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Abstract Number: 1936 • ACR Convergence 2022
A De Novo Dominant-negative PSMB8 mutation is Linked to a More Severe CANDLE-like Phenotype
Sara Alehashemi¹, Adriana Almeida de Jesus², Jonas Johannes Papendorf³, Farzana Bhuyan⁴, Kat Uss⁵, Anvitha Metpally⁶, Bin Lin⁷, Sophia Park⁸, Thais C.L. Moura⁹, Renata R Dias⁹, Mayra B. Dorna⁹, Katia Kozu⁹, Adriana Sallum⁹, Lucia M. A. Campos⁹, Gijs Santen¹⁰, Jesper Kers¹⁰, Onno Teng¹¹, Karin Palmblad¹², Tom Huizinga¹⁰, Frédéric Ebstein¹³, Elke Krüger¹³ and Raphaela Goldbach-Mansky¹⁴, ¹NIH/NIAID/TADS, Clarksville, MD, ²NIAID, NIH, Silver Spring, MD, ³Institut für Medizinische Biochemie und Molekularbiologie, Universitätsmedizin Greifswald, Greifswald, Germany, ⁴National Institutes of Health, Bethesda, MD, ⁵National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, ⁶NIAID, NIH, Bethesda, MD, ⁷NIH, Bethesda, MD, ⁸NIAID, Bethesda, MD, ⁹Children's Hospital of the University of São Paulo, São Paulo, Brazil, ¹⁰Leiden University Medical Center, Leiden, Netherlands, ¹¹Leiden University Medical Center, Leiderdorp, Netherlands, ¹²Karolinska University Hospital, Stockholm, Sweden, ¹³Institut für Medizinische Biochemie und Molekularbiologie (IMBM), Universitätsmedizin Greifswald, Greifswald, Germany, ¹⁴NIH/NIAID, Potomac, MD
Background/Purpose: Homozygote (HM)/compound heterozygote (CH) and digenic (DG) mutations in proteasome subunits cause the autoinflammatory syndrome CANDLE, or proteasome-associated autoinflammatory syndrome (PRAAS). Inflammation is mediated…
Abstract Number: 2015 • ACR Convergence 2022
JAK Selectivity Did Not Affect to the Incidence of Malignancies in the Real-World Setting: Data from a Multicenter Observational Study in Japan
Tomohisa Uchida¹, Naoki Iwamoto², Shoichi Fukui², Fumiko Shomura³, Koichiro Aratake³, Toshiyuki Aramaki⁴, Yukitaka Ueki⁴ and Atsushi Kawakami², ¹Department of Rheumatology, National Hospital Organization Ureshino Medical Center, Department of Immunology and Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Ureshino, Japan, ²Department of Immunology and Rheumatology, Graduate School of Biomedical Sciences, Nagasaki University, Nagasaki, Japan, ³Department of Rheumatology, National Hospital Organization Ureshino Medical Center, Ureshino, Japan, ⁴Department of Rheumatology, Sasebo Chuo Hospital, Sasebo, Japan

Background/Purpose: Tofacitinib and baricitinib are the first and second approved JAK inhibitors for rheumatoid arthritis (RA) patients. Although their clinical efficacy has been well established,…
Abstract Number: 2220 • ACR Convergence 2022
Recurrence of Herpes Zoster Reactivation After Events of Herpes Zoster in Patients Receiving JAK Inhibitors in South Korea: A Nationwide Population-based Study
YoungEun Kim¹, Ye-Jee Kim², Seonok Kim², Soo Min Ahn¹, Ji Seon Oh³, Yong Gil Kim¹, Chang Keun Lee¹, Bin Yoo¹, Sang Hyoung Park⁴ and Seokchan Hong¹, ¹Department of Rheumatology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea, Seoul, Republic of Korea, ²Department of Clinical Epidemiology and Biostatistics, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea, Seoul, Republic of Korea, ³Department of Information Medicine, Big Data Research Center, Asan Medical Center, Seoul, Republic of Korea, ⁴Department of Gastroenterology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea, Seoul, Republic of Korea
