Abstracts tagged "innate immunity"

Abstract Number: 0461 • ACR Convergence 2020
High-Throughput Single-Cell Analysis Reveals Unique Lung Cellular Subsets in a Murine Model of Rheumatoid Arthritis-Inflammatory Lung Disease
Rohit Gaurav¹, Ted Mikuls¹, Geoffrey Thiele¹, Amy Nelson¹, Meng Niu¹, Chittibabu Guda¹, James Eudy¹, Austin Barry¹, Debra Romberger¹, Michael Duryee¹, Bryant England¹ and Jill Poole¹, ¹University of Nebraska Medical Center, Omaha, NE
Background/Purpose: Rheumatoid arthritis (RA)-associated inflammatory lung disease is an extra-articular manifestation of RA associated with increased morbidity and mortality, whose precise molecular mechanisms remain undetermined.…
Abstract Number: 0462 • ACR Convergence 2020
Lupus-like Autoimmunity and Increased Interferon Response in Patients with STAT3-deficient Hyper-IgE Syndrome
Brian Dizon¹, Rishi Goel², Shuichiro Nakabo², Amanda Urban², Meryl Waldman², Lilian Howard², Dirk Darnell², Munir Buhaya², Sarfaraz Hasni³, Mariana Kaplan⁴, Alexandra Freeman² and Sarthak Gupta¹, ¹National Institutes of Health, BETHESDA, MD, ²National Institutes of Health, Bethesda, ³Lupus Clinical Trials Unit, NIAMS/NIH, Bethesda, MD, ⁴National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD
Background/Purpose: Autosomal dominant hyper-IgE syndrome (AD-HIES), also known as Job’s syndrome, is a rare primary immunodeficiency caused by dominant-negative loss-of-function (LOF) mutations in signal transducer…
Abstract Number: 0463 • ACR Convergence 2020
Molecular Diagnosis of Childhood Immunodysregulation, Endocrinopathy and Enteropathy X-linked (IPEX)-Like Syndrome and Implications for Clinical Management
Sarah Baxter¹, Tom Walsh¹, Silvia Casadei¹, Suleyman Gulsuner¹, Eric Allenspach², David Hagin³, Gesmar Segundo⁴, Troy Torgerson⁵ and Mary-Claire King¹, ¹University of Washington, Seattle, ²Seattle Children's Research Institute, Seattle, ³Tel Aviv Medical Center, Tel Aviv, Israel, ⁴Universidade Federal de Uberlandia, Uberlandia, Brazil, ⁵Allen Institute, Seattle
Background/Purpose: Patients with early-onset immunodysregulation, endocrinopathy and enteropathy but without identified mutations in FOXP3 are termed “IPEX-like,” and undergo trial-and-error immunosuppressive treatment with highly variable…
Abstract Number: 0469 • ACR Convergence 2020
IFNγ Is Essential for Alveolar Macrophage Driven Lung Inflammation in Macrophage Activation Syndrome
Denny Gao¹, Maggie Henderlight¹, Christopher Woods¹, Alexei Grom¹, Sherry Thornton¹, Michael Jordan¹, Katheryn Wikenheiser-Brokamp¹ and Grant Schulert², ¹Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²PRCSG, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH
Background/Purpose: Macrophage activation syndrome (MAS) is a life-threatening cytokine storm syndrome frequently complicating systemic juvenile idiopathic arthritis (SJIA) and driven by IFNγ. MAS is also…
Abstract Number: 0644 • ACR Convergence 2020
Characterizations of Cytokine Storm Associated with COVID19
Ofer Perzon¹, Avi Abutbul¹, Sigal Sviri¹ and Dror Mevorach¹, ¹Hadassah-University Hospital, Jerusalem, Yerushalayim, Israel
Background/Purpose: COVID-19, the name given to the clinical syndrome associated with the newly recognized virus SARS-CoV-2 has become pandemic with mortality estimated based on reports…
Abstract Number: 0949 • ACR Convergence 2020
SLAMF7 Engagement Drives Monocyte Super-Activation in Acute and Chronic Inflammation
Daimon Simmons¹, Hung Nguyen², Emma Gomez-Rivas³, YunJu Jeong⁴, William Apruzzese⁵, Edy Kim⁶ and Michael Brenner⁷, ¹Department of Pathology, Brigham and Women's Hospital, Boston, MA, ²Division of Rheumatology, Brigham and Women's Hospital,, Boston, MA, ³Division of Rheumatology, Brigham and Women's Hospital, Boston, MA, ⁴Division of Pulmonary and Critical Care, Brigham and Women's Hospital, Boston, MA, ⁵., Boston, ⁶Brigham and Women’s Hospital, Division of Pulmonary and Critical Care Medicine, Boston, MA, ⁷Brigham and Women's Hospital and Harvard Medical School, Boston, MA
Background/Purpose: Monocytes orchestrate immune responses that protect against microbes but can also drive pathological inflammation and autoimmune disease. Monocytes are thought to be activated primarily…
