Abstracts tagged "ANCA"

Abstract Number: 1679 • 2019 ACR/ARP Annual Meeting
Adaptive Study Design of a Randomized, Multicenter, 2-Part Phase 2 Trial of Replacement of Glucocorticoids by IFX-1, a C5a Inhibitor, in Active Granulomatosis with Polyangiitis and 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 are both forms of anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV). Standard of…
Abstract Number: 2642 • 2019 ACR/ARP Annual Meeting
ANCA Testing: Final Diagnoses in Cases with Positive Immunofluorescence and Negative ELISA
‪Gabriel Breuer‬‏¹, Bashar Fteiha ¹, Alon Benaya ¹, Marwan Abu Sneineh‬‏ ¹ and Gideon Nesher ¹, ¹Shaare Zedek Medical Center, Jerusalem, Yerushalayim, Israel
Background/Purpose: With the widespread availability of anti-neutrophil cytoplasmic antibody (ANCA) testing, interpreting positive results has become increasingly challenging. In addition to ANCA Associated Vasculitis (AAV),…
Abstract Number: 806 • 2019 ACR/ARP Annual Meeting
A Randomized, Controlled Trial of Rituximab versus Azathioprine After Induction of Remission with Rituximab for Patients with ANCA-associated Vasculitis and Relapsing Disease
Rona Smith¹, David Jayne ² and Peter Merkel ³, ¹University of Cambridge, Cambridge, England, United Kingdom, ²Vasculitis and Lupus Clinic, Addenbrooke’s Hospital, University of Cambridge, UK, Cambridge, United Kingdom, ³University of Pennsylvania, Philadelphia
Background/Purpose: Rituximab is an effective therapy for induction of remission in ANCA-associated vasculitis (AAV). However, the effect of rituximab is not sustained, and relapse rates…
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: 2643 • 2019 ACR/ARP Annual Meeting
Comparative Study of Renal Transplantation Due to Rapidly Progressive Glomerulonephritis (RPGN): Study of 42 Patients from a Single Tertiary Centre
Lara Sanchez-Bilbao¹, Marina De Cos-Gomez ², Iñigo Gonzalez-Mazon ³, Belén Atienza-Mateo ¹, Jose Luis Martín-Varillas ¹, Mónica Calderón-Goercke ⁴, DIANA PRIETO- PENA ³, Juan Carlos Ruiz-San Millan ², Miguel A Gonzalez-Gay ¹ and Ricardo Blanco ¹, ¹Rheumatology, Hospital Universitario Marques de Valdecilla, Santander, Spain, ²Nephrology, Hospital Universitario Marques de Valdecilla, Santander, Cantabria, Spain, ³Rheumatology, Hospital Universitario Marques de Valdecilla, Santander, Cantabria, Spain, ⁴Hospital Marqués de Valdecilla, Santander, Cantabria, Spain
Background/Purpose: Rapidly Progressive Glomerulonephritis (RPGN) is characterized clinically by a rapid and severe decline in kidney function. Thus, this entity may lead to an end…
Abstract Number: L04 • 2018 ACR/ARHP Annual Meeting
Pediatric Open-Label Clinical Study of Rituximab for the Treatment of Granulomatosis with Polyangiitis (GPA) and Microscopic Polyangiitis (MPA)
Paul Brogan¹, Gavin Cleary², Aimee O. Hersh³, Ozgur Kasapcopur⁴, Satyapal Rangaraj⁵, Rae S.M. Yeung⁶, Andrew Zeft⁷, Simone Melega⁸, Paul Brunetta⁹, Jennifer Cooper¹⁰, Pooneh Pordeli¹¹ and Patricia B. Lehane¹², ¹Infection Inflammation and Rheumatology, UCL GOL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ²Alder Hey Children's Hospital, Liverpool, United Kingdom, ³Pediatrics/Rheumatology, University of Utah, Salt Lake City, UT, ⁴Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey, ⁵Nottingham University Hospitals NHS Trust, Nottingham, United Kingdom, ⁶Paediatrics, Immunology and Medical Science, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada, ⁷Center for Pediatric Rheumatology & Immunology, The Cleveland Clinic Foundation, Cleveland, OH, ⁸F. Hoffmann-La Roche, Basel, Switzerland, ⁹Genentech, Inc., South San Francisco, CA, ¹⁰Pediatric Rheumatology, Univ. of California San Francisco, San Francisco, CA, ¹¹Hoffmann-La Roche Ltd., Mississauga, ON, Canada, ¹²Roche Products Ltd., Welwyn Garden City, United Kingdom
Background/Purpose: PePRS is a Phase IIa international, multicenter, open-label single arm study of rituximab in pediatric patients with newly diagnosed or relapsing GPA or MPA.…
