Abstracts tagged "complement"

Abstract Number: 0955 • ACR Convergence 2021
The Effect of Treatment with the Complement C5a Receptor Inhibitor Avacopan on Health-Related Quality of Life in ANCA-Associated Vasculitis
Vibeke Strand¹, Pirow Bekker², Huibin Yue², David R.W. Jayne³ and Peter Merkel⁴, ¹Stanford University School of Medicine, Portola Valley, CA, ²ChemoCentryx, San Carlos, CA, ³University of Cambridge, Cambridge, United Kingdom, ⁴University of Pennsylvania, Philadelphia, PA
Background/Purpose: Avacopan, an oral C5a receptor inhibitor, was evaluated in ANCA-associated vasculitis (AAV). Efficacy and safety results were reported previously. Health-related quality of life (HRQoL)…
Abstract Number: 1086 • ACR Convergence 2021
Lack of Effect of SARS-CoV-2 Vaccination on Cell Bound Complement Activation Products (CB-CAPs), Multianalyte Assay Panel (MAP) with Algorithm, and Inflammatory Biomarkers
Mark Rudolph, Anja Kammensheidt and Roberta Alexander, Exagen Inc., Vista, CA
Background/Purpose: SARS-CoV-2 infection can lead to severe inflammation including increased complement activation (Ma, Kulkarni 2021) and the production of several proinflammatory cytokines. The rapid deployment…
Abstract Number: 1370 • ACR Convergence 2021
Complement Factor D and Factor H Represent Disease and Severity Biomarkers for Systemic Sclerosis Associated Pulmonary Arterial Hypertension (SSc-PAH)
Roberta G Marangoni¹, Changyong Feng¹, Ashley Frazer-Abel², Stephen Tomlinson³, Amy Wielgosz¹, Katie Lutz⁴, Michael Pauciulo⁴, William Nichols⁴, V. Michael Holers⁵, Christopher Ritchlin⁶, R James White III¹ and Benjamin Korman¹, ¹University of Rochester, Rochester, NY, ²University of Colorado Denver, Colorado, CO, ³Medical University of South Carolina, Charleston, SC, ⁴Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁵University of Colorado, Denver, CO, ⁶Division of Allergy, Immunology, and Rheumatology, School of Medicine and Dentistry, University of Rochester Medical Center, Rochester, NY
Background/Purpose: Pulmonary arterial hypertension (PAH) is a severe vascular complication of systemic sclerosis (SSc) and a major cause of mortality. Despite significant advances in the…
Abstract Number: 1371 • ACR Convergence 2021
Symptom Management in Systemic Sclerosis: A Pilot Study of a Web-based Intervention Using Peer-Health Coaches
Susan Murphy¹, Yen Chen², Mary Alore², Sheri Hicks² and Dinesh Khanna², ¹University of Michigan, Grosse Ile, MI, ²University of Michigan, Ann Arbor, MI
Background/Purpose: People with systemic sclerosis (SSc) have a high burden of chronic symptoms that have dramatic effects on function and quality of life. Fatigue, along…
Abstract Number: 1493 • ACR Convergence 2021
Association of the Soluble Terminal Complement Complex C5b-9 (sC5b-9) with Urinary Signs of Kidney Disease in a Swiss SLE Cohort
Kristin Schmiedeberg¹, Ruediger B. Mueller², Thomas Neumann³, Ian Pirker¹, Philipp Rein⁴, Camillo Ribi⁵, Andrea Rubbert-Roth⁶, Michael Kirschfink⁷, Jutta Schroeder-Braunstein⁷, Reinhard Voll⁸ and Johannes von Kempis¹, ¹Kantonsspital St. Gallen, St.Gallen, Switzerland, ²Clinic of Rheumatology, Medical University Hospital Aarau, Aarau, Switzerland, ³Kantonsspital St.Gallen, St.Gallen, Switzerland, ⁴Landeskrankenhaus Hohenems, Wolfurt, Austria, ⁵CHUV, Lausanne, Switzerland, ⁶Kantonspital St Gallen, St.Gallen, Switzerland, ⁷Universitätsklinikum Heidelberg, Heidelberg, Germany, ⁸Department of Rheumatology and Clinical Immunology, University Medical Center, Faculty of Medicine, Albert-Ludwigs-University of Freiburg, Freiburg, Germany
