Abstracts tagged "complement"

Abstract Number: 0684 • ACR Convergence 2023
Report on Twelve Patients with Diffuse Alveolar Hemorrhage in the Phase 3 Trial of Avacopan for the Treatment of ANCA-Associated Vasculitis
Ulrich Specks¹, David Jayne² and Peter Merkel³, ¹Mayo Clinic, Rochester, MN, ²University of Cambridge, Cambridge, United Kingdom, ³University of Pennsylvania, Philadelphia, PA
Background/Purpose: Although respiratory tract involvement in ANCA-associated vasculitis (AAV) is frequent and associated with increased mortality, studies focusing on diffuse alveolar hemorrhage (DAH) in AAV…
Abstract Number: 2370 • ACR Convergence 2023
C5 as a Genetic Marker for Discriminating Between IgA Vasculitis and IgA Nephropathy?
JOAO CARLOS BATISTA LIZ¹, Vanesa Calvo Río², María Sebastián Mora-Gil¹, Belén Sevilla-Pérez³, María Teresa Leonardo⁴, Ana Peñalba⁴, María Jesús Cabero⁴, Javier Narvaez⁵, luis martin penagos⁶, Emilio Rodrigo⁶, Lara Belmar-Vega⁶, Cristina Gomez-Fernandez⁷, Luis Caminal-Montero⁸, Paz Collado⁹, Antonio Fernandez-Nebro¹⁰, gisela Díaz-Cordovés¹¹, Maryia Nikitsina¹², Esther Vicente Rabaneda¹³, secundino Cigarrán¹⁴, jesús Calviño¹⁵, carmen cobelo¹⁵, Manuel León Luque¹⁶, Esteban Rubio¹⁶, Juan María Blanco-Madrigal¹⁷, Eva Galindez-Agirregoikoa¹⁸, Santos Castañeda¹⁹, Ricardo Blanco²⁰, Verónica Pulito-Cueto¹ and Raquel López-Mejías¹, ¹Rheumatology Department, Immunopathology Group, Hospital Universitario Marqués de Valdecilla-IDIVAL, Santander, Spain, ²Valdecilla Hospital, Santander, Spain, ³Division of Pediatrics, Hospital Universitario San Cecilio, Granada, Spain, ⁴Division of Pediatrics, Hospital Universitario Marqués de Valdecilla, Santander, Spain, ⁵Hospital Universitario de Bellvitge, Barcelona, Spain, ⁶Division of Nephrology, Immunopathology Group, Hospital Universitario Marqués de Valdecilla-DIVAL, Santander, Spain, ⁷Division of Dermatology,Immunopathology Group, Hospital Universitario Marqués de Valdecilla-IDIVAL, Santander, Spain, ⁸Internal Medicine Department, Hospital Universitario Central de Asturias, Instituto de Investigación Sanitaria del Principado de Asturias (ISPA), Oviedo, Spain, ⁹Division of Rheumatology, Hospital Universitario Severo Ochoa, Madrid, Spain, ¹⁰Hospital Regional Universitario de Málaga, Malaga, Spain, ¹¹Division of Rheumatology, Hospital Regional Universitario Carlos Haya, Málaga, Spain, ¹²Division of Rheumatology, Hospital Universitario de La Princesa, Madrid, Spain, ¹³Rheumatology, Hospital Universitario de La Princesa, Madrid, Spain, ¹⁴Division of Nephrology, Hospital da Costa Burela, Lugo, Spain, ¹⁵Division of Nephrology, Hospital Universitario Lucus Augusti, Lugo, Spain, ¹⁶Division of Rheumatology, Hospital Universitario Virgen del Rocío, Sevilla, Spain, ¹⁷Division of Rheumatology, Hospital Universitario de Basurto, Bilbao, Spain, ¹⁸Basurto University Hospital, Bilbao, Spain, ¹⁹Hospital Universitario de la Princesa, Madrid, Spain, ²⁰Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain
Background/Purpose: Immunoglobulin-A vasculitis (IgAV) and IgA nephropathy (IgAN) are inflammatory conditions that share pathophysiological mechanisms1,2. Similar features are also described between IgAV nephritis (IgAVN) and…