Abstract Number: 1762 • 2018 ACR/ARHP Annual Meeting
Plasma iC3b Level As a Biomarker of Disease Relapse in ANCA-Associated Vasculitis
Catherine E. Najem¹, Martin Schmidt², Chad Stiening³, David Cuthbertson⁴, Simon Carette⁵, Nader A. Khalidi⁶, Curry L. Koening⁷, Carol Langford⁸, Carol A. McAlear⁹, Paul A. Monach¹⁰, Larry W. Moreland¹¹, Christian Pagnoux¹², Philip Seo¹³, Ulrich Specks¹⁴, Antoine G. Sreih¹, Steven R. Ytterberg¹⁵ and Peter A. Merkel^16,17, ¹Rheumatology, Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, ²Kypha, Inc., St. Louis, MO, ³Kypha, Inc., St Louis, MO, ⁴Biostatistics and Informatics, Department of Pediatrics, Department of Biostatistics and Informatics, Department of Pediatrics, University of South Florida, Tampa, FL, ⁵Division of Rheumatology, Mount Sinai Hospital, Toronto, ON, Canada, ⁶Rheumatology, McMaster University, Hamilton, ON, Canada, ⁷Rheumatology, Division of Rheumatology, University of Utah, Salt Lake City, UT, ⁸Rheumatic and Immunologic Diseases, Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, OH, ⁹Division of Rheumatology, University of Pennsylvania; Perelman School of Medicine, Philadelphia, PA, ¹⁰Section of Rheumatology, Boston University School of Medicine, Boston, MA, ¹¹Division of Rheumatology and Clinical Immunology, Division of Rheumatology, University of Pittsburgh, Pittsburgh, PA, ¹²Division of Rheumatology, Division of Rheumatology, Mount Sinai Hospital, Toronto, ON, Canada, ¹³Medicine, Division of Rheumatology, Johns Hopkins University, Baltimore, MD, ¹⁴Mayo Clinic College of Medicine, Rochester, MN, ¹⁵Rheumatology, Division of Rheumatology, Mayo Clinic College of Medicine, Rochester, MN, ¹⁶Division of Rheumatology, Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, ¹⁷Department of Biostatistics, Epidemiology, and Informatics, University of Pennsylvania, Philadelphia, PA
Background/Purpose:C3, the central protein of the complement cascade, participates in an amplification loop that can lead to complement deposition and host tissue damage. If elevated,…
Abstract Number: 2727 • 2018 ACR/ARHP Annual Meeting
Prevalence of Clinical and Subclinical Ophthalmologic Manifestations in Association with Systemic Symptoms, Disease Activity and Damage in Patients with Granulomatosis with Polyangiitis
Andrea Hinojosa-Azaola¹, Annette García-Castro² and Alejandra Juárez-Flores³, ¹Immunology and Rheumatology, Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico, ²Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico, ³Ophthalmology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico
Background/Purpose: Ophthalmologic involvement in Granulomatosis with Polyangiitis (GPA) is present in 50-60% of the patients and can affect any part of the ocular glove and…
Abstract Number: 1763 • 2018 ACR/ARHP Annual Meeting
Characterizing the Gut and Plasma Metabolomes in Patients with ANCA-Associated Vasculitis
Catherine E. Najem¹, Jung-Jin Lee², Ceylan Tanes², Cassidy Strange², Elliot Friedman², Antoine G. Sreih¹, Rennie L. Rhee¹, Abdallah Geara³, Hongzhe Li⁴, Kyle Bittinger², James D. Lewis⁵, Gary Wu⁵ and Peter A. Merkel⁴, ¹Rheumatology, Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, ²Division of Gastroenterology, Hepatology, and Nutrition, Children's Hospital of Philadelphia, Philadelphia, PA, ³Division of Nephrology and Hypertension, University of Pennsylvania, Philadelphia, PA, ⁴Department of Biostatistics, Epidemiology, and Informatics, University of Pennsylvania, Philadelphia, PA, ⁵Division of Gastroenterology, University of Pennsylvania, Philadelphia, PA
Background/Purpose:To explore the mechanisms by which an altered gut microbiota might predispose to ANCA-associated vasculitis (AAV), a comprehensive metabolic profiling of fecal and plasma bile…
Abstract Number: 2729 • 2018 ACR/ARHP Annual Meeting
Increased Risk of Cerebrovascular Accident Among Patients with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Systematic Review and Meta-Analysis of Cohort Studies
