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
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
Alessandra Alongi, Gabriella Giancane, Roberta Naddei, Valentina Natoli, Francesca Ridella, Marco Burrone, Silvia Rosina, Gaelle Chedeville, Ekaterina Alexeeva, Gerd Horneff, Ivan Foeldvari, Giovanni Filocamo, Tamas Constantin, Nicolino Ruperto, Angelo Ravelli, Alessandro Consolaro
Summary: This study aimed to investigate the frequency of a global assessment of disease activity (PhGA) >0 and an active joint count (AJC)=0 in children with juvenile idiopathic arthritis (JIA) and search for determinants of divergence between the two measures. The study found that many pediatric rheumatologists did not mark a score of 0 for patients who they found not to have active joints, and the presence of pain in joints not meeting the definition of active joint used in JIA was the main determinant of this phenomenon.
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)
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
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.
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
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)
Article
Biochemistry & Molecular Biology
Debora Mariarita d'Angelo, Marina Attanasi, Giulia Di Donato, Giuseppe Lapergola, Mariarosaria Flacco, Francesco Chiarelli, Emma Altobelli, Luciana Breda
Summary: The study investigates the potential role of serum calprotectin as a biomarker for disease activity and flare/remission in patients with juvenile idiopathic arthritis (JIA). The results suggest that serum calprotectin has a dual role in predicting future relapse and treatment response, which is important for therapeutic decisions and management during follow-up.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(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
Stephane Mitrovic, Nolan Hassold, Aly Kamissoko, Nicolas Rosine, Alexis Mathian, Guillaume Mercy, Edouard Pertuiset, Gaetane Nocturne, Bruno Fautrel, Isabelle Kone-Paut
Summary: A retrospective national survey revealed features of SpA in patients with SJIA/AOSD, with a prevalence of 6.58% in AOSD patients and 10% in SJIA patients. This suggests a possible overlap between SpA and AOSD that requires further study.
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
Camille Beaufils, Catherine Proulx, Annaliesse Blincoe, Pierre Teira, Henrique Bittencourt, Sonia Cellot, Michel Duval, Marie-Paule Morin, Jean Jacques De Bruycker, Julie Couture, Kathryn Samaan, Helene Decaluwe, Niina Kleiber, Ramy El-Jalbout, Fabien Touzot, Elie Haddad, Julie Barsalou
Summary: This study reports cases of systemic-onset juvenile idiopathic arthritis (sJIA) patients who underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) and reviews published outcomes of allo-HSCT in sJIA. The results indicate that allo-HSCT may be a potential curative option for severe/refractory sJIA.
FRONTIERS IN MEDICINE
(2023)
Article
Rheumatology
C. Bava, F. Mongelli, A. Pistorio, M. Bertamino, G. Bracciolini, S. Dalpra, S. Davi, S. Lanni, V Muratore, S. Pederzoli, S. Rosina, B. Schiappapietra, C. Suffia, G. C. Varnier, S. Verazza, G. Giancane, A. Consolaro, A. Ravelli
Summary: The study aimed to investigate the frequency of arthritis flare and factors affecting occurrence of flare in children with JIA who achieved inactive disease with MTX monotherapy. Results showed that 78.3% of patients experienced arthritis flare after a median of 1.6 years, with most flare episodes not treated with biologic therapy. The risk of flare was independent of withdrawal strategy, and majority of patients who had MTX withdrawn experienced flare.
CLINICAL AND EXPERIMENTAL RHEUMATOLOGY
(2021)
Review
Pediatrics
Ann Marie Szymanski, Hugo Clifford, Tova Ronis
WORLD JOURNAL OF PEDIATRICS
(2020)
Article
Rheumatology
Melissa L. Mannion, Fenglong Xie, Daniel B. Horton, Sarah Ringold, Colleen K. Correll, Anne Dennos, Timothy Beukelman
Summary: Biologic medications have greatly improved disease control and outcomes for patients with juvenile idiopathic arthritis (JIA), but a substantial number of patients may require switching to a second biologic. The time to switch has decreased in recent years, and further studies are needed to evaluate outcomes and optimal timing of biologic switching.
JOURNAL OF RHEUMATOLOGY
(2021)
Article
Rheumatology
Jordan T. Jones, Chelsey Smith, Mara L. Becker, Daniel Lovell
Summary: This study found that patients with Down syndrome-associated arthritis (DA) and juvenile idiopathic arthritis (JIA) had similar therapeutic exposure, but DA patients had higher rates of adverse events and treatment ineffectiveness. At the last visit, DA patients had a greater disease burden compared to JIA patients. More research is needed to determine differences in pathophysiology and optimal therapeutic approaches.
ARTHRITIS CARE & RESEARCH
(2021)
Article
Multidisciplinary Sciences
Ruth J. Napier, Ellen J. Lee, Michael P. Davey, Emily E. Vance, Joao M. Furtado, Paige E. Snow, Kimberly A. Samson, Sydney J. Lashley, Brieanna R. Brown, Reiko Horai, Mary J. Mattapallil, Biying Xu, Michelle C. Callegan, Luke S. Uebelhoer, Christina L. Lancioni, Richard K. Vehe, Bryce A. Binstadt, Justine R. Smith, Rachel R. Caspi, Holly L. Rosenzweig
NATURE COMMUNICATIONS
(2020)
Article
Rheumatology
Dax G. Rumsey, Aimee Lougee, Roland Matsouaka, David H. Collier, Laura E. Schanberg, Jennifer Schenfeld, Natalie J. Shiff, Matthew L. Stoll, Scott Stryker, Pamela F. Weiss, Timothy Beukelman
Summary: This study described characteristics of over 900 children with ERA or juvenile PsA, highlighting high biologic use, especially in those with sacroiliitis. Equal sex representation was found in children with sacroiliitis.