Abstract Number: 0685 • ACR Convergence 2023
Efficacy and Safety of Avacopan in Patients with ANCA-Associated Vasculitis Receiving Rituximab in a Phase 3 Trial
Duvuru Geetha¹, Anisha Dua², Huibin Yue³, Carlo Salvarani⁴, David Jayne⁵ and Peter Merkel⁶, ¹Johns Hopkins University, Baltimore, MD, ²Northwestern University, Chicago, IL, ³Amgen, Inc., Thousand Oaks, CA, ⁴Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy, ⁵University of Cambridge, Cambridge, United Kingdom, ⁶University of Pennsylvania, Philadelphia, PA
Background/Purpose: The randomized, double-blind, double-dummy, controlled Phase 3 ADVOCATE trial tested whether avacopan, an oral selective C5a receptor inhibitor approved for the treatment of ANCA-associated…
Abstract Number: 2494 • ACR Convergence 2023
Complement mRNA Expression in Patients with ANCA-associated Glomerulonephritis
Salem Almaani¹, Arnon Arazi², Huijuan Song³, Pearlly Yan³, Estela Puchulu-Campanella³, Hubao Wang³, Lynn Fussner³, Samir Parikh³ and Brad Rovin³, ¹Ohio State University Medical Center, Columbus, OH, ²Broad Institute of MIT and Harvard, Melrose, MA, ³Ohio State University, Columbus, OH
Background/Purpose: The role of complement in patients with ANCA-associated vasculitis (AAV) has been increasingly appreciated and led to the use of complement system antagonists as…
Abstract Number: 0686 • ACR Convergence 2023
Safety and Efficacy of Avacopan in Patients 65 Years and Older with ANCA-Associated Vasculitis
David Jayne¹, Duvuru Geetha², Christian Pagnoux³, Sebastian Sattui⁴ and Peter Merkel⁵, ¹University of Cambridge, Cambridge, United Kingdom, ²Johns Hopkins University, Baltimore, MD, ³Mount Sinai Hospital, Toronto, ON, Canada, ⁴University of Pittsburgh, Pittsburgh, PA, ⁵University of Pennsylvania, Philadelphia, PA
Background/Purpose: Older adults are at increased risk of glucocorticoid (GC)-related toxicity; minimization of GCs is a major focus for treatment of patients with ANCA-associated vasculitis…
Abstract Number: 0687 • ACR Convergence 2023
Avacopan for the Treatment of ANCA-associated Vasculitis. Real World Experience in Spain
Georgina Espigol-Frigole¹, Maria C Cid², Juliana Bordignon Draibe³, Maria Carmen Prados⁴, Elena Guillen⁵, Ana Huerta⁶, Javier Villacorta⁷, Cristina Vega⁸, Judith Martins⁹, Borja Gracia¹⁰ and Enrique Morales¹¹, ¹Autoimmune Diseases. Hospital Clinic Barcelona, Barcelona, Spain, ²Hospital Clinic Barcelona, Barcelona, Spain, ³Hospital Bellvitge, Barcelona, Spain, ⁴Hospital Torrecárdenas, Almeria, Spain, ⁵Hospital Clínic de Barcelona, Barcelona, Spain, ⁶Hospital Puerta de Hierro, Madrid, Spain, ⁷Hospital Ramón y Cajal, Madrid, Spain, ⁸Hospital La Paz, Madrid, Spain, ⁹Hospital de Getafe, Madrid, Spain, ¹⁰Hospital Lozano Blesa, Zaragoza, Spain, ¹¹Hospital 12 de Octubre, Madrid, Spain
Background/Purpose: ANCA-associated vasculitis are chronic and relapsing diseases. Relapses are frequently associated with organ damage accrual as a consequence of disease activity or treatment-related side…
Abstract Number: 0688 • ACR Convergence 2023
Efficacy and Safety Experience with Avacopan Beyond 52 Weeks in the Early Access Program (EAP)
