Abstracts tagged "Autoinflammatory diseases"

Abstract Number: 0269 • ACR Convergence 2023
Acute Kidney Injury and Plasma Cell-Rich Acute Interstitial Nephritis in VEXAS Syndrome: An Under-recognized Disease Feature
Matthew Koster, Loren HerreraHernandez, Lihong Bu, Daniel Montes, Samih Nasr, Lynn Cornell, Kenneth Warrington, Mrinal S. Patnaik, Abhishek A. Mangaonkar and Kambiz Kalantari, Mayo Clinic, Rochester, MN
Background/Purpose: VEXAS syndrome (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) is a recently identified autoinflammatory disease with a large variety of disease manifestations. While recurrent fever,…
Abstract Number: 1132 • ACR Convergence 2023
Preclinical Profiles of FZ007-119, a Highly Potent and Selective Tyk2 Inhibitor, for the Treatment of Immune Mediated Inflammatory Diseases
Wenhe Zhong, Jielian Lu, Dong Chen, Shiqun Zhang and Daiguo Deng, Guangzhou Fermion Technology Co., LTD, Guangzhou, China
Background/Purpose: Tyrosine Kinase 2 (TYK2) is a signaling protein within the Janus kinase (JAK) family. It plays a crucial role in transmitting signals from pro-inflammatory…
Abstract Number: 1918 • ACR Convergence 2023
Nationwide Analysis of Predictors of Sarcoid Inpatient Mortality
Michael Manansala¹, Faria Sami², Shilpa Arora³ and Augustine Manadan¹, ¹Rush University Medical Center, Chicago, IL, ²John H. Stroger Jr. Hospital of Cook County, Chicago, IL, ³Cook County Hospital, Chicago, IL
Background/Purpose: Sarcoidosis is multisystem autoimmune disease characterized by noncaseating granulomas which can result in significant morbidity and mortality. This study aims to identify variables associated…
Abstract Number: 2505 • ACR Convergence 2023
Discovery of First-in-Class IRAK4 Scaffolding Inhibitors for the Treatment of Inflammatory Disorders
Kate Byth¹, Rodney Morgan², Pierre Michelys³, Kellie Demock², Geetha Kannan², Qing Chen¹, Brian Sanchez², Xiaokang Lu², Alissa Telling², Chuck Lesch², Carmen Yu², Sarah Bradley², Brian Andresen¹, Sherif Hassanien², Clarke Taylor², Zhilin Hou², Ken Borrelli¹, Anna Gardberg¹, Erika Munschy¹, Adam Schwaid¹, Anthony Opipari², Shifeng Pan³ and Luigi Franchi², ¹Odyssey Therapeutics, Boston, MA, ²Odyssey Therapeutics, Ann Arbor, MI, ³Odyssey Therapeutics, San Diego, CA
Background/Purpose: Interleukin-1 receptor-associated kinase 4 (IRAK4), is a key node that mediates inflammatory signaling in response to activation of toll-like receptor (TLR) and IL-1 receptor…
Abstract Number: 0006 • ACR Convergence 2023
Preclinical Characterization of a Novel anti-CD40 Antagonist Antibody-glucocorticoid Conjugate with Superior Preclinical Efficacy and Favorable Safety Profile
Dianru wang¹, Wenming Ren², lu su¹, yuan lin¹ and cheng liao¹, ¹Jiangsu Hengrui Pharmaceuticals Co., Ltd., Shanghai, China, ²Jiangsu Hengrui Pharmaceuticals Co., Ltd., Pudong New District, China
Background/Purpose: Glucocorticoids (GCs) are the first-line anti-inflammatory treatment for many autoimmune diseases, but long-term systemic administration of GCs is associated with unwanted side effects, which…
Abstract Number: 0275 • ACR Convergence 2023
No Cumulative Effect of Infection Rates in Children Receiving Long-term Canakinumab Treatment in Autoinflammatory Periodic Fever Syndromes − Data from the RELIANCE Registry
