Article
Rheumatology
Arianna De Matteis, Claudia Bracaglia, Denise Pires Marafon, Anna Lucia Piscitelli, Maria Alessio, Roberta Naddei, Francesca Orlando, Giovanni Filocamo, Francesca Minoia, Angelo Ravelli, Jessica Tibaldi, Rolando Cimaz, Achille Marino, Gabriele Simonini, Maria Vincenza Mastrolia, Francesco La Torre, Ilaria Tricarico, Francesco Licciardi, Davide Montin, Maria Cristina Maggio, Clotilde Alizzi, Giorgia Martini, Adele Civino, Romina Gallizzi, Alma Nunzia Olivieri, Francesca Ardenti Morini, Giovanni Conti, Fabrizio De Benedetti, Manuela Pardeo
Summary: This study used real-world data to evaluate the effectiveness and safety of canakinumab in Italian patients with sJIA. The results showed that canakinumab was effective in treating sJIA and had no serious adverse events. The history of MAS and higher number of active joints were associated with poorer treatment outcomes.
Article
Rheumatology
Tanja Hinze, Christoph Kessel, Claas H. Hinze, Julia Seibert, Hermann Gram, Dirk Foell
Summary: In patients with SJIA treated with canakinumab, baseline serum biomarkers such as IL-18 and IFN-gamma levels are associated with treatment outcome. Higher IL-18: CXCL9 and IFN-gamma: CXCL9 ratios at baseline are correlated with a better clinical response to canakinumab treatment. Further studies are needed to validate these findings and their applicability to patients with recent MAS.
Article
Rheumatology
Nicolino Ruperto, Hermine Brunner, Cesar Pacheco-Tena, Ingrid Louw, Gabriel Vega-Cornejo, Alberto J. Spindler, Daniel J. Kingsbury, Heinrike Schmeling, Arturo Borzutzky, Ruben Cuttica, C. J. Inman, Victor Malievskiy, Christiaan Scott, Vladimir Keltsev, Maria Teresa Terreri, Diego Oscar Viola, Ricardo M. Xavier, Taciana A. Pedrosa Fernandes, Maria Del Rocio Maldonado Velazquez, Michael Henrickson, Michael B. Clark, Karen A. Bensley, Xiaoming Li, Kim Hung Lo, Jocelyn H. Leu, Chyi-Hung Hsu, Elizabeth C. Hsia, Zhenhua Xu, Alberto Martini, Daniel J. Lovell
Summary: Intravenous golimumab was effective, showed consistent pharmacokinetics, and was relatively safe in pediatric patients with polyarticular-course JIA. Treatment led to significant JIA ACR response rates at 28 and 52 weeks, with steady PK exposure maintained. However, the presence of antibodies in some patients and rare serious infections were noted as potential issues.
Article
Pediatrics
Ellen Go, Mira van Veenendaal, Cedric Manlhiot, Rayfel Schneider, Brian W. McCrindle, Rae S. M. Yeung
Summary: This study describes the disease course of a small proportion of patients with both Kawasaki disease and systemic juvenile idiopathic arthritis, characterized by refractory Kawasaki disease, high prevalence of coronary artery dilatation, and shared immunopathology potentially linking the two conditions.
FRONTIERS IN PEDIATRICS
(2021)
Article
Pediatrics
Mikhail M. Kostik, Eugenia A. Isupova, Konstantin Belozerov, Tatyana S. Likhacheva, Evgeny N. Suspitsin, Rinat Raupov, Vera V. Masalova, Irina A. Chikova, Margarita F. Dubko, Olga V. Kalashnikova, Vyacheslav G. Chasnyk, Randy Q. Cron
Summary: This study provides evidence for the efficacy and safety of short-term increased doses (2-3 times normal) of canakinumab in treating MAS associated with sJIA. Further research on the efficacy and safety of increased doses of canakinumab for the treatment of MAS in children with sJIA is warranted.
