, Pradeep Bambery⁷ and Niranjan Khandelwal², ¹Internal Medicine (Rheumatology Unit), Postgraduate Institute of Medical Education and Research, Chandigarh, India, ²Department of Radiodiagnosis, PGIMER,, Chandigarh, India, ³Department of Internal Medicine,, PGIMER,, Chandigarh, India, ⁴Department of Nephrology,, Postgraduate Institute of Medical Education and Research, Chandigarh, India, ⁵Department of Medical Microbiology,, PGIMER,, Chandigarh, India, ⁶Department of Immunopathology,, PGIMER,, Chandigarh, India, ⁷Rheumatology, Bundaberg base hospital, University of Queensland, Bundaberg, Australia
Background/Purpose: The data on the Computed tomography (CT) changes of chest in Granulomatosis with polyangiitis (GPA) is available mostly from low tuberculosis prevalence countries. We…
Abstract Number: 1978 • 2015 ACR/ARHP Annual Meeting
Tocilizumab in Refractory Large Vessel Vasculitis with Aorta Involvement. Study on 10 Patients Evaluated By Positron Emission Tomoghraphy
Carlotta Nannini¹, Stelvio Sestini², Laura Niccoli³, Emanuele Cassarà⁴, Olga Kaloudi⁵ and Fabrizio Cantini⁶, ¹Rheumatology, Prato Hospital, Prato, Italy, ²Radiology Department, Prato, Italy, ³Prato Hospital, Prato, Italy, ⁴Rheumatology, Hospital of Prato, Prato, Italy, ⁵PRato, Prato Hospital, Prato, Italy, ⁶Prato, Prato, Italy
Background/Purpose: Large vessel vasculitis (LVV) with aorta involvement are often refractory to common immunosuppressive therapy. Due to the pathogenic role of IL-6 in, we prospectively…

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