Jasmin B. Kuemmerle-Deschner¹, Joerg Henes², Birgit Kortus-Goetze³, Prasad T. Oommen⁴, Anne Pankow⁵, Tilmann Kallinich⁶, Tobias Krickau⁷, Catharina Schuetz⁸, Gerd Horneff⁹, Ivan Foeldvari¹⁰, Juergen Rech¹¹, Frank Weller-Heinemann¹², Ales Janda¹³, Markus Hufnagel¹⁴, Florian M. Meier¹⁵, Frank Dressler¹⁶, Michael Borte¹⁷, Ioana Andreica¹⁸, Peter Wasiliew¹⁹, Michael Fiene²⁰, Daniel Windschall²¹, Martin Krusche²², Tania Kuempfel²³, Julia Weber-Arden²⁴ and Norbert Blank²⁵, ¹med.uni-tuebingen, Tübingen, Germany, ²University Hospital Tuebingen, Tuebingen, Germany, ³Department of Internal Medicine, Division of Nephrology,University Hospital of Giessen and Marburg, Marburg, Germany, ⁴Department of Pediatric Oncology, Hematology and Clinical Immunology, Center for Child and Adolescent Health,Medical Faculty Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, ⁵Department of Rheumatology and Clinical Immunology,Charité-Universitätsmedizin Berlin, Berlin, Germany, ⁶Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité Universitätsmedizin Berlin, Nuremberg, Germany, ⁷Pediatrics, Friedrich-Alexander University Erlangen-Nuernberg (FAU), Erlangen, Germany, ⁸Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus,Technische Universität Dresden, Dresden, Germany, ⁹Asklepios Klinik Sankt Augustin GmbH, Bonn, Germany, ¹⁰Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ¹¹University Clinic Erlangen, Erlangen, Germany, ¹²Division of Pediatric Rheumatology, Prof. Hess Children's Hospital, Bremen, Bremen, Germany, ¹³Department of Pediatrics and Adolescent Medicine, University Medical Center Ulm, Ulm, Germany, ¹⁴Division of Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics and Adolescent Medicine, University Medical Center, Medical Faculty, University of Freiburg, Freiburg, Germany, ¹⁵Department of General Pharmacology and Toxicology, Goethe University Hospital and Goethe University Frankfurt, Frankfurt am Main, Germany, ¹⁶Department of Paediatric Pneumology, Allergology and Neonatology, Children's Hospital, Hannover Medical School, Hannover, Germany, ¹⁷Hospital for Children & Adolescents, St. Georg Hospital, Leipzig, Germany, ¹⁸Rheumazentrum Ruhrgebiet Herne, Herne, Germany, ¹⁹Division of Pediatric Rheumatology and autoinflammation reference center Tuebingen, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany, ²⁰Rheumatology Center Greifswald, Greifswald, Germany, ²¹Clinic of Paediatric and Adolescent Rheumatology, St. Josef-Stift Sendenhorst, Northwest German Center for Rheumatology, Sendenhorst, Germany, ²²UKE, Hamburg, Germany, ²³Institute of Clinical Neuroimmunology, Biomedical Center and University Hospital, Ludwig-Maximilians Universität München, Muenchen, Germany, ²⁴Novartis Innovative Medicines, Nuernberg, Germany, ²⁵University Hospital Heidelberg, Heidelberg, Germany
Background/Purpose: Autoinflammatory diseases (AID) have been treated safely and effectively with the interleukin-1β inhibitor canakinumab (CAN) in controlled trials and routine clinical practice. The most…
Abstract Number: 1136 • ACR Convergence 2023
Challenges in Diagnosing VEXAS Syndrome: Delayed Diagnosis, Misdiagnosis, and Associations with Specific Gene Mutations
