Abstracts tagged "macrophage activation syndrome"

Abstract Number: 0864 • ACR Convergence 2022
Baseline Clinical and Laboratory Features of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Systemic Juvenile Idiopathic Arthritis-Associated Lung Disease (SJIA-LD) Cohort
Esraa Eloseily¹, Min-Lee Chang², MaryEllen Riordan³, Alan Russell⁴, Marc Natter², Yukiko Kimura⁵ and Grant Schulert⁶, ¹Division of Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Boston Children's Hospital, Boston, MA, ³Hackensack Meridian Health, Hackensack, NJ, ⁴Duke Clinical Research Institute, Duke, NC, ⁵Hackensack Meridian Health, New York, NY, ⁶Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) associated lung disease (SJIA-LD) is an emerging and life threatening clinical problem, and currently affects as many as 1…
Abstract Number: 0872 • ACR Convergence 2022
Incidence, Risk Factors, and Outcomes of Eosinophilia on IL-1 and IL-6 Inhibitors in Systemic and Non-Systemic Juvenile Idiopathic Arthritis
Holly Wobma¹, Maria Taylor², Ki Pui Lam¹, Helene Brown¹, Siobhan Case¹, Mia Chandler¹, Margaret Chang¹, Ezra Cohen¹, Megan Day-Lewis¹, Olha Halyabar¹, Jonathan Hausmann¹, Melissa Hazen³, Erin Janssen¹, Pui Lee¹, Mindy Lo¹, Esra Meidan¹, Jordan Roberts¹, Mary Beth F. Son¹, Robert Sundel¹, Fatma Dedeoglu¹, Peter Nigrovic¹, Alicia Casey⁴, Joyce Chang¹ and lauren henderson¹, ¹Division of Immunology, Boston Children's Hospital, Boston, MA, ²Division of Immunology, Boston Children's Hospital, Brighton, MA, ³Boston Children's Hospital, Boston, MA, ⁴Division of Pulmonary Medicine, Boston Children's Hospital, Boston, MA
Background/Purpose: Children with systemic juvenile idiopathic arthritis (sJIA) exposed to IL1/6 inhibitors may develop eosinophilia with an atypical rash, reportedly in association with HLA-DRB1*15:XX. There…
Abstract Number: 2213 • ACR Convergence 2022
Multi-omic Analysis of Macrophage Activation Syndrome Associated with sJIA Reveals a Potential Role of Type I Interferons in the Expansion of Cycling T Cells
Kailey Brodeur¹, Liang Chen¹, zhengping huang², Yan Du¹, Holly Wobma³, Maria Taylor⁴, Joyce Chang³, Megan Day-Lewis³, Fatma Dedeoglu³, Olha Halyabar³, Mindy Lo³, Jane W. Newburger⁵, Mary Beth F. Son³, Robert Sundel³, Peter Nigrovic³, lauren henderson³ and Pui Lee³, ¹Boston Children's Hospital, Boston, MA, ²Guangdong Second Provincial Hospital, Guangzhou, China, ³Division of Immunology, Boston Children's Hospital, Boston, MA, ⁴Division of Immunology, Boston Children's Hospital, Brighton, MA, ⁵Department of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, MA
Background/Purpose: Macrophage activation syndrome (MAS) is a complication of systemic juvenile idiopathic arthritis (sJIA) characterized by cytokine storm and overt immune cell activation. We aim…
Abstract Number: 0004 • ACR Convergence 2022
mTORC1 Drives the Spectrum of Pathology in Systemic JIA and Macrophage Activation Syndrome
zhengping huang¹, xiaomeng You², Liang Chen³, Yan Du³, Kailey Brodeur³, Qiang Wang³, David Sykes⁴, Margaret Chang⁵, Julia Charles², Peter Nigrovic⁵ and Pui Lee⁵, ¹Guangdong Second Provincial Hospital, Guangzhou, China, ²Brigham and Women's Hospital, Boston, MA, ³Boston Children's Hospital, Boston, MA, ⁴Massachusetts General Hospital, Boston, MA, ⁵Division of Immunology, Boston Children's Hospital, Boston, MA
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a severe inflammatory syndrome characterized by fever, skin rash and arthritis. A subset of patients with sJIA develop…
Abstract Number: 0562 • ACR Convergence 2022
Large-Scale Targeted Sequencing Study Links Systemic Juvenile Idiopathic Arthritis with Rare Variants of MEFV, LYST, STXBP2, UNC13D
