Abstracts tagged "Lung Disease"

Abstract Number: 926 • 2018 ACR/ARHP Annual Meeting
Truncating Mutations in SAMD9L Cause an Early-Onset Immune-Dysregulatory Syndrome of Neutrophilic Panniculitis, Interstitial Lung Disease and Cytopenias
Adriana Almeida de Jesus¹, Bernadette Marrero¹, Gina A. Montealegre Sanchez¹, Jon (Sandy) Burnham², Alice Chan³, Yuriy Stepanovskiy⁴, Angela Rösen-Wolff⁵, Christian Hedrich⁶, MinAe Lee-Kirsch⁵, Joseph A. Duncan⁷, Jin Yao Mo⁷, Liliana Bezrodnik⁸, Gisela Seminario⁸, Maria Soledad Caldirola⁸, Eric Allenspach⁹, Troy R. Torgerson¹⁰, Laura Finn¹¹, Rachel VanTries¹, Yan Huang¹, Stephen R. Brooks¹², Zuoming Deng¹³ and Raphaela Goldbach-Mansky¹, ¹Translational Autoinflammatory Disease Section (TADS), Laboratory of Clinical Investigation and Microbiology (LCIM), NIAID/NIH, Bethesda, MD, ²Pediatric Rheumatology, Children's Hospital of Philadelphia, Philadelphia, PA, ³Pediatrics, UCSF, San Francisco, CA, ⁴Shupyk National Academy of Postgraduate Education, Kyiv, Ukraine, ⁵Children's Hospital Dresden, Dresden, Germany, ⁶Pediatric Rheumatology and Immunology, Children's Hospital Dresden, Dresden, Germany, ⁷University of North Carolina School of Medicine, Chapel Hill, NC, ⁸Center of Clinical Immunology, Buenos Aires, Argentina, ⁹Immunology/Rheumatology, Seattle Children's Research Institute, Seattle, WA, ¹⁰Center for Immunity and Immunotherapies, Seattle Children's Research Institute, Seattle, WA, ¹¹University of Washington and Seattle Children's Hospital, Seattle, WA, ¹²Biodata Mining and Discovery Section, Office of Science and Technology, NIAMS/NIH, Bethesda, MD, ¹³NIAMS/NIH, Bethesda, MD
Background/Purpose: The Sterile Alpha Motif Domain Containing 9 Like protein that is encoded by SAMD9L plays a role in endosome fusion, and deletions (haploinsufficiency) of…
Abstract Number: 1118 • 2018 ACR/ARHP Annual Meeting
Identification of Transcriptional Regulatory Networks in Systemic Sclerosis
Yue Wang¹, Jennifer Franks² and Michael L. Whitfield³, ¹Molecular and Systems Biology, Geisel School of Medicine at Dartmouth, Hanover, NH, ²Geisel School of Medicine at Dartmouth, Hanover, NH, ³Biomedical Data Science, Geisel School of Medicine at Dartmouth, Hanover, NH
Background/Purpose: Systemic sclerosis (SSc) is a heterogeneous autoimmune disorder with poor outcomes and no FDA-approved therapies. Prior work has shown gene expression patterns associated with…
Abstract Number: 1123 • 2018 ACR/ARHP Annual Meeting
Plasmacytoid Dendritic Cells in Bronchoalveolar Lavage Correlate with Pulmonary Artery Diameter and Infiltrate Perivascular Areas in the Lungs from Patients with Systemic Sclerosis Associated Pulmonary Hypertension
Isela Valera¹, Anagha Divekar², Dinesh Khanna³, Fereidoun Abtin⁴, Rajan Saggar⁴ and Ram R. Singh⁴, ¹Autoimmunity and Tolerance Laboratory, Division of Rheumatology, Department of Medicine, UCLA, Los Angeles, CA, ²Biolegend, Sa Diego, CA, ³Division of Rheumatology, Department of Internal Medicine, University of Michigan Scleroderma Program, University of Michigan, Ann Arbor, MI, ⁴UCLA, Los Angeles, CA