Paula García Escudero¹, Marta López i Gómez², Marta López Maraver³, PALOMA VELA⁴, María Rodríguez Laguna⁵, carolina Merino⁶, Ana Victoria Orenes Vera⁷, Clara García Belando⁸, Diego Dios Santos⁹, Jose Alberto Miranda Filloy¹⁰, Francisco Javier Toyos Sáenz de Miera¹¹, Alicia Garcia Dorta¹², Ignacio Vázquez¹³, José Ángel Hernández Beriain¹⁴, MARIA ELVIRA DIEZ ALVAREZ¹⁵, Marta Ibáñez Martínez¹⁶, Beatriz Frade Sosa¹⁷, Judit Font Urgelles¹⁸, Meritxell Salles Lizarzaburu¹⁹, Elena Riera Alonso²⁰, Ernesto Trallero Araguás²¹, Berta Magallanes López²², Joaquín Belzunegui Otano²³, Carlos de Miguel Sánchez²⁴, Zuriñe Ortiz de Zárate Caballero²⁵ and Jaime Calvo Alén²⁶, ¹Hospital Universitario Araba, Bilbao, Spain, ²Hospital Universitario de Araba, Pamplona, Spain, ³Hospital Universitario de Araba, Vitoria, Spain, ⁴Rheumatology, Hospital General Universitario Alicante, Alicante, Spain, ⁵H. Clínico San Carlos, Madrid, Spain, ⁶Rheumatology Department Hospital Universitario Puerta de Hierro, Majadahonda (Madrid), Spain, ⁷H. Arnau de Vilanova, Valencia, Spain, ⁸Hospital Los Arcos del Mar Menor, Murcia, Spain, ⁹Rheumatology department, Complexo Hospitalario Universitario A Coruña (CHUAC). Instituto de Investigación Biomédica A Coruña (INIBIC), A Coruña, Spain, ¹⁰C. H. U. Lucus Augusti, Lugo, Spain, ¹¹Hospital Virgen Macarena, Sevilla, Spain, ¹²Rheumatology Unit, Hospital Universitario de Canarias, Santa Cruz de Tenerife, Spain, ¹³H. U. Dr. Peset, Valencia, Spain, ¹⁴Rheumatology, Hospital Insular de Gran Canaria, Las Palmas de Gran Canaria, Spain, ¹⁵Rheumatology, Hospital Universitario de León, León, Spain, ¹⁶C. A. U. de Salamanca, Salamanca, Spain, ¹⁷Hospital Clínic de Barcelona, Barcelona, Spain, ¹⁸Hospital Universitario Germans Trías i Pujol, Barcelona, Spain, ¹⁹C. H. de Manresa - Fundació Althaia, Barcelona, Spain, ²⁰Hospital Universitario Mútua Terrassa, Barcelona, Spain, ²¹Department of Rheumatology, Hospital Universitario Vall d'Hebron, Barcelona, Spain, ²²Hospital de la Santa Creu i Sant Pau, Barcelona, Spain, ²³Hospital Universitario Donosti, Donosti, Spain, ²⁴Hospital Universitario de Araba, Hematology, VItoria, Spain, ²⁵Hospital Universitario de Araba, Internal Medicine, Vitoria, Spain, ²⁶Hospital Universitario Araba, Vitoria, Spain
Background/Purpose: The newly described VEXAS syndrome is a very heterogenous disease with rheumatologic and hematologic manifestations, caused by somatic mutations affecting UBA1 gene, that still…
Abstract Number: 1921 • ACR Convergence 2023
Clinical Spectrum of VEXAS Syndrome in a Rheumatology Department
Herman Mann¹, Blanka Stiburkova², Katerina Pavelcova¹, Monika Belickova³, Cyril Salek³, Jana Juhaszova¹ and Jiri Vencovsky⁴, ¹Institute of Rheumatology, Praha 2, Czech Republic, ²Institute of Rheumatology, Prague, Czech Republic, ³Institute of Hematology and Blood Transfusion, Praha 2, Czech Republic, ⁴Institute of Rheumatology, Prague, Czech Republic, Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic
Background/Purpose: VEXAS (vacuoles, E1 activating enzyme, X-linked, auto-inflammatory, somatic) syndrome is a clinically serious and potentially fatal adult-onset disease caused by somatic mutations in the…
Abstract Number: 2519 • ACR Convergence 2023
A Phase II Clinical Study to Investigate the Efficacy and Safety of Hemay005 Tablets in Patients with Active Behçet`sDisease
Charles Jones¹, Zhanguo Li², Zhuoli Zhang³, Jian Wu⁴, Guixiu Shi⁵, Wenjie Zheng⁶, Jianping Tang⁷, Xiaobing Wang⁸, Lie Dai⁹, Lin Chen¹⁰, Yasong Li¹¹, Ling Wu¹², Yongfu Wang¹³, Shengyun Liu¹⁴, Yao Ke¹⁵, Jin Lin¹⁶, Zhenchun Zhang¹⁷, Jiankang Hu¹⁸, Wantai Dang¹⁹, Songlou Yin²⁰, Xin Tang²¹, Mingfei Zhu²², Jinfeng Lin²², Richard Jones²², Weiguo Wan²³ and xianjun Hu²², ¹Hemay Pharmaceuticals, Tianjin, China, ²Peking University Health Science Center, Department of Rheumatology and Immunology, People's Hospital, Beijing, China, ³Peking University First Hospital, Rheumatology and Immunology