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Abstracts tagged "interstitial lung disease"

  • Abstract Number: 038 • 2023 Pediatric Rheumatology Symposium

    HLA-DRB1*15 Alleles in Systemic Juvenile Idiopathic Arthritis with Lung Disease and Macrophage Activation Syndrome in Italy

    Claudia Bracaglia1, Manuela Pardeo1, Maria Troiano2, Giuseppe Testa2, Ivan Caiello1, Arianna De Matteis3, Matteo Trevisan1, Franco Locatelli4, Marco Andreani2 and Fabrizio De Benedetti1, 1Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy, 2Laboratory of Transplant Immunogenetics, Department of Haematology/Oncology, Cell and Gene, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Lazio, Italy, 3Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Roma, Italy, 4Ospedale Pediatrico Bambino Gesù, Department of Hematology/Oncology, Cell and Gene Therapy, Roma, Italy

    Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is characterized by unique clinical features and it is considered as a polygenic autoinflammatory disease. Macrophage activation syndrome (MAS)…
  • Abstract Number: 095 • 2023 Pediatric Rheumatology Symposium

    Systemic Juvenile Idiopathic Arthritis Associated Lung Disease in Europe

    Claudia Bracaglia1, Francesca Minoia2, Christoph Kessel3, Sebastiaan Vastert4, Manuela Pardeo1, Alessia Arduini1, Sarka Fingerhutova5, Irina Nikishina6, Ozge Basaran7, Nural Kiper8, Mikhail Kostik9, Mia Glerup10, Roberta Caorsi11, AnnaCarin Horne12, Giovanni Filocamo2, Helmut Wittkowski3, Marija Jelusic13, Jordi Anton14, Samira Khaldi-Plassart15, Alexandre Belot15, Gerd Horneff16, Seraina Palmer Sarrott17, Elvira Cannizzaro Schneider18, Lampros Fotis19, Pavla Dolezalova5, Angelo Ravelli20, Seza Ozen7 and Fabrizio De Benedetti1, 1Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy, 2Fondazione IRCCS Ca' Grande Ospedale Maggiore Policlinico, Milano, Italy, 3Department of Pediatric Rheumatology & Immunology, WWU Medical Center (UKM), Muenster, Germany, 4Wilhelmina Children’s Hospital, Department of Pediatric Immunology and Rheumatology, Utrecht, The Netherlands, Utrecht, Netherlands, 5Centre for Paediatric Rheumatology and Autoinflammatory Diseases, Department of Paediatrics and Inherited Metabolic Disorders, 1st Faculty of Medicine, Charles University and General University Hospital in Prague, Prague, Czech Republic, 6V.A. Nasonova Research Institute of Rheumatology, Moscow, Russia, 7Department of Pediatrics, Division of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, 8Department of Pediatrics, Division of Pediatric Pulmonology, Hacettepe University, Ankara, Turkey, 9Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russia, 10Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark, 11Department of Pediatrics and Rheumatology, IRRCS Istituto G. Gaslini, Genova, Italy, 12Department of pediatric rheumathology Karolinska University Hospital and Department of pediatrics, Karolinska Institute, Stockholm, Sweden, 13Department of Paediatrics, University of Zagreb School of Medicine, University Hospital Centre, Zagreb, Croatia, 14Pediatric Rheumatology, Hospital Sant Joan de Déu, Universitat de Barcelona, Barcelona, Spain, 15Pediatric Nephrology, Rheumatology, Dermatology Unit, Hôpital Femme Mère Enfant, Hospices Civils de Lyon, Lyon, France, 16Pediatrics, Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany, 17Paediatric Rheumatology University Children’s Hospital Zurich, Zurich, Zurich, Switzerland, 18Paediatric Rheumatology University Children’s Hospital Zurich, Zurich, Switzerland, 19Pediatric Rheumatology Division, 3rd Department of Pediatrics, National and Kapodistrian University of Athens,‘’ATTIKON’’ General University Hospital, Athens, Greece, 20IRRCS Istituto Giannina Gaslini and Università degli Studi di Genova, Genova, Italy

    Background/Purpose: Chronic parenchymal lung disease (LD) is a new emerging severe life-threatening complication of sJIA. The number of sJIA patients with LD is apparently increasing…
  • Abstract Number: 098 • 2023 Pediatric Rheumatology Symposium