Background/Purpose: Few reliable laboratory biomarkers exist to determine disease activity in SLE. The role of the soluble terminal complement complex, sC5b-9, in active SLE has…
Abstract Number: 1504 • ACR Convergence 2021
Association Between Anti-RNP Antibodies and Interferon Gene Expression but Not Complement Consumption in SLE
Erika Hubbard¹, David Pisetsky² and Peter Lipsky¹, ¹AMPEL BioSolutions, Charlottesville, VA, ²Duke University Medical Center, Durham, NC
Background/Purpose: Anti-nuclear antibodies are important serologic features of SLE and facilitate diagnosis. Anti-double stranded DNA (dsDNA) antibodies are routinely monitored for disease prognosis and are…
Abstract Number: 0085 • ACR Convergence 2020
Classifications of Inflammatory Myopathies: Differentially Expressed Membrane-Bound Complement Regulators Allow Specific Patterns of Membrane Attack Complex Deposition
Fadi Charouf¹, Netanel Karbian¹, Itamar Altman¹, Yakov Fellig¹ and Dror Mevorach¹, ¹Hadassah-University Hospital, Jerusalem, Yerushalayim, Israel
Background/Purpose: Idiopathic inflammatory myopathies (IIM) are a group of heterogeneous disorders that typically present with proximal muscle weakness. According to the classification criteria, IMs are…
Abstract Number: 0262 • ACR Convergence 2020
SLE Pregnancies: C4 as Predictor of Flares and Adverse Pregnancy Outcomes
Francesca Crisafulli¹, Laura Andreoli¹, Matteo Filippini¹, Micaela Fredi¹, Maria Chiara Gerardi¹, Roberto Gorla², Maria Grazia Lazzaroni¹, Daniele Lini¹, Cecilia Nalli², Marco Taglietti¹, Andrea Lojacono³, Sonia Zatti³, Cristina Zanardini³, Franco Franceschini¹ and Angela Tincani¹, ¹Rheumatology and Clinical Immunology Unit, Spedali Civili and University of Brescia, Brescia, Lombardia, Italy, ²Rheumatology and Clinical Immunology Unit, ASST Spedali Civili of Brescia, Brescia, Lombardia, Italy, ³Obstetrics and Gynecology Unit, Spedali Civili and University of Brescia, Brescia, Lombardia, Italy
Background/Purpose: SLE pregnancies have an increased risk of Adverse Pregnancy Outcomes (APO). In clinical practice, low C3 and C4 levels are associated with active disease…
Abstract Number: 0528 • ACR Convergence 2020
The Complex Relationship Between C4b Binding Protein, Warfarin and Antiphospholipid Antibodies
Giorgia Grosso¹, Kerstin Sandholm², Aleksandra Antovic¹, Iva Gunnarsson¹, Agneta Zickert¹, Anna Vikerfors³, Lennart Truedsson⁴, Maria Bruzelius¹, Bo Nilsson⁵, Kristina Nilsson Ekdahl⁵ and Elisabet Svenungsson¹, ¹Karolinska University Hospital, Stockholm, Sweden, ²Linnaeus Center for Biomaterials Chemistry, Linnaeus University, Kalmar, Sweden, ³Swedish Medical Products Agency, Uppsala, Sweden, ⁴Lunds University, Lund, Sweden, ⁵Uppsala University, Uppsala, Sweden
Background/Purpose: C4b Binding Protein (C4BP) is the main inhibitor of the classical complement pathway. Both β2glycoprotein-I (β2GPI), the main antigen in the antiphospholipid syndrome (APS),…
Abstract Number: 0938 • ACR Convergence 2020
Low Preconceptional Complement Level Is Related with an Adverse Obstetric Outcome in a Multicentric Cohort of Pregnancy in Patients with APS and aPL Positivity