Department, Beijing, China, ⁴Suzhou University Affiliated First Hospital, Suzhou, China, ⁵the First Affiliated Hospital of Xiamen University, Xiamen, China, ⁶Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Beijing, China, ⁷Shanghai Tongji Hospital, Shanghai, China, ⁸The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, China, ⁹Department of Rheumatology, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, China, ¹⁰Jilin Provincial People's Hospital, Changchun, China, ¹¹11Zhejiang Provincial People's Hospital, Hangzhou, China, ¹²University of Hong Kong Shenzhen Hospital, Shenzhen, China, ¹³The First Affiliated Hospital of Baotou Medical College, Baotou, China, ¹⁴The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China, ¹⁵Jiangsu Provincial People's Hospital, Nanjing, China, ¹⁶The First Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, China, ¹⁷Linyi People's Hospital, Linyi, China, ¹⁸Pingxiang People’s Hospital, PingXiang, China, ¹⁹The First Affiliated Hospital of Chengdu Medical College, Chengdu, China, ²⁰Xuzhou Medical University Affiliated Hospital, Xuzhou, China, ²¹Bestudy (Shanghai) Medical Technology Co., Ltd, Shanghai, China, ²²Clinical Department, Tianjin Hemay Pharmaceutical Co., Ltd., Tianjin, China, ²³Huashan Hospital Affiliated to Fudan University, Shanghai, China
Background/Purpose: Behçet's disease (BD) is a chronic and recurrent vascular inflammatory disease with major manifestations including oral ulcers, genital ulcers, skin damage and ophthalmitis, and…
Abstract Number: 0028 • ACR Convergence 2023
Adenosine Deaminase 2 Is Expressed as a Short Isoform Lacking Deaminase Activity in the Endothelium: Implications for DADA2 Vasculitis
Andrew Porter¹, Robert Maughan¹, Charis Pericleous¹, Lida Kabir¹, Richard Stratton², Dorian Haskard¹, Pui Lee³, Taryn Youngstein¹ and Justin Mason¹, ¹National Heart & Lung Institute, Imperial College London, London, United Kingdom, ²University College London, London, United Kingdom, ³Division of Immunology, Boston Children's Hospital, Boston, MA
Background/Purpose: Deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessive condition caused by biallelic variants in the Adenosine deaminase 2 (ADA2) gene. Clinical manifestations…
Abstract Number: 0276 • ACR Convergence 2023
Disease Control in Patients with Monogenetic Autoinflammatory Diseases Under Canakinumab Treatment – Comparison of 30 Months Interim Data from the RELIANCE Registry
Ivan Foeldvari¹, Tilmann Kallinich², Norbert Blank³, Joerg Henes⁴, Birgit Kortus-Goetze⁵, Prasad T. Oommen⁶, Anne Pankow⁷, Tobias Krickau⁸, Catharina Schuetz⁹, Gerd Horneff¹⁰, Juergen Rech¹¹, Frank Weller-Heinemann¹², Ales Janda¹³, Markus Hufnagel¹⁴, Florian M. Meier¹⁵, Frank Dressler¹⁶, Michael Borte¹⁷, Ioana Andreica¹⁸, Peter Wasiliew¹⁹, Michael Fiene²⁰, Daniel Windschall²¹, Martin Krusche²², Tania Kuempfel²³, Julia Weber-Arden²⁴ and Jasmin B. Kuemmerle-Deschner²⁵, ¹Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ²Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité Universitätsmedizin Berlin, Nuremberg, Germany, ³University Hospital Heidelberg, Heidelberg, Germany, ⁴University Hospital Tuebingen, Tuebingen, Germany, ⁵Department of Internal Medicine, Division of Nephrology,University Hospital of Giessen and Marburg, Marburg, Germany, ⁶Department of Pediatric Oncology, Hematology and Clinical Immunology, Center for Child and Adolescent Health,Medical Faculty Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, ⁷Department