    Characterization of Pulmonary Nodules in Juvenile-onset Systemic Sclerosis: A Single Center Case-Series

    Jonathan Li1, Franziska Rosser1, Sameh Tadros1 and Kathryn Torok2, 1UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, 2University of Pittsburgh, Pittsburgh, PA

    Background/Purpose: Given the high prevalence of intestinal lung disease (ILD) in systemic sclerosis (SSc) and the low sensitivity of pulmonary function testing for diagnosing ILD,…
  • Abstract Number: 0245 • ACR Convergence 2022

    Influence of Forced Vital Capacity Impairment on Treatment Selection and Outcomes in RA-ILD Patients Initiating a Biologic or Targeted-Synthetic DMARD

    Bryant England1, Michael George2, Yangyuna Yang1, Punyasha Roul3, Jorge Rojas4, Brian Sauer5, Grant Cannon6, Joshua Baker7, Jeffrey Curtis8 and Ted Mikuls9, 1University of Nebraska Medical Center, Omaha, NE, 2University of Pennsylvania, Philadelphia, PA, 3UNMC, Omaha, NE, 4George E. Wahlen Department of Veterans Affairs Salt Lake City Health Care System, Salt Lake City, UT, 5Salt Lake City VA/University of Utah, Salt Lake City, UT, 6Salt Lake City VA, Salt Lake city, 7University of Pennsylvania and Corporal Michael J. Crescenz VA Medical Center, Philadelphia, 8Division of Clinical Immunology & Rheumatology, University of Alabama at Birmingham, Birmingham, AL, 9Division of Rheumatology, University of Nebraska Medical Center, Omaha, NE

    Background/Purpose: Patients with RA-associated interstitial lung disease (RA-ILD) suffer from substantial morbidity and premature mortality. The optimal use of biologic/tsDMARDs in this population is poorly…
  • Abstract Number: 0920 • ACR Convergence 2022

    Retention Rate and Safety of Biologic or Targeted Synthetic Disease Modifying Anti-rheumatic Drugs (DMARDs) in Patients with Rheumatoid Arthritis Associated with Interstitial Lung Disease: Results from the KOBIO Registry

    Ji-Won Kim1, Sun-Kyung Lee2, Ju-Yang Jung1, Chang-Hee Suh3, Kichul Shin4 and Hyoun-Ah Kim1, 1Ajou University School of Medicine, Suwon, Republic of Korea, 2Hanyang university, Seoul, Republic of Korea, 3Ajou University Hospital, Suwon, Republic of Korea, 4Seoul Metropolitan Government- Seoul National University Boramae Medical Center, Seoul, Republic of Korea

    Background/Purpose: The aim was to evaluate long-term drug retention and safety of biologic (bDMARDs) or targeted synthetic disease modifying anti-rheumatic drugs (tsDMARDs) and to identify…
  • Abstract Number: 1231 • ACR Convergence 2022

    [68Ga]- CPCR4 Trifluoroacetate PET/CT Imaging of Chemokine Receptor CXCR4- Expression in Systemic Sclerosis Related Interstitial Lung Disease

    Chirag Rajkumar Kopp1, Shefali Sharma2, Venkata Subramanian Krishnaraju3, Chandra Bhushan Prasad3, Jagdeep Singh3, Shashi Anand3, Ranjana Minz3, Ashwani Sood3, Anindita Sinha3, Rajender Kumar Basher3, Sahajal Dhooria3, Varun Dhir4 and sanjay jain3, 1Post Graduate Institute of Medical Education and Research, Chandigarh, India, 2PGIMER< Chandigarh, Chandigarh, India, 3Postgraduate Institute of Medical Education and Research, Chandigarh, India, 4PGIMER, CHD, INDIA, Chandigarh, India

    Background/Purpose: Interstitial lung disease (ILD) in Systemic sclerosis (SSc) is a major contributor of morbidity and mortality. CXCR4/CXCL12 axis has been implicated in the pathogenesis…
  • Abstract Number: 1855 • ACR Convergence 2022

    Clinical Outcomes in an Observational Retrospective Cohort of Patients with Connective Tissue Disease-associated Interstitial Lung Disease (CTD-ILD) Who Taper Immunosuppressive Therapy