Background/Purpose: Janus kinase (JAK) inhibitors are known to increase the risk of herpes zoster (HZ) reactivation; however, studies on the risk of HZ recurrence when…
Abstract Number: 0195 • ACR Convergence 2021
JAK Inhibitors in Refractory Adult and Childhood-Onset Still’s Disease
Louise GILLARD¹, Stéphane Mitrovic², Jacques Pouchot³, Fleur Cohen¹, Martin Michaud⁴, Heloise Reumaux⁵, Isabelle Kone-Paut⁶ and Bruno Fautrel⁷, ¹Pitié Salpêtrière Hospital, Paris, France, ²Pitié Salpêtrière Hospital - Institut Mutaliste Montsouris, Paris, France, ³AP-HP, RUEIL-MALMAISON, France, ⁴Joseph Ducuing Hospital, Toulouse, France, ⁵Jeanne de Flandre Hospital, Lille, France, ⁶APHP, Bicetre hospital, Le Kremlin bictre, France, ⁷Pitié Salpêtrière Hospital, APHP, Sorbonne Université, Paris, France
Background/Purpose: Excessive and inappropriate production of pro-inflammatory cytokines such as interleukin IL-1, IL-6 or IL-18, is a pathogenic cornerstone in adult and childhood onset Still’s…
Abstract Number: 0509 • ACR Convergence 2021
Selective Inhibition of Tyrosine Kinase 2 with Deucravacitinib Compared with Janus Kinase 1/2/3 Inhibitors
Anjaneya Chimalakonda¹, James Burke¹, Lihong Cheng¹, Ian Catlett¹, Michael Tagen¹, Qihong Zhao¹, Aditya Patel¹, Jun Shen¹, Ihab Girgis¹, Subhashis Banerjee¹ and John Throup², ¹Bristol Myers Squibb, Princeton, NJ, ²Bristol Myers Squibb, Princeton
Background/Purpose: Deucravacitinib is a novel, oral, allosteric agent that selectively inhibits intracellular signaling by binding to the tyrosine kinase 2 (TYK2) pseudokinase domain rather than…
Abstract Number: 0812 • ACR Convergence 2021
Are Biologic and Targeted Synthetic Disease Modifying Anti-Rheumatic Drugs Associated with Work Participation Improvement in Early Rheumatoid Arthritis? A Systematic Review and Meta-analysis
Mary Lucy Marques¹, Alessia Alunno², Louise Falzon³, Annelies Boonen⁴ and Sofia Ramiro¹, ¹Leiden University Medical Center, Leiden, Netherlands, ²University of Perugia, Perugia, Italy, ³Center for Personalized Health, Northwell Health, New York City, NY, ⁴Department of Internal Medicine, Division of Rheumatology, Maastricht University Medical Centre+ (MUMC+), Maastricht, Limburg, Netherlands
Background/Purpose: Treatment of early RA with DMARDs is associated with particularly effective responses, resulting in sustained beneficial outcomes, including clinical and radiographic remission and optimal…
Abstract Number: 0841 • ACR Convergence 2021
Persistence on JAK Inhibitors in Daily Practice: Evaluation of the Rhadar-registry