FRONTIERS IN PEDIATRICS
(2022)
Article
Rheumatology
Pierre Quartier, Ekaterina Alexeeva, Tamas Constantin, Vyacheslav Chasnyk, Nico Wulffraat, Karin Palmblad, Carine Wouters, Hermine Brunner, Katherine Marzan, Rayfel Schneider, Gerd Horneff, Alberto Martini, Jordi Anton, Xiaoling Wei, Alan Slade, Nicolino Ruperto, Ken Abrams
Summary: The study evaluated the efficacy and safety of two canakinumab monotherapy tapering regimens to maintain complete clinical remission in children with systemic JIA. Results showed that reducing canakinumab exposure may be feasible in patients who have achieved clinical remission, but consistent interleukin-1 inhibition is necessary to maintain this response.
ARTHRITIS & RHEUMATOLOGY
(2021)
Article
Rheumatology
Naomi Iwata, Kenichi Nishimura, Ryoki Hara, Tomoyuki Imagawa, Masaki Shimizu, Minako Tomiita, Hiroaki Umebayashi, Syuji Takei, Noriko Seko, Ryota Wakabayashi, Shumpei Yokota
Summary: This study reports the efficacy and safety of canakinumab treatment in Japanese patients with sJIA over a 48-week period. The majority of patients showed a high response rate, regardless of the level of joint involvement at baseline. The treatment resulted in corticosteroid tapering in the majority of patients and the most common adverse events were infections. No new safety findings were reported.
MODERN RHEUMATOLOGY
(2022)
Article
Rheumatology
Jelena Vojinovic, Ivan Foeldvari, Joke Dehoorne, Violeta Panaviene, Gordana Susic, Gerd Horneff, Valda Stanevicha, Katarzyna Kobusinska, Zbigniew Zuber, Bogna Dobrzyniecka, Jonathan Akikusa, Tadej Avcin, Cecilia Borlenghi, Edmund Arthur, Svitlana Y. Tatulych, Chuanbo Zang, Vassilis Tsekouras, Bonnie Vlahos, Alberto Martini, Nicolino Ruperto
Summary: CLIPPER2 is an 8-year study on the safety and efficacy of etanercept in patients with juvenile idiopathic arthritis (JIA). The study found that etanercept treatment was well tolerated and effective in these patients.
Article
Biotechnology & Applied Microbiology
Taner Coskuner, Senguel Caglayan, Ozlem Akgun, Ruya Torun, Emine Nur Sunar Yayla, Ilknur Bagrul, Gulsah Kilbas, Gulcin Otar Yener, Hulya Kose, Kubra Ozturk, Ozge Baba, Mustafa Cakan, Ferhat Demir, Hafize Emine Sonmez, Mukaddes Kalyoncu, Sara Nsebnem Kilic, Selcuk Yuksel, Esra Baglan, Sevcan A. Bakkaloglu, Erbil Unsal, Nuray Aktay Ayaz, Betul Sozeri
Summary: Real-life data from a large cohort of patients suggests that Canakinumab is as safe as claimed in clinical trials.
EXPERT OPINION ON BIOLOGICAL THERAPY
(2023)
Article
Rheumatology
Kenichi Nishimura, Ryoki Hara, Hiroaki Umebayashi, Syuji Takei, Naomi Iwata, Tomoyuki Imagawa, Masaki Shimizu, Minako Tomiita, Noriko Seko, Tetsuji Kitawaki, Shumpei Yokota
Summary: The study demonstrates that canakinumab is effective in Japanese patients with sJIA, leading to significant reduction in corticosteroid dose for the majority of patients. Safety analysis shows that the safety profile of canakinumab is consistent with previous studies.
MODERN RHEUMATOLOGY
(2021)
Article
Rheumatology
Hermine I. Brunner, Nicolino Ruperto, Zbigniew Zuber, Ruben Cuttica, Vladimir Keltsev, Ricardo M. Xavier, Ruben Burgos-Vargas, Inmaculada Calvo Penades, Earl D. Silverman, Graciela Espada, Manuel Ferrandiz Zavaler, Yukiko Kimura, Carolina Duarte, Chantal Job-Deslandre, Rik Joos, Wendy Douglass, Sunethra Wimalasundera, Kamal N. Bharucha, Chris Wells, Daniel J. Lovell, Alberto Martini, Fabrizio de Benedetti
Summary: This study aimed to report the 2-year efficacy and safety of tocilizumab (TCZ) in patients with polyarticular-course juvenile idiopathic arthritis (JIA). The results showed high-level disease control for up to 2 years in patients treated with TCZ for polyarticular-course JIA, with a safety profile consistent with previous reports.
