Abstracts tagged "interferons and pediatric rheumatology"

Abstract Number: 3208 • 2016 ACR/ARHP Annual Meeting
Preliminary Response to JAK1/2 Inhibition with Baricitinib in “Candle”,“Savi” and “Candle-like” Diseases. a New Therapeutic Approach for Type I IFN Mediated Autoinflammatory Diseases
Gina A. Montealegre Sanchez¹, Adam Reinhardt², Paul Brogan³, Dawn C. Chapelle⁴, Hanna Kim⁴, Samantha Judd⁴, Bahar Kost⁴, Michelle O'Brien⁴, Wendy Goodspeed⁵, Robert A. Colbert⁴, Meryl Waldman⁶, Deborah L. Stone⁷, Ling Gao⁸, JA Dare⁸, Susanne Schalm⁹, Thomas L. Klausmeier¹⁰, Sara Murias¹¹, Yackov Berkun¹², Diane Brown¹³, John D. Carter¹⁴, Fehime K Eroglu¹⁵, A. Zlotogorski¹⁶, Philip Hashkes¹⁷, Helmut Wittkowski¹⁸, Suzanne Ramsey¹⁹, Seza Ozen²⁰, Adriana Almeida de Jesus²¹ and Raphaela Goldbach-Mansky²², ¹NIAID/NIH, Bethesda, MD, ²Rheumatology, Children's Hosp of Omaha/UNMC, Omaha, NE, ³UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ⁴NIAMS/NIH, Bethesda, MD, ⁵Office of the Clinical Director, NIAMS/NIH, Bethesda, MD, ⁶NIDDK/NIH, Bethesda, MD, ⁷NHGRI/NIH, Bethesda, MD, ⁸University of Arkansas for Medical Sciences, Little Rock, AR, ⁹LMU Munich, Munich, Germany, ¹⁰Riley Hospital for Children, Indianapolis, IN, ¹¹Hospital Infantil La Paz, Madrid, Spain, ¹²Hadassah-Hebrew University Medical Center, Jerusalem, Israel, ¹³Children's Hospital Los Angeles, Los Angeles, CA, ¹⁴Division of Rheumatology, University of South Florida, Tampa, FL, ¹⁵Department of Pediatrics, Hacettepe University Hospitals, Ankara, Turkey, ¹⁶Department of Dermatology, Hadassah-Hebrew University Medical Center, Jerusalem, Israel, ¹⁷Pediatric Rheumatology, Shaare Zedek Medical Center, Jerusalem, Israel, ¹⁸Pediatrics, University of Muenster, Muenster, Germany, ¹⁹Pediatric Rheumatology, IWK Health Centre, Halifax, NS, Canada, ²⁰Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey, ²¹National Institute of Allergy and Infectious Diseases (NIAID), NIH, Bethesda, MD, ²²Translational Autoinflammatory Disease Studies, National Institute of Allergy and Infectious Diseases (NIAID), NIH, Bethesda, MD
Background/Purpose: Chronically elevated serum IP-10 (CXCL10) levels, and a prominent “interferon (IFN)-response gene signature” in patients with chronic neutrophilic dermatosis with lipodystrophy and elevated temperatures…
Abstract Number: 2465 • 2015 ACR/ARHP Annual Meeting
Cytokine Profile Comparison of Monogenic and Complex Conditions with Interferon-Regulated Gene Signatures in Chronic Atypical Neutrophilic Dermatosis with Lipodsytrophy and Elevated Temperature (CANDLE), SAVI, Aicardi-Goutieres Syndrome, JDM, and SLE
Hanna Kim¹, Yin Liu², Adriana Almeida de Jesus¹, Robert Wesley³, Yan Huang¹, Gina A. Montealegre Sanchez¹, Dawn C. Chapelle¹, Wanxia L. Tsai⁴, Massimo G. Gadina⁴, Frederick W. Miller⁵, Sarfaraz Hasni⁶, Adeline Vanderver⁷, Lisa G Rider⁵ and Raphaela Goldbach-Mansky¹, ¹Translational Autoinflammatory Diseases Section, NIAMS, NIH, Bethesda, MD, ²Scientific Review Branch, NIAMS, NIH, Bethesda, MD, ³Clinical Center, National Institutes of Health, Bethesda, MD, ⁴Translational Immunology Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, MD, ⁵Environmental Autoimmunity Group, NIEHS, NIH, Bethesda, MD, ⁶National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ⁷Pediatric Neurology, Children's National Medical Center, Washington, DC