ARTHRITIS CARE & RESEARCH
(2021)
Article
Rheumatology
Ishaan Dave, Brandon Estroff, Talia Gergely, Christina A. Rostad, Lori A. Ponder, Courtney McCracken, Sampath Prahalad
Summary: Children with juvenile idiopathic arthritis are more likely to be born in the winter, specifically in the month of January, compared to the general population. These findings suggest that seasonal variations during gestation and early postnatal periods may play a role in the development of JIA.
JOURNAL OF RHEUMATOLOGY
(2021)
Article
Pediatrics
Nayimisha Balmuri, William Daniel Soulsby, Victoria Cooley, Linda Gerber, Erica Lawson, Susan Goodman, Karen Onel, Bella Mehta
Summary: The study found that higher community poverty levels were associated with a longer time to pediatric rheumatologist presentation after symptom onset in children with polyarticular juvenile idiopathic arthritis.
PEDIATRIC RHEUMATOLOGY
(2021)
Article
Pediatrics
Timothy Beukelman, Aimee Lougee, Roland A. Matsouaka, David Collier, Dax G. Rumsey, Jennifer Schenfeld, Scott Stryker, Marinka Twilt, Yukiko Kimura
Summary: This study characterizes contemporary patterns of ETN use in the CARRA Registry. Treatment was largely in keeping with American College of Rheumatology guidelines.
PEDIATRIC RHEUMATOLOGY
(2021)
Article
Pediatrics
Rajdeep Pooni, Tova Ronis, Tzielan Lee
Summary: The study found that musculoskeletal components of telemedicine exams were rated as the most reliable in pediatric rheumatology, but most providers were unable to gather the necessary information for a complete clinical assessment. Some providers reported increased burnout due to telemedicine, and there were differences in patient engagement and confidentiality compared to in-person visits.
PEDIATRIC RHEUMATOLOGY
(2021)
Article
Rheumatology
Mei Sing Ong, Sarah Ringold, Yukiko Kimura, Laura E. Schanberg, George A. Tomlinson, Marc D. Natter
Summary: The study investigated the effects of early introduction of biologic disease-modifying antirheumatic drugs on untreated polyarticular juvenile idiopathic arthritis. It was found that starting bDMARDs within 3 months of baseline assessment was associated with more rapid achievement of inactive disease. Trajectory analysis of disease course proved to be a useful method for determining treatment efficacy in this context.
ARTHRITIS & RHEUMATOLOGY
(2021)
Article
Rheumatology
Yukiko Kimura, Laura E. Schanberg, George A. Tomlinson, Mary Ellen Riordan, Anne C. Dennos, Vincent Del Gaizo, Katherine L. Murphy, Pamela F. Weiss, Marc D. Natter, Brian M. Feldman, Sarah Ringold
Summary: The optimal timing to initiate biologics in polyarticular juvenile idiopathic arthritis (JIA) remains uncertain. A study compared three different treatment strategies for untreated polyarticular JIA, finding that early combination therapy may be more effective in achieving clinically inactive disease.
ARTHRITIS & RHEUMATOLOGY
(2021)
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
Pediatrics
Meghan E. Ryan, Andrew Warmin, Bryce A. Binstadt, Colleen K. Correll, Emily Hause, Patricia Hobday, Alison Lerman, Shawn A. Mahmud, Mona M. Riskalla, Zachary Shaheen, Richard K. Vehe, Danielle R. Bullock
Summary: The study aimed to improve documentation of critical data elements by implementing quality improvement methods, and found that virtual visits had lower data capture rates compared to in-person visits.
PEDIATRIC RHEUMATOLOGY
(2022)
Article
Pediatrics
Shawn A. Mahmud, Danielle R. Bullock, Colleen K. Correll, Patricia M. Hobday, Mona M. Riskalla, Richard K. Vehe, Bryce A. Binstadt
Summary: Non-criteria antiphospholipid antibodies (NC-aPL) are associated with pediatric patients with antiphospholipid syndrome (APS), and doctors should consider the existence of NC-aPL when there is a high suspicion and routine antiphospholipid antibody tests are negative. Currently, there are no guidelines for NC-aPL in children or adults, so more data needs to be collected.
PEDIATRIC RHEUMATOLOGY
(2022)
Article
Health Care Sciences & Services
Bayardo Garay, Denise Erlanson, Bryce A. Binstadt, Colleen K. Correll, Nora Fitzsimmons, Patricia M. Hobday, Allison Hudson, Shawn Mahmud, Mona M. Riskalla, Sara Kramer, Sheng Xiong, Richard K. Vehe, Danielle R. Bullock
Summary: By implementing a checkout sheet and staggered start times, this study effectively reduced patient wait time in a paediatric rheumatology clinic, leading to a decrease in variation and mean wait time.