ARTHRITIS & RHEUMATOLOGY
(2021)
Article
Pediatrics
Tingyan He, Yu Xia, Ying Luo, Jun Yang
Summary: This study aimed to evaluate the efficacy and potential adverse effects of JAKi in patients with systemic juvenile idiopathic arthritis (SJIA). The results showed that JAKi may be an alternative or adjuvant agent for patients with persistently active disease, glucocorticoid-related adverse reactions, or SJIA-MAS.
FRONTIERS IN PEDIATRICS
(2023)
Article
Cell Biology
Justine Maller, Terry Morgan, Mayu Morita, Frank McCarthy, Yunshin Jung, Katrin J. Svensson, Joshua E. Elias, Claudia Macaubas, Elizabeth Mellins
Summary: The study suggests that intercellular communication mediated by extracellular vesicles plays an important role in the pathogenesis of systemic juvenile idiopathic arthritis. The number and cellular sources of extracellular vesicles differ between the active and inactive states of the disease and healthy controls.
JOURNAL OF LEUKOCYTE BIOLOGY
(2023)
Review
Rheumatology
Claas H. Hinze, Dirk Foell, Christoph Kessel
Summary: Systemic juvenile idiopathic arthritis (sJIA) is a disease characterized by severe systemic inflammation and arthritis. It poses challenges to rheumatologists treating pediatric and adult patients worldwide. Although treatment plans exist for classic sJIA, there is still a lack of clear treatment approaches for early sJIA without arthritis and complicated sJIA.
NATURE REVIEWS RHEUMATOLOGY
(2023)
Article
Pediatrics
Ekaterina Alexeeva, Elizaveta Krekhova, Tatyana Dvoryakovskaya, Ksenia Isaeva, Aleksandra Chomakhidze, Evgeniya Chistyakova, Olga Lomakina, Rina Denisova, Anna Mamutova, Anna Fetisova, Marina Gautier, Dariya Vankova, Ivan Kriulin, Ruslan Saygitov
Summary: This study investigated the switch from tocilizumab to canakinumab in sJIA patients and found that one-year canakinumab therapy was potentially effective as second-line biologic therapy. Outcomes included significant improvement in ACR30 criteria, achievement of inactive disease, and clinical remission in a high percentage of patients.
FRONTIERS IN PEDIATRICS
(2023)
Article
Rheumatology
Marc H. A. Jansen, Christien Rondaan, Geertje E. Legger, Kirsten Minden, Yosef Uziel, Natasa Toplak, Despoina Maritsi, Lotte van den Berg, Guy A. M. Berbers, Patricia Bruijning, Yona Egert, Christophe Normand, Marc Bijl, Helen E. Foster, Isabelle Kone-Paut, Carine Wouters, Angelo Ravelli, Ori Elkayam, Nicolaas M. Wulffraat, Marloes W. Heijstek
Summary: This article updates the EULAR recommendations on vaccination for pedAIIRD patients, providing guidelines on vaccination principles, special circumstances, and the safety for immunosuppressed patients.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Article
Rheumatology
Louise Gillard, Jacques Pouchot, Fleur Cohen-Aubart, Isabelle Kone-Paut, Gael Mouterde, Martin Michaud, Heloise Reumaux, Lea Savey, Alexandre Belot, Bruno Fautrel, Stephane Mitrovic
Summary: JAKi agents can be considered as a therapeutic option for difficult-to-treat Still's disease, especially for patients with partial response to medium- or high-dose corticosteroids or biologics.