Abstract Number: 1156 • ACR Convergence 2020
Comparison of Immunological Biomarkers and Lung Histology in Patients with Elevated IL18 – Pulmonary Alveolar Proteinosis and Recurrent Macrophage Activation Syndrome (IL-18PAP-MAS) and Other Inflammatory Lung Diseases
Alhanouf Alsaleem¹, Adriana de Jesus², Sofia Torreggiani³, Chyi-Chia Lee⁴, Les Folio⁵, Huy Do⁶, Andrew Oler⁷, Caroline Kim³, Stewart Levine⁸, Anthony Suffredini⁹, Cem Gabay¹⁰, Joseph Fontana¹¹, Scott Canna¹² and Raphaela Goldbach-Mansky¹³, ¹Division of Pediatric Rheumatology, Department of pediatrics, King Faisal specialist hospital and research center, Riyadh, Saudi Arabia, RiYADH, Saudi Arabia, ²Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Silver Spring, MD, ³Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, ⁴Pathology Department/NCI/NIH, Bethesda, MD, ⁵Radiology and Imaging Services/NIH, Bethesda, MD, ⁶Radiology and Imaging Sciences, Bethesda, MD, ⁷Bioinformatics and Computational Biosciences Branch/NIAID/NIH, Bethesda, MD, ⁸Laboratory of Asthma and Lung Inflammation, Division of Intramural Research, NHLBI, NIH,, Bethesda, MD, ⁹Critical Care Medicine Department, Clinical Center, NIH, Bethesda, MD, ¹⁰University Hospitals of Geneva, Geneva, Switzerland, ¹¹NHLBI/NIH, Bethesda, MD, ¹²University of PIttsburgh, Pittsburgh, PA, ¹³Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Potomac, MD
Background/Purpose: Recently, pulmonary alveolar proteinosis (PAP) and recurrent macrophage activation syndrome (MAS) have been reported in rare patients (pts) with systemic juvenile idiopathic arthritis (SJIA)…
Abstract Number: 1217 • ACR Convergence 2020
Effects of JAK Inhibitors Against JAK2-mediated Signaling in Innate Immune Cells
Yuya Fujita¹, Naoki Matsuoka¹, Makiko Furuya-Yashiro², Jumpei Temmoku², Yuki Kuroiwa³, Masaru Tanaka⁴, Tomoyuki Asano², Shuzo Sato⁵, Haruki Matsumoto², Hiroshi Watanabe², Hideko Kuzuru⁶, Hiroshi Yatsuhashi⁷, Atsushi Kawakami⁸ and Kiyoshi Migita⁹, ¹Fukushima Medical University School of Medicine, Department of Rheumatology, Fukushima, Fukushima, Japan, ²Fukushima Medical University School of Medicine, Department of Rheumatology, Fukushima, Japan, ³Eli Lilly Japan K.K., Tokyo, ⁴Eli Lilly Japan K.K., Tokyo, Japan, ⁵Fukushima Medical University School of Medicine, Department of Rheumatology, Fukushima, Japan, ⁶NHO Nagasaki Medical Center, Clinical Research Center, Omura, Japan, ⁷NHO Nagasaki Medical Center, Clinical Research Center, Omura, Nagasaki, Japan, ⁸Nagasaki University Graduate School of Biomedical Sciences, Unit of Advanced Preventive Medical Sciences, Department of Immunology and Rheumatology, Nagasaki, Nagasaki, Japan, ⁹Fukushima Medical University School of Medicine, Department of Rheumatology, Fukushima, Fukushima, Japan
Background/Purpose: Janus kinase (JAK) family is comprised of JAK1, JAK2, JAK3 and tyrosine kinase 2 (TYK2). JAKs form homo- or hetero-complexes, the combination of which…
Abstract Number: 159 • 2020 Pediatric Rheumatology Symposium
Interleukin-18 as a Key Cytokine to Understand Pathology and to Decide Appropriate Therapeutic Strategy in Chronic Arthritic Systemic Juvenile Idiopathic Arthritis
Takako Miyamae¹, Yumi Tani ¹, Manabu Kawamoto ², Takayuki Kishi ³ and Masayoshi Harigai ², ¹Pediatric Rheumatology, Institute of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan, ²Institute of Rheumatology, Tokyo Women's Medical University, TOKYO, Japan, ³Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan
Background/Purpose: Long-term outcomes of systemic JIA are highly variable. approximately half of the patients have chronic persistent arthritis requiring extended anti-inflammatory therapy, sometimes into adulthood.…
Abstract Number: 75 • 2019 ACR/ARP Annual Meeting
Microglia-Specific Transcriptional Signatures Correlate with Behavioral Deficits in ‘Neuropsychiatric Symptoms of Systemic Lupus Erythematosus’
Hadijat Makinde¹, Elise Mike ², Chaim Putterman ³, Deborah Winter ⁴ and Carla Cuda ⁵, ¹Northwestern University, Chicago, IL, ²Albert Einstein College of Medicine, Bronx, NY, ³Albert Einstein College of Medicine, New York, NY, ⁴Northwestern University Feinberg School of Medicine, Division of Rheumatology, Chicago, ⁵Northwestern University Feinberg School of Medicine, Division of Rheumatology, Chicago, IL
