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
Immunology
Shuya Kaneko, Masaki Shimizu, Futaba Miyaoka, Asami Shimbo, Hitoshi Irabu, Mao Mizuta, Yasuo Nakagishi, Naomi Iwata, Junya Fujimura, Masaaki Mori, Tomohiro Morio
Summary: This study validates the correlation between laboratory markers reflecting disease activity of macrophage activation syndrome (MAS) and serum cytokine levels, and identifies valuable laboratory markers that change over time for a prompt MAS diagnosis.
CLINICAL IMMUNOLOGY
(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
Rosemary G. Peterson, Rui Xiao, Karen E. James, Hannah Katcoff, Brian T. Fisher, Pamela F. Weiss
Summary: The study found significant variation in the use of biologics and glucocorticoids in children hospitalized with new-onset systemic JIA, with noticeable differences between hospitals. Despite increasing evidence supporting the use of biologics for improved outcomes, many children did not receive biologic treatment and a high rate of glucocorticoid exposure persisted.
ARTHRITIS CARE & RESEARCH
(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)
Review
Rheumatology
Eduardo Liquidano-Perez, Gibert Maza-Ramos, Jose Luis Salazar-Bailon, Marco Antonio Yamazaki-Nakashimada, Francisco Rivas-Larrauri
Summary: This study presents a refractory case of soJIA complicated with MAS successfully treated with plasma exchange, resulting in significant improvement.
RHEUMATOLOGY INTERNATIONAL
(2022)
Article
Medicine, General & Internal
Alberto Martini, Daniel J. Lovell, Salvatore Albania, Hermine Brunner, Kimme L. Hyrich, Susan D. Thompson, Nicolino Ruperto
Summary: Juvenile idiopathic arthritis (JIA) is the most common chronic inflammatory rheumatic condition of childhood, classified into seven categories. The pathogenesis of JIA involves autoimmune and autoinflammatory mechanisms, and the availability of new drugs has significantly improved treatment options.
NATURE REVIEWS DISEASE PRIMERS
(2022)
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
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)
Review
Virology
Giulia Franzoni, Miriam Pedrera, Pedro J. Sanchez-Cordon
Summary: African swine fever (ASF) is a viral disease caused by ASF virus (ASFV) that affects domestic pigs and wild suids worldwide. The absence of licensed vaccine or treatments poses a threat to pig production. Understanding the immunopathogenic mechanisms of ASFV infection is crucial in combating the disease. Cytokines, such as IL-1, IL-6, TNF, and chemokines, play a role in immune responses to ASFV. Increased levels of these cytokines are associated with tissue damage and disease severity, while increased levels of IFN-alpha and IL-10 may have negative effects on animal survival.
Review
Biochemistry & Molecular Biology
Chao-Yi Wu, Huang-Yu Yang, Jing-Long Huang, Jenn-Haung Lai
Summary: Monocytes and macrophages play a crucial role in the pathogenesis of juvenile idiopathic arthritis (JIA), with their activation being linked to inflammation regulation and tissue repair mechanisms in the disease process.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Review
Rheumatology
Emely L. Verweyen, Grant S. Schulert
Summary: Systemic JIA (SJIA) is characterized by severe complications, including macrophage activation syndrome (SJIA-MAS) and lung disease (SJIA-LD), which are driven by interferons (IFNs) and involve the JAK-STAT signaling pathway. New therapeutic advances, such as JAK inhibitors and antibodies targeting IFNs, are urgently needed to address the divergent disease pathogenesis observed in SJIA.
Review
Medicine, General & Internal
Charlotte Girard-Guyonvarc'h, Mathilde Harel, Cem Gabay
Summary: This review summarizes the role and importance of IL-18 and IL-18BP in AOSD and sJIA. It is found that the imbalance of IL-18/IL-18BP is associated with the occurrence of these diseases, and treatment with recombinant IL-18BP shows potential therapeutic effects.
JOURNAL OF CLINICAL MEDICINE
(2022)
Review
Rheumatology
Daniel Reiff, Matthew L. Stoll, Randy Q. Cron
Summary: The introduction of disease-modifying anti-rheumatic drug therapies for treating JIA in children and adolescents has revolutionized care and outcomes. TNF inhibitors have shown effectiveness for most JIA subtypes, but there are still some patients who require different therapeutic approaches. Personalized care and redefining JIA classification offer potential for improved treatment outcomes.
LANCET RHEUMATOLOGY
(2021)
Article
Pediatrics
Christopher Towe, Alexei A. Grom, Grant S. Schulert
Summary: Chronic lung disease in children with systemic juvenile idiopathic arthritis (SJIA-LD) is a potentially life-threatening complication. The optimal approaches to diagnosis and management of SJIA-LD are still unclear. This review discusses the current clinical understanding of SJIA-LD, including its potential pathogenesis and treatment options, and highlights the urgent unmet needs in this area.
