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
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)
Article
Rheumatology
Johannes C. Nossent, Erin Kelty, Helen Keen, David Preen, Charles Inderjeeth
Summary: Systemic juvenile idiopathic arthritis (S-JIA) is a rare, potentially life-threatening autoinflammatory condition in children. A study investigated the epidemiological characteristics and long-term outcomes of S-JIA in the Australasian region. The annual incidence of S-JIA was 0.61/100,000, with a stable prevalence rate of 7.15/100,000. Disease flares occurred in 24% of patients during a median follow-up of 8 years, with higher rates in boys than girls. No deaths were reported, but there were substantial rates of readmission and emergency department visits for other illnesses.
RHEUMATOLOGY INTERNATIONAL
(2023)
Review
Immunology
Bert Malengier-Devlies, Mieke Metzemaekers, Carine Wouters, Paul Proost, Patrick Matthys
Summary: Neutrophils play a crucial role in the innate immune system, using both oxidative and non-oxidative defense mechanisms to eradicate pathogens, and also act as decision shaping cells guiding other leukocytes for immune responses. The development and release of neutrophils are tightly regulated under both homeostatic and emergency conditions.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Medicine, General & Internal
William G. Ambler, Kabita Nanda, Karen Brandt Onel, Susan Shenoi
Summary: This review focuses on the treatment options and potential future therapeutics for systemic juvenile idiopathic arthritis (SJIA). Recent advancements in targeted therapies have improved patient outcomes, but there are still subsets of patients with refractory disease and severe complications.
ANNALS OF MEDICINE
(2022)
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
Medicine, General & Internal
Monika Ostrowska, Emil Michalski, Piotr Gietka, Malgorzata Manczak, Magdalena Posadzy, Iwona Sudol-Szopinska
Summary: This study compared MRI findings between patients clinically suspected of Juvenile Idiopathic Arthritis (JIA) and patients with ankle arthralgia of unknown cause. The results showed that inflammatory features were observed on MRI, with tenosynovitis being significantly more common in JIA patients. However, the MRI summarized score did not effectively differentiate between JIA and non-JIA patients.
JOURNAL OF CLINICAL MEDICINE
(2022)
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
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
Rheumatology
Ruth Costello, Janet McDonagh, Kimme Hyrich, Jenny Humphreys
Summary: This study provides updated national estimates of the incidence and prevalence of JIA in the UK. It finds that there has been no significant change in the rates over the past 25 years and that Scotland has higher rates compared to England.
Review
Medicine, General & Internal
Duilio Petrongari, Paola Di Filippo, Francesco Misticoni, Giulia Basile, Sabrina Di Pillo, Francesco Chiarelli, Marina Attanasi
Summary: Systemic juvenile idiopathic arthritis associated with lung disorders (sJIA-LD) is a subtype of sJIA characterized by chronic life-threatening pulmonary disorders. It was exceptionally rare before 2013, but the reported prevalence is now approximately 6.8%. Clinically, children with sJIA-LD exhibit relatively mild symptoms, but the severity of pulmonary inflammation is high. sJIA-LD is often associated with early onset, macrophage activation syndrome, and high interleukin-18 circulating levels.
Article
Rheumatology
Nicolino Ruperto, Hermine Brunner, Athimalaipet Ramanan, Gerd Horneff, Ruben Cuttica, Michael Henrickson, Jordi Anton, Alina Lucica Boteanu, Inmaculada Calvo Penades, Kirsten Minden, Heinrike Schmeling, Markus Hufnagel, Jennifer E. Weiss, Manuela Pardeo, Kabita Nanda, Johannes Roth, Nadina Rubio-Perez, Joy C. Hsu, Sunethra Wimalasundera, Chris Wells, Kamal Bharucha, Wendy Douglass, Min Bao, Navita L. Mallalieu, Alberto Martini, Daniel Lovell, Fabrizio De Benedetti
Summary: The study found that the dosing regimens of s.c. tocilizumab in systemic JIA and polyarticular JIA are similar to i.v. tocilizumab with good efficacy. Additionally, the subcutaneous administration route is more convenient and suitable for at-home use.
