Article
Nutrition & Dietetics
Marta Gruca, Justyna Zamojska, Katarzyna Niewiadomska-Jarosik, Agnieszka Wosiak, Aleksandra Stasiak, Karolina Sikorska, Jerzy Stanczyk, Elzbieta Smolewska
Summary: The aim of this study was to assess CVD risk factors in patients with JIA. IMT, metabolic parameters and health behaviors were evaluated. The study found that CVD risk factors were present in both groups, with more JIA patients having abnormal BMI compared to the control group. Dietary habits were not associated with IMT or other parameters in the JIA group. Only one JIA patient met the criteria for ideal cardiovascular health. The pursuit of ideal cardiovascular health should be given attention in the pediatric population, especially in patients with chronic diseases like JIA. Further studies are needed to apply carotid artery IMT measurement in assessing CVD risk.
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
Medicine, General & Internal
Christoph Tappeiner, Han Sol Bae, Kai Rothaus, Karoline Walscheid, Arnd Heiligenhaus
Summary: The study aimed to analyze the occurrence and risk factors for macular edema (ME) in patients with juvenile idiopathic arthritis-associated uveitis (JIA-U). The results indicated that older age, worse eye conditions, and higher laser-flare photometry values were independent risk factors for ME manifestation.
JOURNAL OF CLINICAL MEDICINE
(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
Immunology
Julia E. Rood, Ayman Rezk, Jennifer Pogoriler, Laura S. Finn, Jon M. Burnham, Maureen B. Josephson, Amit Bar-Or, Edward M. Behrens, Scott W. Canna
Summary: This case report describes the improvement of a patient with refractory SJIA-LD after treatment with MAS-825, including pulmonary symptoms, lung inflammation, and burden of immunomodulatory therapy. This suggests that simultaneous targeting of both IL-1 beta and IL-18 may be a safe and effective treatment strategy in SJIA-LD.
JOURNAL OF CLINICAL IMMUNOLOGY
(2023)
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
Holly Wobma, Sage R. Arvila, Maria L. Taylor, Ki Pui Lam, Marina Ohashi, Catherine Gebhart, Helene Powers, Siobhan Case, Mia T. Chandler, Margaret H. Chang, Ezra Cohen, Megan Day-Lewis, Martha P. Fishman, Olha Halyabar, Jonathan S. Hausmann, Melissa M. Hazen, Pui Y. Lee, Mindy S. Lo, Esra Meidan, Jordan E. Roberts, Mary Beth F. Son, Robert P. Sundel, Fatma Dedeoglu, Peter A. Nigrovic, Alicia Casey, Joyce Chang, Lauren A. Henderson
Summary: This study retrospectively evaluated the incidence and risk factors for eosinophilia and lung disease in systemic JIA patients exposed to IL-1/IL-6 inhibitors. The results showed that eosinophilia is common in JIA patients using these inhibitors and may be associated with severe disease and lung manifestations in systemic JIA.
ARTHRITIS CARE & RESEARCH
(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
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
Piero Ruscitti, Valentina Natoli, Alessandro Consolaro, Roberta Caorsi, Silvia Rosina, Gabriella Giancane, Roberta Naddei, Ilenia Di Cola, Claudia Di Muzio, Onorina Berardicurti, Daniela Iacono, Ilenia Pantano, Gelsomina Rozza, Silvia Rossi, Ludovico De Stefano, Silvia Balduzzi, Antonio Vitale, Francesco Caso, Luisa Costa, Marcella Prete, Luca Navarini, Annamaria Iagnocco, Fabiola Atzeni, Giuliana Guggino, Federico Perosa, Luca Cantarini, Bruno Frediani, Carlomaurizio Montecucco, Francesco Ciccia, Paola Cipriani, Marco Gattorno, Roberto Giacomelli, Angelo Ravelli
Summary: This study compares the clinical features and treatments of systemic JIA and adult-onset Still's disease. The results reveal remarkable disparities in the prevalence of clinical manifestations between the two illnesses, which may partly depend on their classification by different criteria.
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
Rheumatology
Johanna Roemer, Ariane Klein, Gerd Horneff
Summary: This study aimed to investigate the prevalence of depressive symptoms and depression in children with Juvenile idiopathic arthritis (JIA) and identify associated risk factors. The results showed a high prevalence of depressive symptoms and depression in JIA patients, which were significantly associated with disease activity, treatment response, and other factors. Therefore, early recognition and treatment of psychological distress are essential for preventing deterioration in quality of life and long-term prognosis in JIA patients.