Background/Purpose: An Interferon (IFN) Regulated Gene Signature (IRS) was previously reported in patients with two complex autoimmune diseases, juvenile systemic lupus (JSLE)1 and juvenile dermatomyositis…
Abstract Number: 3076 • 2015 ACR/ARHP Annual Meeting
Interferon Gamma (IFNg) Is the Driving Mediator of Secondary Hemophagocytic Lymphohistiocytosis (sHLH) in TLR9-Mediated Pathogenesis in Mice and Is Correlated to Disease Parameters in Children
Cristina De Min¹, Vanessa Buatois¹, Laurence Chatel¹, Laura Cons², Francoise Richard³, Claudia Bracaglia⁴, Fabrizio De Benedetti⁵, Marie Kosco-Vilbois¹ and Walter Ferlin¹, ¹NovImmune S.A., Geneva, Switzerland, ²NovImmune S.A., Plan-Les-Ouates, Geneva, Switzerland, ³NovImmune SA, Geneva, Switzerland, ⁴Pediatric Medicine, Division of Rheumatology, Rome, Italy, ⁵Division of Rheumatology, Department of Pediatric Medicine, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy
Background/Purpose: Life-threatening hyperinflammatory syndromes caused by severe hypercytokinemia include primary (p) and secondary (s) hemophagocytic lymphohistiocytosis (HLH). Increased circulating levels of interferon gamma (IFNg) in…
Abstract Number: 3097 • 2015 ACR/ARHP Annual Meeting
Innovative Approach for the Identification of an Appropriate Dose Regimen of a Targeted Treatment, NI-0501, an Anti-Interferon Gamma (IFNg) Antibody, in Patients with Hemophagocytic Lymphohistiocytosis (HLH)
Cristina De Min¹, Philippe Jacqmin², Christian Laveille³, Robert Nelson¹, Florence Guilhot¹, Maureen Deehan¹, Marie Kosco-Vilbois¹, Walter Ferlin¹ and Genevieve Lapeyre¹, ¹NovImmune S.A., Geneva, Switzerland, ²SGS Exprimo, Mechelen, Belgium, ³SGS Exprimo NV, Mechelen, Belgium
Background/Purpose: Based on the growing evidence that IFNg plays a pivotal role in HLH, NI-0501, an anti-IFNg monoclonal antibody, is being developed as the first…
Abstract Number: 2190 • 2013 ACR/ARHP Annual Meeting
Clinical and Immunologic Description Of Pediatric Conditions With Interferon-Regulated Gene Signatures (Chronic Atypical Neutrophilic Dermatosis Lipodystrophy Elevated Tempature, Aicardi Goutieres Syndrome, Juvenile Dermatomyositis, Juvenile Systemic Lupus Erythematosus)
Hanna Kim¹, Adriana Almeida de Jesus², Yin Liu³, Yan Huang¹, Gina Montealegre⁴, Dawn Chapelle⁵, Nicole Plass¹, Wanxia Tsai⁶, Massimo Gadina⁷, Lisa G. Rider⁸, Adeline Vanderver⁹ and Raphaela Goldbach-Mansky¹, ¹Translational Autoinflammatory Diseases Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, MD, ²Translational Autoinflammatory Disease Section, NIAMS/NIH, Bethesda, MD, ³Translational Autoinflammatory Disease Section, Office of the Clinical Director, NIAMS/NIH, Bethesda, MD, ⁴Office of the Clinical DIrector, NIAMS / NIH, Bethesda, MD, ⁵Office of the Clinical Director, NIAMS / NIH, Bethesda, MD, ⁶Translational Immunology Section, NIAMS / NIH, Bethesda, MD, ⁷Translational Immunology Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, Bethesda, MD, ⁸Environmental Autoimmunity Group, NIEHS, NIH, Bethesda, MD, ⁹Pediatric Neurology, Children's National Medical Center, Washington, DC
Background/Purpose: Increased interferon (IFN) regulated gene (IRG) expression has been reported in juvenile systemic lupus (JSLE)1 and juvenile dermatomyositis (JDM)2. Recently, two monogenic conditions chronic…

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