Linus Risser¹, Torsten Witte², Peter Bartz-Bazzanella³, Cay Von der Decken⁴, Johannes Knitza⁵, Sándor P. Fekete⁶, Alexander Zink⁷, Georg Gauler⁸, Patrick Wurth⁹, Peer Aries¹⁰, Kirsten Karberg¹¹, Christoph Kuhn¹², Florian Schuch¹³, Susanna Späthling-Mestekemper¹⁴, Wolfgang Vorbrüggen¹⁵, Mathias Englbrecht¹⁶, Martin Welcker¹⁷ and Stefan Kleinert¹⁸, ¹Medizinische Hochschule Hannover, Hannover, Germany, ²Hannover Medical School, Hannover, Germany, ³Rhein-Maas Klinikum, Wuerselen, Germany, ⁴Klinik für Internistische Rheumatologie, Rhein-Maas-Klinikum, Würselen, Germany, ⁵Department of Internal Medicine 3 Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nrnberg, University Hospital Erlangen, Erlangen, Germany, ⁶Department of Computer Science, TU Braunschweig, Braunschweig, Germany, ⁷Department of Dermatology and Allergy, School of Medicine, Technical University of Munich, Munich, Germany, ⁸rheumapraxis, Osnabrck, Germany, ⁹Rheumatologische Schwerpunktpraxis, Osnabrück, Germany, ¹⁰Rheumatologie in Hamburg, Hamburg, Germany, ¹¹Praxis für Rheumatologie und Innere Medizin, Berlin, Germany, Berlin, Germany, ¹²Praxis für Rheumatologie, Karlsruhe, Germany, Karlsruhe, Germany, ¹³Praxisgemeinschaft Rheumatologie-Nephrologie, Erlangen, Germany, ¹⁴Rheumapraxis München, München, Germany, ¹⁵Verein zur Förderung der Rheumatologie e.V., Würselen, Germany, ¹⁶Freelance Healthcare Data Scientist, Eckental, Germany., Eckental, Germany, ¹⁷MVZ für Rheumatologie, Planegg, Germany, ¹⁸Praxisgemeinschaft Rheumatologie - Nephrologie (PGRN), Erlangen, Germany
Background/Purpose: The treatment strategy for rheumatoid arthritis (RA) includes achieving remission or at least low disease activity within six months of starting therapy. Active RA…
Abstract Number: 1208 • ACR Convergence 2021
Composite Articular Index Including Acute Phase Reactants Should Not Be Used in Patients with Rheumatoid Arthritis Treated with Il6 Inhibitors but May Be Useful in Those Receiving Jak Inhibitors: Ultrasound Evidence
Andrés Ponce¹, Beatriz Frade-Sosa², Ramirez Julio³, Núria Sapena¹, Roberto Gumucio², Virginia Ruiz-Esquide², Rosa Morlà², Marta Bassas², Juan D Cañete⁴ and Jose A Gomez-Puerta², ¹Rheumatology Department, Hospital Clínic, Barcelona, Spain, ²Hospital Clínic de Barcelona, Barcelona, Spain, ³Hospital Universitari Clinic, Barcelona, Spain, ⁴Rheumatology Department, Hospital Clinic, Barcelona, Spain
Background/Purpose: IL6 inhibitors (IL6i) have a dramatic effect on the reduction of C reactive protein (CRP) that does not always correlate with a significant improvement…
Abstract Number: 1209 • ACR Convergence 2021
Plasma Calprotectin as a Biomarker of Active Synovitis in Rheumatoid Arthritis: A Clinical and Ultrasound Study in Patients Treated with Il6 and JAK Inhibitors
Beatriz Frade-Sosa¹, Andrés Ponce², Roberto Gumucio³, José inciarte-Mundo⁴, María Torradeflot⁵, Virginia Ruiz-Esquide³, Rosa Morlà², Ana Belén Azuaga⁶, Núria Sapena², Marta Bassas², Julio Ramirez³, Juan D Cañete⁶, Jose A Gomez-Puerta¹ and Raimon Sanmarti¹, ¹Hospital Clínic de Barcelona, Barcelona, Spain, ²Rheumatology Department, Hospital Clínic, Barcelona, Spain, ³Rheumatology departmen. Hospital Clinic de Barcelona, Barcelona, Spain, ⁴Institut d'Investigacions Biomèdiques August Pi i Sunyer,( IDIBAPS), Barcelona, Spain, ⁵Immunology Department, Centre de Diagnòstic Biomèdic, Hospital Clínic de Barcelona, Universitat de Barcelona, IDIBAPS, Barcelona, Spain, ⁶Rheumatology Department, Hospital Clinic, Barcelona, Spain