Letter
Rheumatology
Anna Gaussem, Perrine Dusser, Van Huynh, Caroline Galeotti, Adrien Schvartz, Lea Jaume, Linda Rossi-Semerano, Isabelle Kone-Paut, Charlotte Borocco
Article
Biochemistry & Molecular Biology
Kourosh Ahmadzadeh, Marie Pereira, Margot Vanoppen, Eline Bernaerts, Jeong-Hun Ko, Tania Mitera, Christy Maksoudian, Bella B. Manshian, Stefaan Soenen, Carlos D. Rose, Patrick Matthys, Carine Wouters, Jacques Behmoaras
Summary: Macrophages can undergo cell fusion and multinucleation to form multinucleated giant cells. Comparative transcriptomics and functional assays reveal that multinucleation causes downregulation of macrophage identity and specialization in functionality, such as enhanced phagocytosis and mitochondrial function.
Article
Pediatrics
Ellen M. Cody, Scott E. Wenderfer, Kathleen E. Sullivan, Alfred H. J. Kim, Wesley Figg, Harneet Ghumman, Tingting Qiu, Bin Huang, Prasad Devarajan, Hermine Brunner
Summary: The Renal Activity Index for Lupus (RAIL) can non-invasively identify lupus nephritis (LN) and assess the efficacy of LN induction therapy. RAIL scores can differentiate clinically active LN from inactive LN or without LN. RAIL scores significantly decrease after complete LN remission.
PEDIATRIC NEPHROLOGY
(2023)
Correction
Biochemistry & Molecular Biology
Kourosh Ahmadzadeh, Marie Pereira, Margot Vanoppen, Eline Bernaerts, Jeong-Hun Ko, Tania Mitera, Christy Maksoudian, Bella B. Manshian, Stefaan Soenen, Carlos D. Rose, Patrick Matthys, Carine Wouters, Jacques Behmoaras
Letter
Medicine, General & Internal
Ellen De Langhe, Sien Van Loo, Bert Malengier-Devlies, Mieke Metzemaekers, Frederik Staels, Jessica Vandenhaute, Nathalie Berghen, Raf Sciot, Anniek Corveleyn, Olga Tsuiko, Mieke Gouwy, Jan Lenaerts, Patrick Verschueren, Carine H. Wouters, Paul Proost, Patrick Matthys, Eric Legius, Rik Schrijvers
Summary: The article reports a case of adult-onset inflammatory disease. It is revealed that two somatic genetic events are responsible for the disease and its onset in adulthood.
NEW ENGLAND JOURNAL OF MEDICINE
(2023)
Article
Rheumatology
Hermine Brunner, Nikolay Tzaribachev, Ingrid Louw, Inmaculada Calvo Penades, Francisco Avila-Zapata, Gerd Horneff, Ivan J. Foeldvari, Daniel Kingsbury, Maria Eliana Paz Gastanaga, Carine Wouters, Johannes Breedt, Robert Wong, Margarita Askelson, Joe Zhuo, Alberto J. Martini, Daniel Lovell, Nicolino Ruperto
Summary: This study aimed to investigate the frequency and trajectories of individual patients with polyarticular-course juvenile idiopathic arthritis (JIA) achieving various composite end points on abatacept treatment. The results showed that patients achieved composite end points of low disease activity (LDA) with minimal pain, LDA with Childhood Health Assessment Questionnaire disability index score of 0, and 50% improvement in American College of Rheumatology criteria for JIA (ACR50) with minimal pain at 4 months. The proportion of patients meeting these end points increased over 21 months of abatacept treatment.
ARTHRITIS CARE & RESEARCH
(2023)
Article
Rheumatology
Laura E. Schanberg, Lily (Yeruk) Mulugeta, Bolanle Akinlade, Hermine I. Brunner, Jianmeng Chen, Robert A. Colbert, Vincent Delgaizo, Marc R. Gastonguay, Rachel Glaser, Lisa Imundo, Daniel J. Lovell, Jocelyn H. Leu, Nael M. Mostafa, Robert M. Nelson, Peter A. Nigrovic, Nikolay P. Nikolov, Lisa G. Rider, Rebecca Rothwell, Chandrahas Sahajwalla, Renu Singh, Vikram Sinha, Carolyn L. Yancey, Lynne Yao
Summary: This report summarizes a workshop on drug development for polyarticular juvenile idiopathic arthritis (pJIA), highlighting the importance of accelerating drug development and the necessity of collaboration between stakeholders.