Cecilia Nalli¹, Daniele Lini², Laura Andreoli², Francesca Crisafulli², Micaela Fredi², Maria Grazia Lazzaroni², Victoria Bitsadze³, Antonia Calligaro⁴, Valentina Canti⁵, Roberto Caporali⁶, Francesco Carubbi⁷, Cecilia Chighizola⁸, Paola Conigliaro⁹, Caterina De Carolis¹⁰, Teresa Del Ross⁴, Maria Favaro⁴, Maria Gerosa¹¹, Annamaria Iuliano¹², Jamilya Khizroeva³, Alexander Makatsariya³, Pierlugi Meroni¹³, Marta Mosca¹⁴, Melissa Padovan¹⁵, Roberto Perricone⁹, Patrizia Rovere-Querini¹⁶, Gian Domenico Sebastiani¹², Chiara Tani¹⁴, Marta Tonello⁴, Dina Zucchi¹⁴, Franco Franceschini¹⁷ and Angela Tincani², ¹Rheumatology and Clinical Immunology Unit, ASST Spedali Civili of Brescia, Brescia, Lombardia, Italy, ²Rheumatology and Clinical Immunology Unit, Spedali Civili and University of Brescia, Brescia, Lombardia, Italy, ³Department of Obstetrics and Gynecology of I.M. Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation (Sechenov University), Moscow, Russia, ⁴Rheumatology Unit, Department of Medicine, University Hospital of Padua, Padua, Italy, ⁵Immunology, Rheumatology, Allergology and Rare Disease-IRCCS San Raffaele Hospital, Laboratory of Autoimmunity and vascular inflammation San Raffaele, Milan, Italy, ⁶Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy, ⁷Department of Biotechnological and Applied Clinical Science, Rheumatology Unit, School of Medicine, University of L'Aquila, L'Aquila, Italy, ⁸Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy; Experimental Laboratory of Immunorheumatological Researches, Istituto Auxologico Italiano, Milan, Italy, ⁹Rheumatology, Allergology and Clinical Immunology, Department of "Medicina dei Sistemi", University of Rome Tor Vergata, Rome, Italy, ¹⁰Polymedical Center for Prevention of Recurrent Spontaneous Abortion, Rome, Italy, ¹¹Division of Rheumatology, Department of Clinical Sciences and Community Health, Ospedale Gaetano Pini, University of Milan, Milan, Italy, ¹²Rheumatology Unit, Azienda Ospedaliera San Camillo-Forlanini, Rome, Italy, ¹³Division of Rheumatology, ASST.G Pini, Department of Clinical Sciences and Community Health, University of Milan and Istituto Auxologico Italiano, Milan, Italy, ¹⁴Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy, ¹⁵UO e Sezione di Reumatologia, Dipartimento di Scienze Mediche, Universita' degli Studi di Ferrara, Ferrara, Italy, ¹⁶Unit of Immunology, Rheumatology, Allergy and Rare Diseases, Università Vita-Salute San Raffaele - IRCCS Ospedale San Raffaele, Milan, Italy, ¹⁷Rheumatology and Clinical Immunology, Spedali Civili and University of Brescia, Brescia, Italy
Background/Purpose: The role of complement in the aPL related pathology has been widely studied in animal models. aPL can induce fetal loss in experimental animals but…
Abstract Number: 1510 • ACR Convergence 2020
Platelet-bound C4d Is Associated with Platelet Activation and Arterial Thrombotic Events
Yevgeniya Gartshteyn¹, Adam Mor², Daichi Shimbo², Leila Khalili³, Teja Kapoor⁴, Laura Geraldino-Pardilla⁵, Roberta Vezza Alexander⁶, Thierry Dervieux⁷ and Anca Askanase², ¹Columbia University College of Physicians and Surgeons, Glen Rock, NJ, ²Columbia University College of Physicians and Surgeons, New York, NY, ³Columbia University Medical Center, New Haven, CT, ⁴Columbia University College of Physicians and Surgeons, Leonia, NJ, ⁵Division of Rheumatology, Columbia University Vagelos College of Physicians and Surgeons, New York, ⁶Exagen Inc, Vista, CA, ⁷Prometheus Biosciences Inc, Irvine, CA