Letter
Rheumatology
Seza Ozen, Fabrizio De Benedetti
ANNALS OF THE RHEUMATIC DISEASES
(2022)
Article
Pediatrics
Manuela Colucci, Barbara Ruggiero, Alessandra Gianviti, Maria Manuela Rosado, Rita Carsetti, Claudia Bracaglia, Fabrizio De Benedetti, Francesco Emma, Marina Vivarelli
Summary: The presence of IgM on T cells can effectively distinguish between pediatric patients with SLE and FHN, with T cell IgM positivity showing high sensitivity and specificity for the diagnosis of SLE.
PEDIATRIC NEPHROLOGY
(2021)
Article
Allergy
Simona Coppola, Antonella Insalaco, Erika Zara, Martina Di Rocco, Denise Pires Marafon, Francesca Spadaro, Luca Pannone, Luciapia Farina, Luca Pasquini, Simone Martinelli, Fabrizio De Benedetti, Marco Tartaglia
Summary: This study clinically and functionally classified three pathogenic variants of CDC42 at the C-terminus and found that they differently impact protein stability, localization, and function, causing various clinical diseases.
JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY
(2022)
Article
Rheumatology
Marta Bustaffa, Isabelle Kone-Paut, Seza Ozen, Gayane Amaryan, Efimia Papadopoulou-Alataki, Romina Gallizzi, Maria Carrabba, Yonatan Butbul Aviel, Luca Cantarini, Maria Alessio, Jordi Anton, Laura Obici, Faysal Gok, Ezgi Deniz Batu, Estefania Moreno, Paul Brogan, Maria Trachana, Gabriele Simonini, Donato Rigante, Yosef Uziel, Antonella Insalaco, Maria Cristina Maggio, Nicolino Ruperto, Marco Gattorno, L. Rossi Semerano
Summary: The new EPCC and INSAID classification of MEFV variants can identify two distinct groups of FMF patients, which differ in clinical characteristics, therapeutic approach, and response to treatment.
SEMINARS IN ARTHRITIS AND RHEUMATISM
(2022)
Article
Immunology
Marianna Nicoletta Rossi, Silvia Federici, Andrea Uva, Chiara Passarelli, Camilla Celani, Ivan Caiello, Valentina Matteo, Stefano Petrocchi, Eva Piano Mortari, Fabrizio De Benedetti, Giusi Prencipe, Antonella Insalaco
Summary: A20 gene mutations are associated with the development of HA20, an autoimmune disease, and functional analysis confirmed the pathogenicity of the mutations. The study demonstrated the role of A20 not only in regulating the NF-kB pathway, but also the IFN gamma pathway.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Hematology
Arianna De Matteis, Manuela Colucci, Marianna N. Rossi, Ivan Caiello, Pietro Merli, Nicola Tumino, Valentina Bertaina, Manuela Pardeo, Claudia Bracaglia, Franco Locatelli, Fabrizio De Benedetti, Giusi Prencipe
Summary: The activation profile of CD8(+) T cells can distinguish different types of HLH patients and those with early sepsis. The frequency of CD4(dim)CD8(+) T cells is correlated with the severity of MAS.
Article
Immunology
Luciapia Farina, Gaetana Minnone, Stefano Alivernini, Ivan Caiello, Lucy MacDonald, Marzia Soligo, Luigi Manni, Barbara Tolusso, Simona Coppola, Erika Zara, Libenzio Adrian Conti, Angela Aquilani, Silvia Magni-Manzoni, Mariola Kurowska-Stolarska, Elisa Gremese, Fabrizio De Benedetti, Luisa Bracci-Laudiero
Summary: The study shows that the activation of the p75NTR/proNGF axis promotes pro-inflammatory responses in synovial fibroblasts, contributing to chronic synovial inflammation. Inhibiting the p75NTR receptor may be a novel therapeutic approach for chronic arthritis.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Medicine, General & Internal
Antonio Vitale, Francesca Della Casa, Gaafar Ragab, Ibrahim A. Almaghlouth, Giuseppe Lopalco, Rosa Maria Pereira, Silvana Guerriero, Marcello Govoni, Petros P. Sfikakis, Roberto Giacomelli, Francesco Ciccia, Sara Monti, Piero Ruscitti, Matteo Piga, Claudia Lomater, Abdurrahman Tufan, Daniela Opris-Belinski, Giacomo Emmi, Jose Hernandez-Rodriguez, Burak Karkas, Gian Domenico Sebastiani, Elena Bartoloni, Nurullah Akkoc, Marco Cattalini, Giovanni Conti, Gulen Hatemi, Armin Maier, Paola Parronchi, Emanuela Del Giudice, Sukran Erten, Antonella Insalaco, Francesca Li Gobbi, Maria Cristina Maggio, Farhad Shahram, Valeria