ARTHRITIS & RHEUMATOLOGY
(2023)
Article
Rheumatology
Ellen M. Cody, Bridget E. Wilson, Ekemini A. Ogbu, Jennifer L. Huggins, Chen Chen, Tingting Qiu, Tracy Ting, Francisco Flores, Bin Huang, Hermine Brunner
Summary: This study compared three treat-to-target (T2T) states and their association with the prognosis of childhood-onset SLE (cSLE). It found that patients with lupus nephritis and pre-existing damage were at higher risk of disease worsening, and suggested using the lupus low disease activity state (LLDAS) as the preferred T2T measure.
LUPUS SCIENCE & MEDICINE
(2023)
Article
Pediatrics
M. Wallimann, K. Bouayed, E. Cannizzaro, D. Kaiser, A. Belot, E. Merlin, S. Poignant, C. Wouters, F. Hofer, T. Saurenmann, A. Koryllou, R. Carlomagno, M. Mejbri, M. Hofer, K. Theodoropoulou
Summary: This study aims to identify predictors of disease evolution in patients with systemic juvenile idiopathic arthritis (sJIA). The results suggest that early disease onset, persistence of arthritis in the first year, and the use of synthetic DMARD may predict a persistent disease course.
PEDIATRIC RHEUMATOLOGY
(2023)
Article
Rheumatology
Hermine Brunner, Aisha Vadhariya, Christina Dickson, Wallace Crandall, Casey Kar-Chan Choong, Julie A. Birt, Nicolino Ruperto, Athimalaipet Ramanan
Summary: This study examined treatment regimens and patterns in childhood-onset SLE (cSLE) and adult-onset SLE (aSLE) cohorts, finding similarities in treatment plans, duration, and adherence. Treatment for cSLE involved more intensive medication use, highlighting the need for safe medications approved for cSLE.
LUPUS SCIENCE & MEDICINE
(2023)
Article
Rheumatology
Robert C. Brucia, Kristen Hayward, Hermine I. Brunner, Maricarmen Lopez-Pena, Susan Shenoi, Arzu Soybilgic, James J. Nocton
Summary: A practice analysis process was used to define the scope of clinical practice of pediatric rheumatology. The resulting documents, including a practice analysis document (PAD) and a test content outline (TCO), can be used to improve training and create relevant certification examinations.
ACR OPEN RHEUMATOLOGY
(2023)
Meeting Abstract
Cardiac & Cardiovascular Systems
Matthias M. Engelen, Quentin Van Thillo, Albrecht Betrains, Iwein Gyselinck, Caroline P. Martens, Val Erie Spalart, Anna Ockerman, Caroline Devooght, Joost Wauters, Jan Gunst, Carine Wouters, Christophe Vandenbriele, Steffen Rex, Laurens Liesenborghs, Alexander Wilmer, Philippe Meersseman, Greet Van den Berghe, Dieter Dauwe, Ann Belmans, Michiel Thomeer, Tom Fivez, Dieter Mesotten, David Ruttens, Luc Heytens, Ilse Dapper, Sebastiaan Tuyls, Brecht De Tavernier, Peter Verhamme, Thomas Vanassche
Article
Immunology
Luna Dillemans, Youri Bekhuis, Albrecht Betrains, Karen Yu, Maarten van Hemelen, Noemie Portner, Lien De Somer, Patrick Matthys, Jeroen Breckpot, Thomas Tousseyn, Marijke Peetermans, Paul Proost, Carine Wouters, Steven Vanderschueren
Summary: We report a fatal case of a 20-year-old woman with refractory adult-onset Still's disease accompanied by fulminant macrophage activation syndrome and atypical hemolytic uremic syndrome. This case highlights the importance of prompt genetic assessment of complement-encoding alleles, in addition to HLH-related genes, in patients with severe adult-onset Still's disease with recurrent macrophage activation syndrome and features of thrombotic microangiopathy.
CLINICAL IMMUNOLOGY
(2023)