    Paul Dellaripa, Paul Hoover and Tracy Doyle, Brigham and Women's Hospital, Boston, MA

    Background/Purpose: Patients with CTD-ILD are often treated with a combination of corticosteroids and steroid sparing agents to limit the progression of the inflammatory response in…
  • Abstract Number: 2250 • ACR Convergence 2022

    Genetic and Environmental Risk Factors in Rheumatoid Arthritis-Associated Interstitial Lung Disease

    Austin Wheeler1, Yangyuna Yang1, Joshua Baker2, Jill Poole1, Dana Ascherman3, Gail Kerr4, Andreas Reimold5, Gary Kunkel6, Grant Cannon7, Katherine Wysham8, Namrata Singh9, Deana Lazaro10, Paul Monach11, S. Louis Bridges, Jr.12, Ted Mikuls13 and Bryant England1, 1University of Nebraska Medical Center, Omaha, NE, 2University of Pennsylvania, Philadelphia, PA, 3University of Pittsburgh, Pittsburgh, PA, 4Washington DC VAMC/Georgetown and Howard Universities, Washington, DC, 5University of Texas Southwestern Medical Center, Dallas, TX, 6University of Utah, Salt Lake City, UT, 7Retired, Salt Lake City, UT, 8VA Puget Sound/University of Washington, Seattle, WA, 9University of Washington, Bellevue, WA, 10VA New York Harbor Healthcare system, Short Hills, NJ, 11VA Boston Healthcare System, Boston, MA, 12Hospital for Special Surgery, New York, NY, 13Division of Rheumatology, University of Nebraska Medical Center, Omaha, NE

    Background/Purpose: RA-associated interstitial lung disease (RA-ILD) is an extra-articular manifestation of RA causing substantial morbidity and mortality. Genetic risk variants, such as the MUC5B rs35705950…
  • Abstract Number: 0248 • ACR Convergence 2022

    Monocyte Chemoattractant Protein-1, Vascular Cell Adhesion Protein-1 and Asymmetric Dimethylarginine as Potential Biomarkers of Interstitial Lung Disease Associated with RA

    Veronica Pulito-Cueto1, Sara Remuzgo-Martinez1, Fernanda Genre1, Belén Atienza-Mateo1, Víctor M. Mora-Cuesta1, David Iturbe-Fernández1, Leticia Lera-Gómez1, Diana Prieto-Peña1, Virgi Portilla1, Ricardo Blanco2, Alfonso Corrales1, Oreste Gualillo3, José M. Cifrián1, Raquel Lopez Mejias4 and Miguel Ángel González-Gay5, 1Research 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, 2Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, 3Hospital Clínico Universitario de Santiago, SERGAS (Servizo Galego de Saude) and IDIS (Instituto de Investigación Sanitaria de Santiago), NEIRID Lab (Neuroendocrine Interactions in Rheumatology and Inflammatory Diseases), Research Laboratory 9, Santiago de Compostela, Spain, 4IDIVAL, Santander, Spain, 5Department of Medicine and Psychiatry, Universidad de Cantabria; Rheumatology Division, Hospital Universitario Marqués de Valdecilla; Research group on genetic epidemiology and atherosclerosis in systemic diseases and in metabolic diseases of the musculoskeletal system, IDIVAL, Santander, Spain. Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa

    Background/Purpose: Interstitial lung disease (ILD) is one of the main comorbidities of patients with RA contributing to an increased mortality risk [1]. The pathogenesis of…
  • Abstract Number: 1047 • ACR Convergence 2022

    A Novel Prediction Tool for Progression of Systemic Sclerosis-Interstitial Lung Disease Despite Treatment with Immunosuppression

    Elizabeth Volkmann1, Holly Wilhalme1, Grace Kim1, Jonathan Goldin1, Shervin Assassi2, Masataka Kuwana3, Donald Tashkin1 and Michael roth1, 1University of California Los Angeles, Los Angeles, CA, 2McGovern Medical School, University of Texas, Houston, TX, 3Nippon Medical School Graduate School of Medicine, Tokyo, Japan

    Background/Purpose: Systemic sclerosis-interstitial lung disease (SSc-ILD) is the leading cause of SSc-related death. While some patients respond favorably to treatment with immunosuppression, a subset of…
  • Abstract Number: 1246 • ACR Convergence 2022