Mariana Correia Marques¹, Danielle Rubin², Emily Shuldiner², Elizabeth Schmitz², Elizabeth Baskin², Andrew Patt³, Alexei Grom⁴, Dirk Foell⁵, Marco Gattorno⁶, John Bohnsack⁷, Rae Yeung⁸, Sampath Prahalad⁹, Elizabeth Mellins¹⁰, Jordi Antón¹¹, Claudio Len¹², Sheila Oliveira¹³, Patricia Woo¹⁴, Seza Ozen¹⁵, INCHARGE Consortium¹⁶ and Michael Ombrello¹⁷, ¹National Institute of Arthritis and Musculoskeletal and Skin Diseases / Children`s National Hospital, Bethesda, MD, ²National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, ³National Center for Advancing Translational Sciences, Bethesda, MD, ⁴Divisions of Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ⁵University Hospital Münster, Münster, Germany, ⁶Pediatric Clinic and Rheumatology Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy, ⁷University of Utah, Salt Lake City, UT, ⁸The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada, ⁹Emory + Children's Pediatric Institute, Atlanta, GA, ¹⁰Stanford University, Stanford, CA, ¹¹Pediatric Rheumatology Department. Hospital Sant Joan de Déu. Universitat de Barcelona, Esplugues de Llobregat, Spain, ¹²Universidade Federal de São Paulo, São Paulo, Brazil, ¹³Universidade Federal do Rio de Janeiro, Rio De Janeiro, Brazil, ¹⁴University College London, London, United Kingdom, ¹⁵Hacettepe University Faculty of Medicine, Ankara, Turkey, ¹⁶International Childhood Arthritis Genetics Consortium, Bethesda, MD, ¹⁷National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a genetically complex inflammatory condition. It can be marked by severe systemic inflammation that resembles the hereditary periodic…
Abstract Number: 0569 • ACR Convergence 2022
Cytokine Storm Susceptibility Factors IL-18 and Cytotoxic Impairment Converge on Pathologic CD8 T-cell Hyperactivation
Emily Landy¹, Scott Canna², Paul Tsoukas³, Vinh Dang² and Jemy Varghese², ¹University of Pittsburgh, Pittsburgh, PA, ²Children's Hospital of Philadelphia, Philadelphia, PA, ³The Hospital for Sick Children, Toronto, ON, Canada
Background/Purpose: Mounting evidence suggests partial defects in cytotoxicity-related genes may promote hyperinflammation in Macrophage Activation Syndrome (MAS) patients, who uniformly have highly-elevated IL-18. In mice,…
Abstract Number: 0799 • ACR Convergence 2022
Serum Laboratory Parameters of Hemophagocytic Lymphohistiocytosis Syndrome in Patients with Autoimmune Rheumatic Diseases Infected with SARS-CoV-2: A Retrospective Comparative Study
Omar Alsaed, Samar Al emadi, Karima Becetti, Eman Satti, Hadil Ashour, Yousef Alrimawi, Miral H.Gharib, Mohamad Alkahlout, Rawan Saleh, Basem Awadh and Mohammed Hammoudeh, Hamad Medical Corporation, Doha, Qatar
Background/Purpose: The serum features of the cytokine storm induced by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection are similar to those of hemophagocytic lymphohistiocytosis…
Abstract Number: 0825 • ACR Convergence 2022
Predicting Outcomes in Macrophage Activation Syndrome (MAS): A Retrospective Study on 43 Consecutive Patients with MAS
Sumanth Madan¹, Suma Balan¹, MITHUN CHALAKKARAYIL BHAGAVALDAS², Spoorthy DR¹, Sudeep Rath¹, Joslyn Thattil¹, Amisha Shahul¹ and Niveditha Kartha¹, ¹Amrita Institute of Medical Sciences, Kochi, India, ²Amrita Institute of Medical Sciences, Muvattupuzha, India
Background/Purpose: Macrophage activation syndrome is a diagnosis along the spectrum of hemophagocytic lymphohistiocytosis (HLH) spectrum disorders. It is a rare, yet potentially fatal disease characterized…
Abstract Number: L20 • ACR Convergence 2021
Efficacy of Emapalumab, an Anti-IFNγ Antibody in Patients with Macrophage Activation Syndrome (MAS) Complicating Systemic Juvenile Idiopathic Arthritis (sJIA) Who Had Failed High-Dose Glucocorticoids (GCs)