Article
Rheumatology
Masaki Shimizu, Kenichi Nishimura, Naomi Iwata, Takahiro Yasumi, Hiroaki Umebayashi, Yasuo Nakagishi, Yuka Okura, Nami Okamoto, Noriko Kinjo, Mao Mizuta, Masato Yashiro, Junko Yasumura, Hiroyuki Wakiguchi, Tomohiro Kubota, Mariko Mouri, Utako Kaneko, Masaaki Mori
Summary: A multicenter, retrospective study in Japan revealed that the first-line treatment for MAS in pediatric rheumatology institutes is methylprednisolone pulse therapy and/or cyclosporine A. Dexamethasone palmitate (DEX-P) can be considered as an effective and safe therapeutic option for patients with corticosteroid-resistant MAS.
INTERNATIONAL JOURNAL OF RHEUMATIC DISEASES
(2023)
Article
Rheumatology
Justine Maller, Emily Fox, K. T. Park, Sarah Sertial Paul, Kevin Baszis, Charlotte Borocco, Sampath Prahalad, Pierre Quartier, Adam Reinhardt, Dieneke Schonenberg-Meinema, Lauren Shipman-Duensing, Maria Teresa Terreri, Julia Simard, Idit Lavi, Elizabeth Chalom, Joyces Hsu, Devy Zisman, Elizabeth D. Mellins
Summary: The occurrence of inflammatory bowel disease in the setting of systemic juvenile idiopathic arthritis is rare, with patients showing favorable response to tumor necrosis factor-alpha inhibitors.
JOURNAL OF RHEUMATOLOGY
(2021)
Article
Rheumatology
Vivian E. Saper, Michael J. Ombrello, Adriana H. Tremoulet, Gonzalo Montero-Martin, Sampath Prahalad, Scott Canna, Chisato Shimizu, Gail Deutsch, Serena Y. Tan, Elaine F. Remmers, Dimitri Monos, Timothy Hahn, Omkar K. Phadke, Elaine Cassidy, Ian Ferguson, Vamsee Mallajosyula, Jianpeng Xu, Jaime S. Rosa Duque, Gilbert T. Chua, Debopam Ghosh, Ann Marie Szymanski, Danielle Rubin, Jane C. Burns, Lu Tian, Marcelo A. Fernandez-Vina, Elizabeth D. Mellins, Jill A. Hollenbach
Summary: Drug reaction with eosinophilia and systemic symptoms (DRESS) can occur in Still's disease patients treated with IL-1/IL-6 inhibitors, with features such as eosinophilia, liver enzyme elevation, and persistent rash. HLA-DRB1*15 haplotypes are significantly enriched in Still's disease with DRESS. Pre-prescription HLA typing and vigilance for serious reactions to these drugs are recommended.
ANNALS OF THE RHEUMATIC DISEASES
(2022)
Article
Biochemical Research Methods
Niclas Olsson, Wei Jiang, Lital N. Adler, Elizabeth D. Mellins, Joshua E. Elias
Summary: This study investigates the effects of DO:DM ratios on peptide presentation by creating a panel of HLA-DR4-expressing APC lines and analyzing over 10,000 unique DR4-presented peptides. The findings provide insights into the characteristics that influence peptide presentation and have implications for HLA-II prediction algorithms and research strategies.
MOLECULAR & CELLULAR PROTEOMICS
(2022)
Article
Cell Biology
Saborni Chakraborty, Joseph C. Gonzalez, Benjamin L. Sievers, Vamsee Mallajosyula, Srijoni Chakraborty, Megha Dubey, Usama Ashraf, Bowie Yik-Ling Cheng, Nimish Kathale, Kim Quyen Thi Tran, Courtney Scallan, Aanika Sinnott, Arianna Cassidy, Steven T. Chen, Terri Gelbart, Fei Gao, Yarden Golan, Xuhuai Ji, Seunghee Kim-Schulze, Mary Prahl, Stephanie L. Gaw, Sacha Gnjatic, Thomas U. Marron, Miriam Merad, Prabhu S. Arunachalam, Scott D. Boyd, Mark M. Davis, Marisa Holubar, Chaitan Khosla, Holden T. Maecker, Yvonne Maldonado, Elizabeth D. Mellins, Kari C. Nadeau, Bali Pulendran, Upinder Singh, Aruna Subramanian, Paul J. Utz, Robert Sherwood, Sheng Zhang, Prasanna Jagannathan, Gene S. Tan, Taia T. Wang
Summary: A damaging inflammatory response in severe COVID-19 is associated with early non-neutralizing, afucosylated IgG antibodies specific to SARS-CoV-2. Human IgG-Fc-gamma receptor interactions regulate inflammation in the lung, and antibodies elicited by mRNA SARS-CoV-2 vaccines have reduced inflammatory potential.