Background/Purpose: In patients with systemic sclerosis (SSc), plasmacytoid dendritic cells (pDCs) increase in the bronchoalveolar lavage (BAL) and lung tissues (Kafaja S, et al, JCI…
Abstract Number: 1486 • 2017 ACR/ARHP Annual Meeting
Interstitial Lung Disease in Primary Sjögren’s Syndrome : Clinical Presentation, Serological Biomarkers and Long Term Outcome
Chiara Baldini¹, Ilaria Puxeddu², Martina Orlandi³, Francesco Ferro⁴, Elena Elefante⁴, Nicoletta Luciano⁴, Marco Matucci-Cerinic³, Paola Migliorini² and Marta Mosca⁴, ¹Internal Medicine, Rheumatology Unit, University of Pisa, Pisa, Italy, ²Allergology and Clinical Immunology Unit, University of Pisa, Pisa, Italy, ³Rheumatology Unit, University of Florence, Florence, Italy, ⁴Rheumatology Unit, University of Pisa, Pisa, Italy
Background/Purpose: Interstitial lung disease (ILD) is a rare but potentially severe manifestation of primary Sjögren’s syndrome (pSS). The aims of this study were to: 1)…
Abstract Number: 1488 • 2017 ACR/ARHP Annual Meeting
Correlation between Lung Ultrasound, HRCT Findings and Pulmonary Function Tests in Primary Sjögren’s Syndrome (pSS)-Associated Interstitial Lung Disease
Francesco Ferro¹, Alessandra Bulleri², Andrea Delle Sedie¹, Simone Barsotti¹, Nicoletta Luciano¹, Elisa Cioffi³, Elena Elefante¹, Gianfranco Puppo⁴, Marta Mosca¹ and Chiara Baldini⁵, ¹Rheumatology Unit, University of Pisa, Pisa, Italy, ²Radiology Unit, University of Pisa, Pisa, Italy, ³Rheumatology Unit, Rheumatology Unit, University of Pisa, Pisa, Italy, ⁴Pneumology Unit, University of Pisa, Pisa, Italy, ⁵Internal Medicine, Rheumatology Unit, University of Pisa, Pisa, Italy
Background/Purpose: Recently, pleural irregularity (PI), a new lung ultrasound (US) sign, has been proposed as an emerging tool for the diagnosis of interstitial lung disease…
Abstract Number: 1722 • 2017 ACR/ARHP Annual Meeting
Identification of a Sub-Population of Autoantibodies Targeting BICD2 Cross-Reacting with CENP-a Derived Peptides in Patients with Systemic Sclerosis
Michael Mahler¹, Chelsea Bentow¹, Jay Milo¹, May Choi², Mianbo Wang³, Petra Budde⁴, Murray Baron⁵, Marie Hudson⁶ and Marvin J. Fritzler⁷, ¹Research and Development, Inova Diagnostics, San Diego, CA, ²University of Calgary, Calgary, AB, Canada, ³Lady Davis Institute for Medical Research, Montreal, QC, Canada, ⁴Protagen AG, Dortmund, Germany, ⁵Rheumatology, Jewish General Hospital, Montreal, QC, Canada, ⁶Division of Rheumatology, Jewish General Hospital, Lady David Institute for Medical Research, Montreal, QC, Canada, ⁷Medicine, University of Calgary, Calgary, AB, Canada
Background/Purpose: Anti-nuclear antibodies (ANA) are present in approximately 90% of sera from systemic sclerosis (SSc) patients and play an important role in the diagnosis and…
Abstract Number: 1756 • 2017 ACR/ARHP Annual Meeting
Interstitial Lung Disease in ANCA Associated Vasculitis: A Distinct or an Incomplete Subset in ANCA Vasculitis Patients?