Fabrizio De Benedetti¹, Alexei Grom², Paul Brogan³, Claudia Bracaglia¹, Manuela Pardeo¹, Giulia Marucci¹, Despina Eleftheriou³, Charalampia Papadopoulou³, Pierre Quartier⁴, Jordi Antón⁵, Rikke Frederiksen⁶, Veronica Asnaghi⁶ and Cristina De Min⁶, ¹Bambino Gesù Children's Hospital IRCCS, Rome, Italy, ²Cincinnati Children’s Hospital, Division of Rheumatology, Cincinnati, OH, ³UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ⁴Université de Paris, IMAGINE Institute, RAISE reference centre, Pediatric Immuno-Hematology and Rheumatology Unit, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France, ⁵Hospital Sant Joan de Déu, Division of Pediatric Rheumatology, University of Barcelona, Barcelona, Spain, ⁶Swedish Orphan Biovitrum AG (Sobi), Basel, Switzerland
Background/Purpose: MAS is a life-threatening complication of rheumatic diseases, occurring most frequently in sJIA. The mainstay of MAS treatment is high dose GCs; however, GCs…
Abstract Number: 0196 • ACR Convergence 2021
A Single Center, Double Blind, Randomized, Placebo-Controlled Trial of Anakinra in Adult Patients with Features of Cytokine Storm Syndrome in COVID-19
Lesley Jackson¹, Randy Cron¹, Nitasha Khullar², Christopher Chapleau³, Dongmei Sun⁴ and Winn Chatham¹, ¹University of Alabama at Birmingham, Birmingham, AL, ²University of Alabama Birmingham, Birmingham, AL, ³UAB Pharmacy, University of Alabama at Birmingham, Birmingham, AL, ⁴Division of Clinical Immunology and Rheumatology, The University of Alabama at Birmingham, Birmingham, AL
Background/Purpose: Some patients with COVID-19 develop respiratory distress and cytokine storm syndrome (CSS) which is characterized by hyperinflammation and may progress to multi-organ failure. Anakinra…
Abstract Number: 0246 • ACR Convergence 2021
Macrophage Activating Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis Treated with Biological Drugs
Betul Sozeri¹, Kenan Barut², Erdal Atalay³, Aysenur Pac Kisaarslan⁴, Semanur Ozdel⁵, Ozge Altug⁶, Ferhat Demir¹, Balahan Makay⁷, Nuray Aktay Ayaz⁸, Ersen Acar⁹, Fatih Haslak², Erdal Sag¹⁰, Mehmet YILDIZ², Ummusen Kaya Akca¹⁰, Amra Adrovic², Yelda Bilginer¹⁰, Hakan Poyrazoglu¹¹, Erbil Unsal⁷, Ozgur Kasapcopur¹² and Seza Ozen³, ¹University of Health Sciences, Umraniye Tranining and Research Hospital Division of Pediatric Rheumatology, Istanbul, Turkey, ²Istanbul University-Cerrahpasa, Faculty of Medicine, Pediatric Rheumatology, İstanbul, Turkey, ³Hacettepe University Faculty of Medicine, Pediatric Romatology, Ankara, Turkey, ⁴Erciyes University Medical Faculty, Pediatric Rheumatology, Kayeri, Turkey, ⁵Ankara Dr.Sami Ulus Gynecology and Pediatrics Research and Training Hospital, Pediatric Rheumatology, Ankara, Turkey, ⁶Dr Behcet Uz Children's Hospital, Pediatric Rheumatology, Izmir, Turkey, ⁷Dokuz Eylul University Faculty of Medicine, Pediatric Rheumatology, Izmir, Turkey, ⁸Istanbul University, Faculty of Medicine, Pediatric Rheumatology, İstanbul, Turkey, ⁹Novartis, Istanbul, Turkey, ¹⁰Hacettepe University, Division of Pediatric Rheumatology, Department of Pediatrics, Faculty of Medicine, Ankara, Turkey, ¹¹Erciyes University Faculty of Medicine, Pediatric Rheumatology, Kayseri, Turkey, ¹²Istanbul University-Cerrahpasa, Cerrahpasa Medical School, İstanbul, Turkey
Background/Purpose: Systemic Juvenile Idiopathic Arthritis (SJIA) is the most acute and severe form of JIA. Despite the substantial evidence supporting the effectiveness of biologic drugs…
Abstract Number: 0252 • ACR Convergence 2021
Role of Clinical and Laboratory Parameters in Differentiating Infection from Disease Flare in Febrile Patients of Systemic Juvenile Idiopathic Arthritis
Rajesh Kanumuri¹, Suma Balan², Vishal Marwaha², Sajitha Krishnan² and Pranav Chickermane², ¹Amrita Institute of Medical Sciences, Guntur, India, ²Amrita Institute of Medical Sciences, Kochi, India
Background/Purpose: Fever is the most common presentation of systemic juvenile idiopathic arthritis (SJIA) and it is difficult to predict whether the fever is due to…
Abstract Number: 0254 • ACR Convergence 2021
Patient and Disease-Level Factors Associated with Sustained Cessation of Medication for Disease Remission in Systemic Juvenile Idiopathic Arthritis