Background/Purpose: Platelets have a well-defined role in arterial thrombosis, and platelet-bound complement activation products (PC4d) correlate with vascular thromboses in patients with Systemic Lupus Erythematosus…
Abstract Number: 1636 • ACR Convergence 2020
8 Years Follow-Up of a Novel Autoinflammatory Disease: CD59 Malfunction Causes Hemolytic Anemia, Recurrent Guillain-Barre Syndrome, and Strokes in Pediatric Populations and Respond Well to Eculizumab and Pozelimab
Dror Mevorach¹ and Netanel Karbian¹, ¹Hadassah-University Hospital, Jerusalem, Yerushalayim, Israel
Background/Purpose: In 2013 we have described the first patients with a novel autoinflammatory disease manifested in 4 children with recurrent Guillain-Barre syndrome and hemolytic anemia…
Abstract Number: 1670 • ACR Convergence 2020
Low Copy Number of Long C4 Genes Is a Genetic Risk Factor for Childhood Onset SLE (cSLE) but Is Associated with Higher Age of Disease Onset
Fatima Barbar-Smiley¹, Danlei Zhou², Joanne Drew², Bi Zhou², Cagri Yildirim-Toruner², Vidya Sivaraman³, Wael Jarjour⁴, Stacy Ardoin² and Chack-Yung Yu⁵, ¹Nationwide Children's Hospital/The Ohio State University, Columbus, OH, ²Nationwide Children's Hospital, Columbus, OH, ³Nationwide Children's Hospital, Bexley, OH, ⁴The Ohio State University, Columbus, OH, ⁵Abigail Wexner Research Institute at Nationwide Children's Hospital, Columbus, OH
Background/Purpose: Hypocomplementemia is a marked feature of systemic lupus erythematosus (SLE), which may be a result of consumption initiated by immune complexes between self-nuclear antigens…
Abstract Number: 1791 • ACR Convergence 2020
Renal Tubular Complement C9 Deposition Is Associated with Renal Tubular Damage and Fibrosis in Lupus Nephritis
Shudan Wang¹, Ming Wu², Luis Chiriboga², Beatrice Goilav³, Shuwei Wang⁴, Chaim Putterman⁵, Daniel Schwartz⁶, James Pullman⁶, Anna Broder⁷ and H. Michael Belmont⁸, ¹Montefiore Medical Center / Albert Einstein College of Medicine, Bronx, NY, ²NYU Langone Health, New York, ³The Children’s Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, NY, ⁴Morristown Medical Center, Morristown, NJ, ⁵Albert Einstein College of Medicine, Bronx, NY, ⁶Montefiore Medical Center, Bronx, NY, ⁷Albert Einstein College of Medicine/Montefiore Medical Center, Bronx, NY, ⁸New York University, New York, NY
Background/Purpose: Tubulointerstitial damage in lupus nephritis (LN) is a strong predictor of progression to chronic kidney disease (CKD) and end stage renal disease (ESRD). While…
Abstract Number: 1792 • ACR Convergence 2020
Platelet-bound C4d Is Associated with an Increased Risk of Arterial and Venous Thromboses in SLE
Yevgeniya Gartshteyn¹, Roberta Vezza Alexander², John Conklin³, Thierry Dervieux⁴ and Anca Askanase⁵, ¹Columbia University College of Physicians and Surgeons, Glen Rock, NJ, ²Exagen Inc, Vista, CA, ³Exagen Inc., Vista, CA, ⁴Prometheus Biosciences Inc, San Diego, CA, ⁵Columbia University College of Physicians and Surgeons, New York, NY
Background/Purpose: Platelet-bound complement activation products (PC4d), defined as PC4d20 net mean fluorescent intensity [MFI], or a thrombotic risk score that includes PC4d, C3 and anti-phosphatidylserine/prothrombin…

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