RHEUMATOLOGY INTERNATIONAL
(2023)
Review
Pediatrics
Emil Carlsson, Michael W. Beresford, Athimalaipet Ramanan, Andrew D. Dick, Christian M. Hedrich
Summary: Early treatment and frequent screening are crucial for controlling JIA-associated uveitis, which poses a significant risk for serious complications. The identification of specific risk factors for JIA-associated uveitis is a key focus of research as our understanding of pathogenic drivers is still incomplete. Genomic, transcriptomic, and proteomic factors have been identified as potential risk factors, and therapeutic strategies are being discussed.
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
Sindhu R. Johnson, Dafna D. Gladman, Hermine Brunner, David Isenberg, Ann E. Clarke, Megan R. W. Barber, Laurent Arnaud, Paul R. Fortin, Marta Mosca, Alexandre E. Voskuyl, Susan Manzi, Cynthia Aranow, Anca Askanase, Graciela S. Alarcon, Sang-Cheol Bae, Nathalie Costedoat-Chalumeau, Jessica A. English, Guillermo J. Pons-Estel, Bernardo A. Pons-Estel, Rebecca Gilman, Ellen M. Ginzler, John G. Hanly, Soren Jacobsen, Kenneth Kalunian, Diane L. Kamen, Chynace Lambalgen, Alexandra Legge, S. Sam Lim, Anselm Mak, Eric F. Morand, Christine A. Peschken, Michelle Petri, Anisur Rahman, Rosalind Ramsey-Goldman, John A. Reynolds, Juanita Romero-Diaz, Guillermo Ruiz-Irastorza, Jorge Sanchez-Guerrero, Elisabet Svenungsson, Zahi Touma, Murray Urowitz, Evelyne Vinet, Ronald F. van Vollenhoven, Heather Waldhauser, Daniel J. Wallace, Asad Zoma, Ian N. Bruce
Summary: The SLICC, ACR, and Lupus Foundation of America are developing a revised SLE damage index (SDI) due to shifts in the concept of damage in SLE. A qualitative study was conducted to evaluate contemporary constructs in SLE damage and develop a conceptual framework for the revised SDI.
ARTHRITIS CARE & RESEARCH
(2023)
Article
Rheumatology
Hermine Brunner, Ivan Foeldvari, Ekaterina Alexeeva, Nuray Aktay Ayaz, Inmaculada Calvo Penades, Ozgur Kasapcopur, Vyacheslav G. Chasnyk, Markus Hufnagel, Zbigniew Zuber, Grant Schulert, Seza Ozen, Adelina Rakhimyanova, Athimalaipet Ramanan, Christiaan Scott, Betul Sozeri, Elena Zholobova, Ruvie Martin, Xuan Zhu, Sarah Whelan, Luminita Pricop, Alberto Martini, Daniel Lovell, Nicolino Ruperto
Summary: This study demonstrates that secukinumab has a significant effect in prolonging the time to disease flare in children with ERA and JPsA, with a consistent safety profile to the adult indications.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
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)
Article
Rheumatology
Kristine A. Kuhn, Andrew Stahly, Maximilian F. Konig, Kristine Carandang, Connie Herndon, Catherine Backman, Leigh F. Callahan, Grant Schulert, Liana Fraenkel, Alexis Ogdie
Summary: This study assessed the impact of the COVID-19 pandemic on trainees and early career investigators and proposed ways for the ACR and RRF to address their needs. The survey revealed that 75% of respondents pursuing academic research careers had to put their primary projects on hold during the pandemic. The suggested interventions included increased funding, funding process reform, and expanding mentoring and networking resources.
ARTHRITIS CARE & RESEARCH
(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
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
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)
Article
Rheumatology
Fabrizio De Benedetti, Alexei A. Grom, Paul A. Brogan, Claudia Bracaglia, Manuela Pardeo, Giulia Marucci, Despina Eleftheriou, Charalampia Papadopoulou, Grant S. Schulert, Pierre Quartier, Jordi Anton, Christian Laveille, Rikke Frederiksen, Veronica Asnaghi, Maria Ballabio, Philippe Jacqmin, Cristina de Min
Summary: The objective of this study was to confirm the adequacy of an emapalumab dosing regimen in relation to interferon-γ (IFN-γ) activity and assess its efficacy and safety in treating MAS secondary to sJIA or AOSD. The results showed that emapalumab was efficacious in inducing remission of MAS and viral infections were observed.
ANNALS OF THE RHEUMATIC DISEASES
(2023)