Simone Barsotti^1,2, Francesco Ferro¹, Elena Elefante¹, Rossella Neri³, Marta Mosca¹ and Chiara Baldini⁴, ¹Rheumatology Unit, University of Pisa, Pisa, Italy, ²Department of Medical Biotechnologies, University of Siena, Siena, Italy, ³Rheumatology Unit, University of Pisa, PISA, Italy, ⁴Internal Medicine, Rheumatology Unit, University of Pisa, Pisa, Italy
Background/Purpose: ANCA-associated vasculitis (AAV) are heterogeneous disorders with a clinical presentation that may range from a full-blown disease to incomplete forms with isolated organ involvement.…
Abstract Number: 2386 • 2017 ACR/ARHP Annual Meeting
The Risk Factors of Newly Developing/Worsening Pulmonary Abnormalities in Rheumatoid Arthritis
Ayae Tanaka¹, Kazuhiro Kurasawa¹, Yuta Takamura¹, Tomoyuki Miyao¹, Ryutaro Yamazaki², Satoko Arai¹, Takayoshi Owada¹, Reika Maezawa¹ and Masafumi Arima¹, ¹Rheumatology, Dokkyo Medical University, Mibu, Tochigi, Japan, ²Rheumatology, Dokkyo Medical University, Mibu, tochigi, Japan
Background/Purpose: Rheumatoid arthritis (RA) is a systemic inflammatory disease that affects various organs including the lung. The pulmonary involvement is critical for prognosis of the…
Abstract Number: 2566 • 2017 ACR/ARHP Annual Meeting
Myxomavirus-Derived Serpin (Serp-1) Reduces Diffuse Alveolar Hemorrhage in a Murine Model of Lupus
Haoyang Zhuang¹, Shuhong Han¹, Lijun Yang², Sriram Ambadapadi³, Alexandra Lucas⁴ and Westley Reeves⁵, ¹Medicine, University of Florida, Gainesville, FL, ²Pathology, Immunology and laboratory medicine, University of Florida, Gainesville, FL, ³Arizona state University, Tempe, AZ, ⁴Arizona State University, Tempe, AZ, ⁵Rheumatology & Clinical Immology, University of Florida, Gainesville, FL
Background/Purpose: Diffuse alveolar hemorrhage (DAH) is an unusual complication of lupus with over 50% mortality. In both humans and mice, DAH is associated with ANCA-negative…
Abstract Number: 2688 • 2017 ACR/ARHP Annual Meeting
Collagen Metabolite Biomarker Levels Are Associated with the Amount of Fibrosis in Internal Organs in Systemic Sclerosis Patients
Anne Sofie Siebuhr¹, Satoshi Kubo², Pernille Juhl³, K Nakano⁴, S Nakayamada⁵, Morten Karsdal⁶, Anne-C. Bay-Jensen⁷ and Yoshiya Tanaka⁸, ¹the first department of internal medicine, University of occupational and environmental health, Fukuoka, Japan, ²The first department of internal medicine, University of Occupational en environmental Health, Fukuoka, Japan, ³Nordic Bioscience, Herlev, Denmark, ⁴the first department of internal medicin, University of occupational and environmental health, fukuoka, Japan, ⁵The first department of internal medicine, University of Occupational and environmental Health, fukuoka, Japan, ⁶Biomarkers and Reseacrh, Nordic Bioscience, Herlev, Denmark, ⁷Biomarkers and Reseach, Nordic Bioscience, Herlev, Denmark, ⁸The First Department of Internal Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan
Background/Purpose: Biomarkers that can monitor the fibrotic burden will aid in trial enrichment and personalized health care in SSc. SSc is associated with internal organ…
Abstract Number: 2737 • 2017 ACR/ARHP Annual Meeting
Pulmonary Manifestations of Primary Systemic Vasculitides
Jean-Paul Makhzoum¹, Raashid Luqmani², Richard A. Watts³, Anthea Craven⁴, Peter A. Merkel⁵ and Christian Pagnoux⁶, ¹Rheumatology, Vasculitis Clinic, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada, ²Botnar Research Centre, University of Oxford, Oxford, United Kingdom, ³Rheumatology Department, The Ipswich Hospital, Ipswich, Great Britain, ⁴Nuffield Department of Orthopaedics Rheumatology and Musculoskeletal Sciences, Botnar Research Centre, Oxford University Hospitals, Oxford, United Kingdom, ⁵Division of Rheumatology, University of Pennsylvania; Perelman School of Medicine, Philadelphia, PA, ⁶Rheumatology-Vasculitis clinic, Mount Sinai Hospital, University of Toronto, Toronto, ON, Canada
Background/Purpose: Pulmonary involvement in systemic primary vasculitides is diverse and occurs with variable incidence depending on the type of vasculitis. This study aimed to describe…
Abstract Number: 2776 • 2017 ACR/ARHP Annual Meeting
Plasmacytoid Dendritic Cells in Systemic Fibrosis: Pathogenic Role in Bleomycin-Induced Fibrosis Model and Correlation with Disease in Patients with Systemic Sclerosis