Background/Purpose: Systemic lupus erythematosus (SLE) is a complex autoimmune syndrome affecting multiple organs, including the brain. More than 50% of patients experience neuropsychiatric symptoms of…
Abstract Number: 76 • 2019 ACR/ARP Annual Meeting
Effect of Ifnα and Costimulatory Blockade on Brain Infiltration in a Model of ‘Neuropsychiatric Symptoms of Systemic Lupus Erythematosus’
Hadijat Makinde¹, Chirag Raparia ², Anne Davidson ² and Carla Cuda ³, ¹Northwestern University, Chicago, IL, ²Feinstein Institutes for Medical Research, Manhasset, ³Northwestern University Feinberg School of Medicine, Division of Rheumatology, Chicago, IL
Background/Purpose: Systemic lupus erythematosus (SLE) is a complex autoimmune disease that affects many end organs including the brain. Despite a prevalence of over 50% in…
Abstract Number: 79 • 2019 ACR/ARP Annual Meeting
Unique Primed Status of Microglia Under the Systemic Autoimmune Condition of Lupus-Prone Mice
Atsushi Nomura¹, Daisuke Noto ², Goh Murayama ³, Asako Chiba ¹ and Sachiko Miyake ¹, ¹Department of Immunology, Juntendo University School of Medicine, Tokyo, Japan, ²Juntendo University School of Medicine, Tokyo, Japan, ³Department of Internal Medicine and Rhumatology, Juntendo University School of Medicine, Tokyo, Japan
Background/Purpose: Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the production of various autoantibodies. This disease causes disabling neuropsychiatric symptoms even in the…
Abstract Number: 800 • 2019 ACR/ARP Annual Meeting
Cryopyrin-Associated Periodic Syndrome in Korea: 19 Years of Experience
Young Ho Kim¹, Bongjik Kim ², Byung Yoon Choi ², Haeng Jin Lee ³, Dae-Chul Jeong ⁴, Jinhee Han ², Hye-Rim Park ², Jayoung Oh ², Seungmin Lee ², Dooyi Oh ⁵ and Soyoung Lee ¹, ¹Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Seoul-t'ukpyolsi, Republic of Korea, ²Department of Otorhinolaryngology-Head and Neck Surgery, Seoul National University Bundang Hospital, Seongnam, Kyonggi-do, Republic of Korea, ³Department of Ophthalmology, Seoul National University Hospital, Seoul, Republic of Korea, ⁴Department of Pediatrics, The Catholic University of Korea, Seoul, Seoul-t'ukpyolsi, Republic of Korea, ⁵Department of Otorhinolaryngology-Head and Neck Surgery, Seoul National University Bundang Hospital, Seoul, Kyonggi-do, Republic of Korea
Background/Purpose: Cryopyrin-associated periodic syndrome (CAPS) is rare auto-inflammatory disorder characterized by recurrent episodes fever with variable manifestation of systemic inflammation such as urticarial skin rash,…
Abstract Number: 1020 • 2019 ACR/ARP Annual Meeting
NLRP12 Regulates Interferon-α Expression and Is a Biomarker for Disease Activity of Systemic Lupus Erythematosus
Ming-Han Chen¹, Yen-Po Tsao ¹ and Szu-Ting Chen ², ¹Division of Allergy, Immunology & Rheumatology/Taipei Veterans General Hospital, Taipei, Taiwan (Republic of China), ²Institutes of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan (Republic of China)
Background/Purpose: Systemic lupus erythematous (SLE) is a systemic autoimmune disease with diverse etiological factors. It was well recognized that interferon (IFN) signature did the perpetration…
Abstract Number: 2736 • 2019 ACR/ARP Annual Meeting
Mucosal-associated Invariant T Cells Can Be Therapeutically Targeted in Lupus
Goh Murayama¹, Asako Chiba ², Tomohiro Mizuno ³, Atsushi Nomura ², Taiga Kuga ⁴, HIrofumi Amano ⁴, Ken Yamaji ⁴, Naoto Tamura ⁴ and Sachiko Miyake ², ¹Department of Internal Medicine and Rhumatology, Juntendo University School of Medicine, Tokyo, Japan, ²Department of Immunology, Juntendo University School of Medicine, Tokyo, Japan, ³Department of Immunlogy, Juntendo University School of Medicine, Tokyo, Japan, ⁴Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, Tokyo, Japan
Background/Purpose: Mucosal-associated invariant T (MAIT) cells are innate T cells that are restricted by the nonpolymorphic MHC-related molecule-1 (MR1) and express a semi-invariant TCRα chain:…

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