Federico Alberici¹, Carlo Salvarani², Christine Chan³, Achim Obergfell⁴ and Tamara Popov⁴, ¹Universita degli Studi di Brescia, Brescia, Italy, ²Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy, ³CSL Vifor, Redwood City, CA, ⁴CSL Vifor, Glattbrugg, Switzerland
Background/Purpose: Avacopan, a selective C5aR1 inhibitor, has demonstrated efficacy and safety over 52 weeks in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis. However, efficacy and…
Abstract Number: 0736 • ACR Convergence 2023
Deciphering Complement System-dependent Cellular Pathways in Human Rheumatoid Arthritis Synovial Tissue Using Large Single-cell Computational Omics
Juan Vargas¹, Ian Mantel², Nirmal Banda¹, Anna Helena Jonsson³, Kevin Wei⁴, Deepak Rao³, Susan Goodman⁵, Kevin Deane¹, The Accelerating Medicines Partnership SLE/RA¹, Jennifer Anolik⁶, Michael Brenner⁴, Soumya Raychaudhuri³, Jennifer Seifert⁷, Michael Holer¹, Laura Donlin⁵ and Fan Zhang⁸, ¹University of Colorado Anschutz Medical Campus, Aurora, CO, ²Weill Cornell Medicine, New York, NY, ³Brigham and Women's Hospital, Boston, MA, ⁴Brigham and Women's Hospital and Harvard Medical School, Boston, MA, ⁵Hospital for Special Surgery, New York, NY, ⁶University of Rochester Medical Center, Rochester, NY, ⁷the Accelerating Medicines Partnership (AMP) RA/SLE Network, Boston, MA, ⁸University of Colorado, Aurora, CO

Background/Purpose: The complement system is a major component of innate immunity and plays a vital role in autoimmune disease pathogenesis. In patients with rheumatoid arthritis…
Abstract Number: 0094 • ACR Convergence 2023
Granzyme K Elicits a New Pathway for Complement Activation in RA Synovium
Anna Helena Jonsson¹, Carlos Donado², Erin Theisen², Dominique Jones¹, Aparna Nathan³, Fan Zhang⁴, Accelerating Medicines Partnership (AMP): RA/SLE¹, Soumya Raychaudhuri¹ and Michael Brenner², ¹Brigham and Women's Hospital, Boston, MA, ²Brigham and Women's Hospital and Harvard Medical School, Boston, MA, ³Harvard Medical School, Boston, MA, ⁴University of Colorado, Aurora, CO
Background/Purpose: T cells are major drivers of rheumatoid arthritis (RA) pathogenesis. While most research has focused on CD4+ T cells, we have found that CD8+…
Abstract Number: 0857 • ACR Convergence 2023
Change in Albuminuria in Patients with ANCA-Associated Vasculitis Treated with Avacopan
Duvuru Geetha¹, Frank Cortazar², alexandre Karras³, Annette Bruchfeld⁴, Huibin Yue⁵, Peter Merkel⁶ and David Jayne⁷, ¹Johns Hopkins University, Baltimore, MD, ²New York Nephrology, Watervliet, NY, ³HEGP - APHP, Paris, France, ⁴Karolinska Institutet, Stockholm, Sweden, ⁵Amgen, Inc., Thousand Oaks, CA, ⁶University of Pennsylvania, Philadelphia, PA, ⁷University of Cambridge, Cambridge, United Kingdom
Background/Purpose: Urinary albumin:creatinine ratio (UACR) is an important biomarker of active glomerulonephritis, a common complication of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV). In most glomerular…
Abstract Number: 0099 • ACR Convergence 2023
Cell-bound Complement Activation Products in Antiphospholipid Antibody-positive Patients Without Other Systemic Autoimmune Diseases
Doruk Erkan¹, JoAnn Vega¹, Tyler O'Malley² and Andrew Concoff³, ¹Hospital for Special Surgery, New York, NY, ²Exagen, Vista, CA, ³Exagen, Los Angeles, CA