Suzanne Kafaja¹, Isela Valera², Anagha Divekar³, Rajan Saggar⁴, Dinesh Khanna⁵, Daniel E. Furst⁶ and Ram R. Singh⁷, ¹Department of Internal Medicine, University of California Los Angeles, David Geffen School of Medicine, Division of Rheumatology, Los Angeles, CA, ²Autoimmunity and Tolerance Laboratory, Division of Rheumatology, Department of Medicine, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, CA, ³Biolegend, Sa Diego, CA, ⁴Medicine, University of California, Los Angeles, David Geffen School of Medicine, Los Angeles, CA, ⁵University of Michigan, Ann Arbor, MI, ⁶David Geffen School of Medicine at UCLA, Los Angeles, CA, ⁷Autoimmunity and Tolerance Laboratory, Department of Medicine/Rheumatology, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, CA
Background/Purpose: Fibrosis is the end-result of most inflammatory conditions, but its pathogenesis remains unclear. Studies in patients and animal models suggest a role for T-cells…
Abstract Number: 24 • 2017 ACR/ARHP Annual Meeting
Trafficking of Innate B Cells to the Lungs As a Novel Mechanism in a Model of Pulmonary Lupus and Vasculitis
Priti Prasad¹, Michael Fishbein², Rohan Sharma³, Ramesh Halder⁴, Isela Valera¹ and Ram R. Singh⁵, ¹Autoimmunity and Tolerance Laboratory, Division of Rheumatology, Department of Medicine, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, CA, ²Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at University of California Los Angeles, Los ángeles, CA, ³Autoimmunity and Tolerance Laboratory, Division of Rheumatology, Department of Medicine, David Geffen School of Medicine at University of California Los Angeles, Los angeles, CA, ⁴Autoimmunity and Tolerance Laboratory, Division of Rheumatology, Department of Medicine, David Geffen School of Medicine at University of California Los Angeles, Los ángeles, CA, ⁵Autoimmunity and Tolerance Laboratory, Department of Medicine/Rheumatology, David Geffen School of Medicine at University of California Los Angeles, Los Angeles, CA
Background/Purpose: Lung is involved in up to 50% patients with SLE. Among manifestations of pulmonary lupus, pneumonitis, vasculitis and diffuse pulmonary hemorrhage (DPH) carry a…
Abstract Number: 257 • 2017 ACR/ARHP Annual Meeting
The Use of Positron Emission Tomography (PET)-Scan for the Quantitative Assessment of Interstitial Lung Disease in Systemic Sclerosis
Daphne Peelen¹, Ben Zwezerijnen², Esther Nossent¹, Lilian Meijboom¹, Otto Hoekstra³, Conny van der Laken⁴ and Alexandre Voskuyl⁴, ¹VUmc, Amsterdam, Netherlands, ²Nuclear Medicine, VUmc, Amsterdam, Netherlands, ³Department of Radiology & Nuclear Medicine, VU University Medical Center, Amsterdam, The Netherlands, Amsterdam, Netherlands, ⁴Department of Rheumatology, Amsterdam Rheumatology and immunology Center - location VU University Medical Center, Amsterdam, The Netherlands, Amsterdam, Netherlands
Background/Purpose: Interstitial lung disease (ILD) in systemic sclerosis is treated by immunosuppressive drugs (e.g. cyclophosphamide), aimed at reduction of inflammatory response . Differentiation between inflamed…
Abstract Number: 400 • 2017 ACR/ARHP Annual Meeting
Combination of the Collagen-Induced Arthritis and Organic Dust-Induced Airway Inflammation Models As a Model of Interstitial Lung Disease in Rheumatoid Arthritis
Katherine Janike¹, Jill Poole¹, Geoffrey M. Thiele², Michael J. Duryee³, Lynell W. Klassen⁴, Amy Nelson⁵, Kristi Warren⁶, Benjamin Swanson⁷ and Ted R. Mikuls⁸, ¹Medicine, University of Nebraska Medical Center, Omaha, NE, ²Int Med/Sec of Rheum/Immun, Univ of NE Medical Ctr, Omaha, NE, ³Internal Medicine Division of Rheumatology, University of Nebraska Medical Center, Omaha, NE, ⁴Department of Internal Medicine, University of Nebraska Medical Center, Omaha, NE, ⁵Department of Medicine, University of Nebrasa Medical Center, Omaha, NE, ⁶Department of Medicine, University of Nebraska Medical Center, Omaha, NE, ⁷Department of Pathology, University of Nebraska Medical Center, Omaha, NE, ⁸Internal Medicine, Division of Rheumatology, University of Nebraska Medical Center, Omaha, NE
Background/Purpose: Rheumatoid Arthritis (RA) is characterized by extra-articular involvement including interstitial/inflammatory lung disease (ILD). Whereas the coexistence of RA and ILD is known, mechanisms…

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