    Improving Radiologic Interpretation of Diffuse Lung Disease in Pediatric Rheumatologic (PR) Patients Using Trained Scoring with Semi-quantitative Chest Computed Tomography (CT) Analysis

    Michal Cidon1, Beverley Newman2, Terry Robinson3, Paul Iskander4, Paul Thacker5, Evan Zucker2, Tzielan Lee6 and Rex Moats3, 1Children's Hospital Los Angeles, Los Angeles, CA, 2Stanford Children's Health, Stanford, CA, 3Children's Hospital of Los Angeles, Los Angeles, CA, 4University of California Los Angeles, Los Angeles, CA, 5Mayo Clinic, Rochester, MN, 6Stanford University School of Medicine, Palo Alto, CA

    Background/Purpose: We presented preliminary data in 2021 to support the feasibility of using a semi-quantitative chest CT scoring system (Pediatric Rheumatologic Diffuse Lung Disease Score…
  • Abstract Number: 1857 • ACR Convergence 2022

    Increasing Proportion of MDA-5 Associated Conditions in a Tertiary Myositis Centre Following Onset of the Covid-19 Pandemic

    Jennifer Hannah1, Saadia Sasha Ali1, Sloni Arora1, John Cazabon1, James Galloway1 and Patrick Gordon2, 1King's College London, London, United Kingdom, 2King’s College Hospital NHS Foundation Trust, London, United Kingdom

    Background/Purpose: Anti-MDA5 dermatomyositis is a clinical subtype of DM that is strongly associated with a rapidly progressive phenotype of interstitial lung disease resulting in a…
  • Abstract Number: 2251 • ACR Convergence 2022

    Reduction in Rheumatoid Arthritis-Associated Interstitial Lung Disease Risk in Patients Treated with Tofacitinib

    Matthew Baker1, Yuhan Liu1, Rong Lu1, Janice Lin1, Jason Melehani1 and William Robinson2, 1Stanford University, Stanford, CA, 2Stanford University School of Medicine, Palo Alto, CA

    Background/Purpose: Clinically significant interstitial lung disease (ILD) occurs in roughly 10% of patients with rheumatoid arthritis (RA). There are limited data on the pathogenesis of…
  • Abstract Number: 0249 • ACR Convergence 2022

    Identification of Distinct Peripheral Blood Myeloid Cell Subpopulations in Patients with Rheumatoid Arthritis-associated Interstitial Lung Disease

    Jill Poole1, Bryant England1, Kathryn Cole1, James Talmadge1, Amy Nelson1, Rohit Gaurav1, Aaron Schwabb1, Angela Gleason1, Michael Duryee1, Rhonda Walenz1, Bridget Kramer1, Joel VanDeGraaff1, Sara May1, Geoffrey Thiele1 and Ted Mikuls2, 1University of Nebraska Medical Center, Omaha, NE, 2Division of Rheumatology, University of Nebraska Medical Center, Omaha, NE

    Background/Purpose: Interstitial lung disease (ILD) is associated with significant morbidity and mortality in rheumatoid arthritis (RA). The key cellular players of RA-ILD remain largely unknown.…
  • Abstract Number: 1048 • ACR Convergence 2022

    Nailfold Capillaroscopy and Candidate Biomarker Levels in Systemic Sclerosis-associated Pulmonary Hypertension, Profiling of Non-invasive Markers: A Cross Sectional Study

    Jacqueline Lemmers1, Arjan van Caam2, Brigit Kersten2, Els Ende, van den2, Hanneke KNAAPEN2, Arie Dijk, van2, Wanda Hagmolen of ten Have2, Frank van den Hoogen2, Hans Koenen2, Sander van Leuven3, wynand Alkema4, Ruben Smeets2 and Madelon Vonk5, 1Radboud University Medical Centre Nijmegen, Nijmegen, Netherlands, 2Radboudumc, Nijmegen, Netherlands, 3Radboud University, Nijmegen, Netherlands, 4Hanze University, Groningen the Netherlands, Dept. Data Science for Life Sciences & Health, Groningen, Netherlands, 5Department of Rheumatology, Radboud University Medical Center, Nijmegen, Netherlands

    Background/Purpose: Pulmonary hypertension (PH) is one of the leading causes of death in Systemic Sclerosis (SSc). Early detection and treatment of PH in SSc is…
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