SCIENCE TRANSLATIONAL MEDICINE
(2022)
Article
Cell Biology
Debopam Ghosh, Tho D. Pham, Padma P. Nanaware, Deepanwita Sengupta, Lital N. Adler, Caiyun G. Li, Xiao He, Mary E. O'Mara, Aaron B. Kantor, Khoa D. Nguyen, Yang Yang, Laurence C. Eisenlohr, Peter E. Jensen, Leonore A. Herzenberg, Lawrence J. Stern, Scott D. Boyd, Eliver E. B. Ghosn, Elizabeth D. Mellins
Summary: The absence of H2-M protein affects the survival and immunoglobulin selection of B-1 cells, and also alters the specificity of the B-1 cell clonal repertoire.
Article
Rheumatology
Guangbo Chen, Gail H. Deutsch, Grant S. Schulert, Hong Zheng, SoRi Jang, Bruce Trapnell, Pui Y. Lee, Claudia Macaubas, Katherine Ho, Corinne Schneider, Vivian E. Saper, Adriana Almeida de Jesus, Mark A. Krasnow, Alexei Grom, Raphaela Goldbach-Mansky, Purvesh Khatri, Elizabeth D. Mellins, Scott W. Canna
Summary: This study investigated the immunopathology and identified biomarkers in systemic JIA, MAS, and SJIA-LD by measuring over 1,300 analytes in sera. The proteome alterations in active systemic JIA and MAS overlapped, with novel elevations in heat-shock proteins and glycolytic enzymes.
ARTHRITIS & RHEUMATOLOGY
(2022)
Article
Immunology
Claudia Macaubas, Shamma S. Rahman, Idit Lavi, Amir Haddad, Muna Elias, Deepanwita Sengupta, Devy Zisman, Elizabeth D. Mellins
Summary: Psoriatic arthritis (PsA) is a chronic inflammatory arthritis that affects a significant portion of patients with psoriasis. In this study, high dimensional mass cytometry was used to analyze the levels of phosphorylated STAT3 (pSTAT3) in different immune cell subpopulations from PsA patients. The results showed that during active PsA, multiple immune cell subpopulations had increased levels of pSTAT3, potentially playing a role in the pathophysiology of PsA.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Pediatrics
William Daniel Soulsby, Nayimisha Balmuri, Victoria Cooley, Linda M. Gerber, Erica Lawson, Susan Goodman, Karen Onel, Bella Mehta
Summary: Social determinants of health have a significant impact on the outcomes of rheumatoid arthritis treatment. Our study found that community poverty level, race/ethnicity, and economic factors are associated with disease activity and functional disability in polyarticular juvenile idiopathic arthritis patients.
PEDIATRIC RHEUMATOLOGY
(2022)
Article
Rheumatology
Natalie J. Shiff, Peter Shrader, Colleen K. Correll, Anne Dennos, Thomas Phillips, Timothy Beukelman
Summary: This study describes the trajectories of disease activity scores in patients with Juvenile Arthritis (JIA) over a two-year period. Five distinct trajectories were identified, and their associated baseline characteristics were analyzed.