Background/Purpose: Based on animal models, complement activation is part of Antiphospholipid Syndrome (APS) pathogenesis. However, studies investigating complement activation in antiphospholipid antibody (aPL)-positive patients are…
Abstract Number: 0899 • ACR Convergence 2023
Complement Factor I (CFI) Gene Expression by Kidney Tubular Cells Is Increased in Lupus Nephritis Patients with Interstitial Fibrosis and Tubular Atrophy
Shudan Wang¹, John Greally², Masako Suzuki³, Jee-Young Moon², Tao Wang², Yvonne M Saenger², Brad Rovin⁴ and J. Michelle Kahlenberg⁵, ¹Albert Einstein College of Medicine / Montefiore Medical Center, New York, NY, ²Albert Einstein College of Medicine, Bronx, NY, ³Texas A&M University, College Station, TX, ⁴Ohio State University, Columbus, OH, ⁵University of Michigan, Ann Arbor, MI
Background/Purpose: Tubulointerstitial injury is a strong predictor of progression to kidney failure in lupus nephritis (LN). Prior animal studies suggest intrarenal complement activation has an…
Abstract Number: 0257 • ACR Convergence 2023
Activity of IgG4-related Disease Differs Depending on the Presence or Absence of Concomitant Hypocomplementemia
Risa Wakiya¹, Hiroki Ozaki², Hiromi Shimada¹, Shusaku Nakashima¹, Taichi Miyagi³, Yusuke Ushio⁴, Koichi Sugihara⁵, Mao Mizusaki¹, Rina Mino⁶, Kanako Chujo⁷, Ryoko Kagawa⁴, Hayamasa Yamaguchi⁴, Tomohiro Kameda¹ and Hiroaki Dobashi¹, ¹Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Kagawa, Japan, ²Department of Rheumatology, KKR Takamatsu Hospital, Kagawa, Japan, ³Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Kidagun, Japan, ⁴Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa University, Miki-cho, Kita-gun, Japan, ⁵Kagawa University, Miki-cho, Kita-gun, Japan, ⁶Kagawa University, Division of Hematology, Rheumatology and Respiratory Medicine, Department of Internal Medicine, Faculty of Medicine, Kagawa, Japan, ⁷Kagawa University, Miki, Kita District, Kagawa, Japan
Background/Purpose: Hypocomplementemia (HC) is often observed in IgG4-related diseases (IgG4-RD), but there are also IgG4-RD without HC. This study aimed to clarify the difference of…
Abstract Number: 0900 • ACR Convergence 2023
Complement Deposition on Extracellular Mitochondria Induces Platelet Activation in Vitro
Marina Barguil Macedo and Christian Lood, University of Washington, Seattle, WA
Background/Purpose: Mitochondria are extruded upon cell death and platelet activation, being elevated in several inflammatory conditions, including systemic lupus erythematosus (SLE). Mitochondria are immunogenic, with…
Abstract Number: 0556 • ACR Convergence 2023
Classical Complement Activation in Lupus Nephritis Correlates with Disease Biomarkers: Results from Two Observational Studies
Edmund Chang¹, Julian Low¹, Noosha Yousefpour¹, Min Bao¹, Jeannette Osterloh¹, Qing Chang¹, Dean Artis¹, Henk-Andre Kroon¹, Yaisa Andrews-Zwilling¹, Maria Dall'Era², Ted Yednock¹ and Ann Mongan¹, ¹Annexon Biosciences, Brisbane, CA, ²University of California San Francisco, San Francisco, CA
Background/Purpose: Lupus nephritis (LN) is an autoantibody-mediated disease that can activate C1q and the classical complement pathway. Pathogenic anti-C1q antibodies (PACAs) are often present, amplifying…

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