of Rheumatology and Clinical Immunology,Charité-Universitätsmedizin Berlin, Berlin, Germany, ⁸Pediatrics, Friedrich-Alexander University Erlangen-Nuernberg (FAU), Erlangen, Germany, ⁹Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus,Technische Universität Dresden, Dresden, Germany, ¹⁰Asklepios Klinik Sankt Augustin GmbH, Bonn, Germany, ¹¹University Clinic Erlangen, Erlangen, Germany, ¹²Division of Pediatric Rheumatology, Prof. Hess Children's Hospital, Bremen, Bremen, Germany, ¹³Department of Pediatrics and Adolescent Medicine, University Medical Center Ulm, Ulm, Germany, ¹⁴Division of Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics and Adolescent Medicine, University Medical Center, Medical Faculty, University of Freiburg, Freiburg, Germany, ¹⁵Department of General Pharmacology and Toxicology, Goethe University Hospital and Goethe University Frankfurt, Frankfurt am Main, Germany, ¹⁶Department of Paediatric Pneumology, Allergology and Neonatology, Children's Hospital, Hannover Medical School, Hannover, Germany, ¹⁷Hospital for Children & Adolescents, St. Georg Hospital, Leipzig, Germany, ¹⁸Rheumazentrum Ruhrgebiet Herne, Herne, Germany, ¹⁹Division of Pediatric Rheumatology and autoinflammation reference center Tuebingen, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany, ²⁰Rheumatology Center Greifswald, Greifswald, Germany, ²¹Clinic of Paediatric and Adolescent Rheumatology, St. Josef-Stift Sendenhorst, Northwest German Center for Rheumatology, Sendenhorst, Germany, ²²UKE, Hamburg, Germany, ²³Institute of Clinical Neuroimmunology, Biomedical Center and University Hospital, Ludwig-Maximilians Universität München, Muenchen, Germany, ²⁴Novartis Innovative Medicines, Nuernberg, Germany, ²⁵med.uni-tuebingen, Tübingen, Germany
Background/Purpose: Treatment of autoinflammatory periodic diseases (AID) with the interleukin-1β inhibitor canakinumab (CAN) has been shown to be safe and effective in controlled trials and…
Abstract Number: 1139 • ACR Convergence 2023
From Skin to Bone Lesions: A Pioneer Study Unraveling the Underdiagnosis of SAPHO Syndrome in the Dominican Republic
Leeany Maletta¹, Diana Jimenez² and Rosanna de Jesus³, ¹UNIBE School of Medicine, San Antonio, TX, ²UNIBE School of Medicine, Miami, FL, ³Hospital General Plaza de la Salud, Distrito Nacional, Dominican Republic
Background/Purpose: SAPHO syndrome is a rare chronic inflammatory condition that affects the skin and osteoarticular structures. Despite being first described over 30 years ago, it…
Abstract Number: 1925 • ACR Convergence 2023
Importance of the Patient Pain Experience in Disease Activity Assessment in the Adult SAPHO and Chronic Nonbacterial Osteomyelitis Study
Aleksander Lenert¹, Robyn Domsic², Karmela Kim Chan³, Melissa Oliver⁴, Jisna Paul⁵, Courtney Kremer⁶, Emma Leisinger¹, Helena Abodeely¹, Sandy Hong⁶, Arundathi Jayatilleke⁷, Petar Lenert⁸, T. Shawn Sato¹, Yongdong (Dan) Zhao⁹, Jonathan Templin¹, Mary Vaughan-Sarrazin¹, Daniel Solomon¹⁰ and Polly Ferguson¹¹, ¹University of Iowa, Iowa City, IA, ²University of Pittsburgh, Pittsburgh, PA, ³Hospital For Special Surgery, New York, NY, ⁴Indiana University, Indianapolis, IN, ⁵Ohio State University Hospital, Columbus, OH, ⁶University of Iowa Stead Family Children's Hospital, Iowa City, IA, ⁷Temple University, Philadelphia, PA, ⁸University of Iowa Hospitals and Clinics, Iowa City, IA, ⁹University of Washington, Seattle, WA, ¹⁰Brigham and Women's Hospital, Boston, MA, ¹¹University of Iowa Carver College of Medicine, Iowa City, IA