Article
Allergy
Hesam Movassagh, Mary Prunicki, Abhinav Kaushik, Xiaoying Zhou, Diane Dunham, Eric M. M. Smith, Ziyuan He, German R. Aleman R. Muench, Minyi Shi, Annika K. K. Weimer, Shu Cao, Sandra Andorf, Amir Feizi, Michael P. P. Snyder, Pejman Soroosh, Elizabeth D. D. Mellins, Kari C. C. Nadeau
Summary: This study found that exposure to fine particulate matter (PM2.5) leads to specific alterations in monocytes, especially in children with asthma. Further mechanistic research revealed that fine particulate matter induces innate trained immunity in circulating monocytes, resulting in an enhanced inflammatory response. These findings suggest that the alterations of monocytes may serve as an immune signature for pediatric asthma, and pollution-induced trained immunity could be a potential therapeutic target for asthmatic children.
Article
Developmental Biology
Agnieszka Kalinowski, Lu Tian, Reenal Pattni, Hanna Ollila, Maroof Khan, Cindy Manko, Melissa Silverman, Meiqian Ma, Laurie Columbo, Bahare Farhadian, Susan Swedo, Tanya Murphy, Mats Johnson, Elisabeth Fernell, Christopher Gillberg, Margo Thienemann, Elizabeth D. Mellins, Douglas F. Levinson, Alexander E. Urban, Jennifer Frankovich
Summary: Pediatric Acute-onset Neuropsychiatric Syndrome (PANS) is a sudden-onset neuropsychiatric disorder with an increased prevalence of co-morbid autoimmune illness, particularly arthritis. PANS patients with low C4B copy number are at higher risk for subsequent Juvenile Idiopathic Arthritis (JIA) diagnosis. There is a possible association between low C4B and PANS age of onset, suggesting a potential role of C4B in different types of arthritis.
DEVELOPMENTAL NEUROSCIENCE
(2023)
Article
Developmental Biology
Allison Vreeland, Denise Calaprice, Noga Or-Geva, Richard E. Frye, Dritan Agalliu, Herbert M. Lachman, Christopher Pittenger, Stefano Pallanti, Kyle Williams, Meiqian Ma, Margo Thienemann, Antonella Gagliano, Elizabeth Mellins, Jennifer Frankovich
Summary: Post-infectious neuroinflammation is implicated in acute onset obsessive-compulsive disorder. This review article outlines the evidence supporting the role of neuroinflammation in these disorders, including animal models, imaging studies, and research on sleep and metabolites. The authors encourage interdisciplinary collaboration to further research on clinical syndromes with neuropsychiatric manifestations.
DEVELOPMENTAL NEUROSCIENCE
(2023)
Article
Rheumatology
Kunjan Khanna, Hui Yan, Muneshwar Mehra, Nidhi Rohatgi, Gabriel Mbalaviele, Elizabeth D. Mellins, Roberta Faccio
Summary: This study reveals the regulatory mechanism of NLRP3 inflammasome in systemic juvenile idiopathic arthritis (sJIA). The down-regulation of TMEM178 levels is associated with disease activity and may serve as a marker for identifying patients who would benefit from inflammasome targeting.
ARTHRITIS & RHEUMATOLOGY
(2023)
Article
Engineering, Biomedical
Fuqiang Ren, Feifei Wang, Ani Baghdasaryan, Ying Li, Haoran Liu, Rusiou Hsu, Chuchu Wang, Jiachen Li, Yeteng Zhong, Felix Salazar, Chun Xu, Yingying Jiang, Zhuoran Ma, Guanzhou Zhu, Xiang Zhao, Kerry Kaili Wong, Richard Willis, K. Christopher Garcia, Anna Wu, Elizabeth Mellins, Hongjie Dai
Summary: This study reports a cancer vaccine that can track and image immune cells non-invasively in vivo, providing insights into the immune responses induced by vaccination. The vaccine rapidly migrates to lymph nodes through lymphatic vessels and eradicates pre-existing tumors with two doses. The use of nanoparticle-based vaccines and imaging probes emitting infrared light has important implications for the design and optimization of immunotherapies.
NATURE BIOMEDICAL ENGINEERING
(2023)
Editorial Material
Health Care Sciences & Services
Jennifer Frankovich, Heer Nanda, Elizabeth D. D. Mellins, Harumi Jyonouchi, Richard G. G. Boles, Stephen J. J. Walker, John Gaitanis, Richard E. E. Frye
JOURNAL OF PERSONALIZED MEDICINE
(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)