Background/Purpose: IL6 inhibitors (IL6i) have a dramatic effect on acute phase reactants (AFR) such as C reactive protein (CRP) in rheumatoid arthritis (RA). However, CRP…
Abstract Number: 1245 • ACR Convergence 2021
Characteristics of RA Patients Treated with JAK Inhibitors Before versus After VTE Warnings: Results of a Real-World Multicentric Study
Cecile PHILIPPOTEAUX¹, Valentine DEPREZ², Jean-Guillaume LETAROUILLY¹, Emeline CAILLIAU³, Eric HOUVENAGEL⁴, Xavier DEPREZ⁵, Aurore NOTTEZ⁶, Peggy PHILIPPE¹, Tristan PASCART⁷, Vincent GOEB² and Rene-Marc FLIPO⁸, ¹Rheumatology Department, University Hospital of Lille, Lille, France, ²Rheumatology Department, University Hospital of Amiens, Amiens, France, ³Assessment of Health Technologies and Medical Practices Department, University Hospital of Lille, Lille, France, ⁴Rheumatology Department, Lille Catholic Hospitals, Lomme, France, ⁵Rheumatology Department, Hospital Center of Valenciennes, Valenciennes, France, ⁶Rheumatology Department, University Hospital of Dunkerque, Dunkerque, France, ⁷Department of Rheumatology, Lille Catholic Hospitals, Lomme, France, ⁸Rheumatology Department, Lille University Hospital, Lille, France
Background/Purpose: In recent decades, the therapeutic arsenal in RA has dramatically expanded. Baricitinib (BARI) and Tofacitinib (TOFA) were the first JAK inhibitors (JAKi) to be…
Abstract Number: 1486 • ACR Convergence 2021
A Novel Requirement for IFNβ1 Signaling for IFNκ Induction in Keratinocytes
Bin Xu¹, Yee Sun Tan¹, Jon Musai¹, William R Swindell², Mrinal Sakar¹, Johann Gudjonsson¹, J. Michelle Kahlenberg¹ and Grace Hile¹, ¹University of Michigan, Ann Arbor, MI, ²Ohio University, Cincinnati, OH
Background/Purpose: Cutaneous lupus erythematosus (CLE) affects up to 70% of patients with systemic lupus erythematosus (SLE), and type I interferons (IFNs) are important promoters of…
Abstract Number: 1545 • ACR Convergence 2021
Safety of JAK Inhibitor in Patients with Rheumatoid Arthritis Who Developed Reactivation of Herpes Zoster Virus After Receiving JAK Inhibitor
Wonho Choi¹, Soo Min Ahn², Yong Gil Kim¹, Chang-Keun Lee¹, Bin Yoo² and Seokchan Hong¹, ¹Asan Medical Center, Seoul, Republic of Korea, ²ASAN MEDICAL CENTER, Seoul, South Korea
Background/Purpose: Janus kinase inhibitor (JAKi) increases the risk of the reactivation of herpes zoster (HZ) virus and may thus be temporarily discontinued in cases of…
Abstract Number: 0010 • ACR Convergence 2020
Antirheumatic Disease Therapies in Patients with COVID-19: A Systematic Review and Meta-analysis
Michael Putman¹, Yu Pei Eugenia Chock², Herman Tam³, Alfred Kim⁴, Sebastian Sattui⁵, Francis Berenbaum⁶, Maria (Maio) Danila⁷, Peter Korsten⁸, Catalina Sanchez Alvarez⁹, Jeffrey Sparks¹⁰, Laura Coates¹¹, Candace Palmerlee¹², Andrea Pierce¹³, Arundathi Jayatilleke¹⁴, Sindhu Johnson¹⁵, Adam Kilian¹⁶, Jean Liew¹⁷, Larry Prokop⁹, Hassan Murad⁹, Rebecca Grainger¹⁸, Zachary Wallace¹⁹ and Ali Duarte-Garcia⁹, ¹Northwestern University, Chicago, IL, ²Yale School of