Background/Purpose: Musculoskeletal pain is a key symptom experienced by patients with SAPHO and chronic nonbacterial osteomyelitis (SAPHO-CNO), yet its relation to disease activity assessment remains…
Abstract Number: 2570 • ACR Convergence 2023
Thrombosis in Patients with VEXAS Syndrome: A Retrospective Cohort Study
Atefeh Ghorbanzadeh¹, Yael Kusne², Alina Dulau-Florea³, Ruba Shalhoub³, Pedro Alcedo³, Khanh Nghiem³, Marcela Ferrada⁴, alexander hines¹, Sumith Panicker³, amanda ombrello⁵, Kaaren K. Reichard¹, Ivana Darden³, Wendy Goodspeed³, Lorena wilson³, Horatiu Olteanu¹, Terra L. Lasho¹, Daniel Kastner⁶, Kenneth Warrington¹, Abhishek A. Mangaonkar¹, Ronald Go¹, David Beck⁷, Mrinal S. Patnaik¹, Neal S. Young⁸, Katherine R. Calvo³, Ana Casanegra⁹, Peter Grayson¹⁰, Matthew Koster¹, Colin O Wu³, Damon E. Houghton⁹, Bhavisha Patel¹¹, Yogen Kanthi¹² and Emma M. Groarke¹³, ¹Mayo Clinic, Rochester, MN, ²Mayo Clinic, Phoenix, AZ, ³National Institutes of Health (NIH), Bethesda, MD, ⁴National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ⁵National Institutes of Health, Rockville, MD, ⁶National Human Genome Research Institute, Bethesda, MD, ⁷New York University, New York, NY, ⁸National Institutes of Health (NIH), Rochester, MD, ⁹Mayo Clinic, Rochester, MD, ¹⁰National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Chevy Chase, MD, ¹¹National Institutes of Health, Beltsville, MD, ¹²National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD, ¹³Hematology Branch, National Heart, Lung, and Blood Institute, National Institutes of Health (NIH), Bethesda, MD
Background/Purpose: VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome, due to somatic mutations in the UBA1 gene, is an autoinflammatory disorder associated with an increased…
Abstract Number: 0039 • ACR Convergence 2023
Enrichment of Rare Variants of Hemophagocytic Lymphohistiocytosis Genes in Systemic Juvenile Idiopathic Arthritis
Mariana Correia Marques¹, Danielle Rubin¹, Emily Shuldiner², Mallika Datta¹, Elizabeth Schmitz¹, Alexei Grom³, Dirk Foell⁴, Marco Gattorno⁵, John Bohnsack⁶, Rae Yeung⁷, Sampath Prahalad⁸, Elizabeth Mellins⁹, Jordi Anton Lopez¹⁰, Claudio Len¹¹, Sheila Oliveira¹², Patricia Woo¹³, Seza Ozen¹⁴, INCHARGE Consortium¹, Zuoming Deng¹⁵ and Michael Ombrello¹, ¹National Institute of Arthritis & Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ²Department of Biology, Stanford University, Stanford, CA, ³Division of Rheumatology, Cincinnati Children`s Hospital, Cincinnati, OH, ⁴University Hospital Münster, Münster, Germany, ⁵UOC Reumatologia e Malattie Autoinfiammatorie, Genoa, Italy, ⁶University of Utah, Salt Lake City, UT, ⁷The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada, ⁸Emory University School of Medicine, Children's Pediatric Institute, Atlanta, GA, ⁹Stanford University, Stanford, CA, ¹⁰Pediatric Rheumatology Department, Hospital Sant Joan e Deu, Barcelona University, Barcelona, Spain, ¹¹São Paulo Federal University, São Paulo, Brazil, ¹²Universidade Federal do Rio de Janeiro, Rio De Janeiro, Brazil, ¹³University College London, London, United Kingdom, ¹⁴Hacettepe University Medical Faculty, Ankara, Turkey, ¹⁵National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a complex inflammatory condition of childhood. It can be complicated by macrophage activation syndrome (MAS), a secondary form…

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