Elaine Flanagan¹, Rosemary Peterson², Susan Shenoi³, Helena Chang⁴, Kelly Wang⁴, Rebecca Trachtman⁴ and Karen Onel⁵, ¹Emory/CHOA, Atlanta, GA, ²Dell Children's Medical Center, Austin, TX, ³Seattle Children's Hospital, Seattle, WA, ⁴Icahn School of Medicine at Mount Sinai, New York, NY, ⁵Hospital for Special Surgery, New York, NY
Background/Purpose: The emergence of IL-1 and IL-6 inhibitors (biologics) for the treatment of systemic juvenile idiopathic arthritis (SJIA) has dramatically improved patient outcomes. With higher…
Abstract Number: 1541 • ACR Convergence 2021
Preliminary Criteria for Macrophage Activation Syndrome Associated with Coronavirus Disease-19
Shirkhan Amikishiyev¹, Mehmet Guven Gunver², Murat Bektas¹, Sarvan Aghamuradov¹, Burak Ince¹, Nevzat Koca¹, Ege Sinan Torun¹, Numune Aliyeva¹, Selma Sari¹, Cigdem Cetin³, Banu Cicek Yalcin Dulundu¹, Rabia Deniz⁴, Fatih Kemik⁴, Besim Fazil Agargun⁴, Ubeyde Ayse Gulseren⁴, Beliz Besisik⁴, Onur Alkan⁴, Ceren Bağrıaçık⁴, Yavuz Burak Tor⁴, Naci Senkal⁴, Yunus Catma⁴, Gorkem Durak⁵, Sevim Mese⁶, Ali Agacfidan⁶, Murat Kose⁴, Mustafa Erelel⁷, Arif Atahan Çağatay⁸, Sevgi Kalayoglu Besısık⁹, Figen Esen⁷ and Ahmet Gül¹⁰, ¹Istanbul Faculty of Medicine, Istanbul University, Division of Rheumatology, İstanbul, Turkey, ²Department of Biostatistics, Istanbul Medical Faculty, İstanbul, Turkey,, Istanbul, Turkey, ³Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey, ⁴Istanbul Faculty of Medicine, Istanbul University, Department of Internal Medicine, İstanbul, Turkey, ⁵Istanbul Faculty of Medicine, Istanbul University, Department of Radiology, İstanbul, Turkey, ⁶Istanbul Faculty of Medicine, Istanbul University, Department of Medical Microbiology, Division of Virology and Fundamental Immunology, İstanbul, Turkey, ⁷Istanbul Faculty of Medicine, Istanbul University, Department of Anesthesiology, İstanbul, Turkey, ⁸Istanbul Faculty of Medicine, Istanbul University, Department of Infectious Diseases and Clinical Microbiology, İstanbul, Turkey, ⁹Istanbul Faculty of Medicine, Istanbul University, Division of Hematology and Therapeutic Apheresis Unit, İstanbul, Turkey, ¹⁰Division of Rheumatology, Istanbul University Istanbul School of Medicine, Istanbul, Turkey
Background/Purpose: COVID-19 runs a severe disease associated with acute respiratory distress syndrome in a subset of patients, and a hyperinflammatory response developing in the second…
Abstract Number: 1547 • ACR Convergence 2021
Potential Predictors of Outcome for Anakinra Treatment in COVID-19 Patients with Macrophage Activation Syndrome
Shirkhan Amikishiyev¹, Rabia Deniz², Mehmet Guven Gunver³, Sarvan Aghamuradov¹, Nevzat Koca¹, Burak Ince¹, Murat Bektas¹, Gorkem Durak⁴, Murat Kose², Mustafa Erelel⁵, Arif Atahan Çağatay⁶, Sevgi Kalayoglu Besısık⁷, Figen Esen⁵ and Ahmet Gül⁸, ¹Istanbul Faculty of Medicine, Istanbul University, Division of Rheumatology, İstanbul, Turkey, ²Istanbul Faculty of Medicine, Istanbul University, Department of Internal Medicine, İstanbul, Turkey, ³Department of Biostatistics, Istanbul Medical Faculty, İstanbul, Turkey,, Istanbul, Turkey, ⁴Istanbul Faculty of Medicine, Istanbul University, Department of Radiology, İstanbul, Turkey, ⁵Istanbul Faculty of Medicine, Istanbul University, Department of Anesthesiology, İstanbul, Turkey, ⁶Istanbul Faculty of Medicine, Istanbul University, Department of Infectious Diseases and Clinical Microbiology, İstanbul, Turkey, ⁷Istanbul Faculty of Medicine, Istanbul University, Division of Hematology and Therapeutic Apheresis Unit, İstanbul, Turkey, ⁸Division of Rheumatology, Istanbul University Istanbul School of Medicine, Istanbul, Turkey
Background/Purpose: Coronavirus Disease 2019 (COVID-19) runs a severe course in a subset of patients with acute respiratory distress syndrome and multiorgan failure, and a hyperinflammatory…

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