Medicine, Greenwich, CT, ³The Hospital for Sick Children, Toronto, ON, Canada, ⁴Washington University School of Medicine, St. Louis, MO, ⁵Hospital for Special Surgery, New York, NY, ⁶Sorbonne Universit�, Paris, France, ⁷University of Alabama at Birmingham (UAB), Birmingham, AL, ⁸University Medical Center Göttingen, Gottingen, Germany, ⁹Mayo Clinic, Rochester, MN, ¹⁰Division of Rheumatology, Inflammation, and Immunity; Brigham and Women’s Hospital and Harvard Medical School, Boston, MA, ¹¹University of Oxford, Oxford, United Kingdom, ¹²12. Patient Research Partner, Berkeley, CA, ¹³Patient Research Partner, New York City, ¹⁴Lewis Katz School of Medicine, Temple University, Philadelphia, PA, ¹⁵University of Toronto, Toronto, ON, Canada, ¹⁶George Washington University, Washington, DC, ¹⁷University of Washington, Seattle, WA, ¹⁸University of Otago, Wellington, New Zealand, ¹⁹Massachusetts General Hospital, Newton, MA
Background/Purpose: Antirheumatic disease therapies have been used to treat coronavirus disease 2019 (COVID-19) and its complications. There has been particular interest in the antimalarial agent…
Abstract Number: 0145 • ACR Convergence 2020
Treat-to-Target in Rheumatoid Arthritis: Rates of Treatment Changes in Patients Engaged with Care Management Services Compared to Historically Reported National Registry Based Estimates
Pranav Patel¹, Andrew Krueger², Aliza Lipson³, Michele Hamburger⁴ and Craig Hunter⁵, ¹CVS Health, Toledo, OH, ²Accordant Health Services, Greensboro, NC, ³Emory University, Palmetto Bay, FL, ⁴Frances Hamburger Institute, Hauppauge, NY, ⁵CVS Health, Northbrook
Background/Purpose: Treat-to-target is the foundation of rheumatoid arthritis (RA) management. Prioritizing routine measurement of patient-reported disease activity along with conventional physician, laboratory and imaging assessments…
Abstract Number: 0231 • ACR Convergence 2020
Treatment Patterns of Biologic/Targeted Synthetic DMARDs for the Management of Rheumatoid Arthritis in Australia: An Analysis of the OPAL Dataset
Helen Cooley¹, Tegan Smith², Peter Youssef³, Kathleen Tymms⁴, David Mathers⁵, Sabina Ciciriello⁶, Hedley Griffiths⁷, Catherine OSullivan² and Geoffrey Littlejohn⁸, ¹Hobart Private Hospital, Hobart, TAS, Taroona, Australia, ²OPAL Rheumatology Ltd, Sydney, NSW, Kogarah, New South Wales, Australia, ³University of Sydney, Sydney, NSW; Royal Prince Alfred Hospital, Camperdown, NSW, Camperdown, New South Wales, Australia, ⁴Canberra Rheumatology, Canberra, ACT, Canberra, Australian Capital Territory, Australia, ⁵Georgetown Rheumatology, Georgetown, NSW, Georgetown, New South Wales, Australia, ⁶Royal Melbourne Hospital, Melbourne, VIC, Melbourne, Victoria, Australia, ⁷Barwon Rheumatology Service, Geelong, VIC, Geelong, Victoria, Australia, ⁸Monash Rheumatology, Clayton, VIC; OPAL Rheumatology Ltd, Sydney, NSW, Melbourne, Victoria, Australia
Background/Purpose: In Australia the cost of biological/targeted synthetic DMARDs (b/tsDMARDs) for treatment of RA is subsidized if the patient has documented high levels of clinical/laboratory…

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