Patompong Ungprasert¹, Karn Wijarnpreecha² and Wisit Cheungpasitporn³, ¹Clinical Epidemiology Unit, Department of Research and Development, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand, ²Internal medicine, Bassett medical center, cooperstown, NY, ³Medicine, University of Mississippi Medical center, Jackson, MS
Increased Risk of Cerebrovascular Accident among Patients with Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Systematic Review and Meta-analysis of Cohort StudiesAbstractBackground/Purpose: An increased risk of cardiovascular…
Abstract Number: 13 • 2018 ACR/ARHP Annual Meeting
Shared Genetic Origins Among Anti-Proteinase 3 and Anti-Glomerular Basement Membrane Double-Positive Human Autoantibodies
Jeffrey R. Ord¹, Amy G. Clark¹ and Mary H. Foster², ¹Duke University Medical Center, Durham, NC, ²Medicine, Duke University Medical Center, Durham, NC
Background/Purpose: Patients with ANCA vasculitis and anti-glomerular basement membrane (GBM) nephritis (Goodpasture’s Disease) develop pathogenic autoantibodies (autoAb) that destroy the microvasculature in lungs and kidneys.…
Abstract Number: 1764 • 2018 ACR/ARHP Annual Meeting
The Utility of Serum Angiopoietin-1 and Angiopoietin-2 in Patients with Anti-Neutrophil Cytoplasmic Autoantibody-Associated Vasculitis
Yoko Wada¹, Takeshi Kuroda², Masaaki Nakano³ and Ichiei Narita¹, ¹Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan, ²Health Administration Center, Niigata University, Niigata, Japan, ³Department of Medical Technology, School of Health Sciences, Faculty of Medicine, Niigata University, Niigata, Japan
Background/Purpose: Angiopoietin-1 (Ang-1) and Angiopoietin-2 (Ang-2) are antagonistic ligands which bind with similar affinity to the extracellular domain of the tyrosine kinase with Ig-like and…
Abstract Number: 2731 • 2018 ACR/ARHP Annual Meeting
Clinical Characteristics and Outcomes of ANCA-Vasculitides Associated Renal Disease in a Multi-Ethnic Population from a County Hospital
Shilpa Arora¹, Ambrish Athavale² and Peter Hart², ¹Medicine, John H Jr Stroger Hospital of Cook County, Chicago, IL, ²John H Jr Stroger Hospital of Cook County, Chicago, IL
Background/Purpose: Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides include granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and renal-limited vasculitis (RLV). We aimed to study the clinical features…
Abstract Number: 904 • 2018 ACR/ARHP Annual Meeting
Long-Term Safety of Rituximab in Granulomatosis with Polyangiitis or Microscopic Polyangiitis: Results of the Four-Year Study of Rituximab in ANCA-Associated Vasculitis Registry
John L. Niles¹, Peter A. Merkel², Lester Mertz³, Patricia B. Lehane⁴, Pooneh Pordeli⁵ and Félix Erblang⁶, ¹Department of Medicine, Division of Nephrology, Massachusetts General Hospital, Boston, MA, ²Division of Rheumatology, Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, ³Rheumatology, Mayo Clinic, Rochester, MN, ⁴Roche Products Ltd., Welwyn Garden City, United Kingdom, ⁵Hoffmann-La Roche Ltd., Mississauga, ON, Canada, ⁶Hoffmann-La Roche Ltd., Basel, Switzerland
Background/Purpose: Potential therapy-related toxicities are important causes of morbidity in patients with the ANCA-associated vasculitides granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA). Long-term safety…
Abstract Number: 1765 • 2018 ACR/ARHP Annual Meeting
Cerebrospinal Fluid Biomarker of Disease Activity: Significance in ANCA-Related Hypertrophic Pachymeningitis
Yasuhiro Shimojima¹, Dai Kishida² and Yoshiki Sekijima², ¹Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan, ²Department of Medicine (Neurology and Rheumatology), Shinshu University School of Medicine, Matsumoto, Japan
Background/Purpose: Hypertrophic pachymeningitis (HP), which becomes the cause of chronic headache, seizure, and cranial neuropathy, is an inflammatory disorder demonstrating focal and diffuse thickening of…