Caggiano, Mohamed Tharwat Hegazy, Kazi Nur Asfina, Maria Morrone, Leandro L. Prado, Rosanna Dammacco, Francesca Ruffilli, Aikaterini Arida, Luca Navarini, Ilenia Pantano, Lorenzo Cavagna, Alessandro Conforti, Alberto Cauli, Elena Maria Marucco, Hamit Kucuk, Ruxandra Ionescu, Irene Mattioli, Gerard Espinosa, Olga Araujo, Claudia Canofari, Jurgen Sota, Ahmed Hatem Laymouna, Asma A. Bedaiwi, Sergio Colella, Henrique Ayres M. Giardini, Valeria Albano, Andrea Lo Monaco, George E. Fragoulis, Riza Can Kardas, Virginia Berlengiero, Mohamed A. Hussein, Francesca Ricci, Francesco La Torre, Donato Rigante, Ewa Wiesik-Szewczyk, Micol Frassi, Stefano Gentileschi, Gian Marco Tosi, Marilia Ambiel Dagostin, Ayman Abdel-Monem Ahmed Mahmoud, Maria Tarsia, Giovanni Alessio, Rolando Cimaz, Teresa Giani, Carla Gaggiano, Florenzo Iannone, Paola Cipriani, Mariam Mourabi, Veronica Spedicato, Sara Barneschi, Emma Aragona, Alberto Balistreri, Bruno Frediani, Claudia Fabiani, Luca Cantarini
Summary: The purpose of this paper is to discuss the design, development, and implementation of the AutoInflammatory Disease Alliance (AIDA) International Registry for Behcet's disease (BD) patients. The registry aims to collect standardized real-life data from pediatric and adult patients, using the Research Electronic Data Capture (REDCap) tool. As of February 7th, 2022, 110 centers from 23 countries have participated, with 54 centers obtaining approval from their local Ethics Committees. The registry collects a wide range of data, including demographics, clinical manifestations, therapies, and healthcare access.
INTERNAL AND EMERGENCY MEDICINE
(2022)
Article
Medicine, General & Internal
Antonio Vitale, Valeria Caggiano, Francesca Della Casa, Jose Hernandez-Rodriguez, Micol Frassi, Sara Monti, Abdurrahman Tufan, Salvatore Telesca, Edoardo Conticini, Gaafar Ragab, Giuseppe Lopalco, Ibrahim Almaghlouth, Rosa Maria R. Pereira, Derya Yildirim, Marco Cattalini, Achille Marino, Teresa Giani, Francesco La Torre, Piero Ruscitti, Emma Aragona, Ewa Wiesik-Szewczyk, Emanuela Del Giudice, Petros P. Sfikakis, Marcello Govoni, Giacomo Emmi, Maria Cristina Maggio, Roberto Giacomelli, Francesco Ciccia, Giovanni Conti, Djouher Ait-Idir, Claudia Lomater, Vito Sabato, Matteo Piga, Ali Sahin, Daniela Opris-Belinski, Ruxandra Ionescu, Elena Bartoloni, Franco Franceschini, Paola Parronchi, Amato de Paulis, Gerard Espinosa, Armin Maier, Gian Domenico Sebastiani, Antonella Insalaco, Farhad Shahram, Paolo Sfriso, Francesca Minoia, Maria Alessio, Joanna Makowska, Gulen Hatemi, Nurullah Akkoc, Francesca Li Gobbi, Antonio Gidaro, Alma Nunzia Olivieri, Sulaiman M. Al-Mayouf, Sukran Erten, Stefano Gentileschi, Ibrahim Vasi, Maria Tarsia, Ayman Abdel-Monem Ahmed Mahmoud, Bruno Frediani, Musa Fares Alzahrani, Ahmed Hatem Laymouna, Francesca Ricci, Fabio Cardinale, Karina Jahnz-Rozyk, Gian Marco Tosi, Francesca Crisafulli, Alberto Balistreri, Marilia A. Dagostin, Mahmoud Ghanema, Carla Gaggiano, Jurgen Sota, Ilenia Di Cola, Claudia Fabiani, Henrique A. Mayrink Giardini, Alessandra Renieri, Alessandra Fabbiani, Anna Carrer, Monica Bocchia, Federico Caroni, Donato Rigante, Luca Cantarini
Summary: This paper presents an international registry for VEXAS syndrome, designed to collect real-life data and provide real-world evidence for daily clinical practice, potentially enhancing international collaboration and data sharing.
FRONTIERS IN MEDICINE
(2022)
Article
Cardiac & Cardiovascular Systems
Antonio Brucato, Alistair Wheeler, Sushil Allen Luis, Antonio Abbate, Paul C. Cremer, Liangxing Zou, Antonella Insalaco, Martin Lewinter, Basil S. Lewis, David Lin, Stephen Nicholls, Massimo Pancrazi, Allan L. Klein, Massimo Imazio, John F. Paolini
Summary: This study suggests that rapid discontinuation of oral therapies for recurrent pericarditis while transitioning to rilonacept monohterapy is feasible without triggering recurrence.
Article
Medicine, General & Internal
Carla Gaggiano, Antonio Vitale, Abdurrahman Tufan, Gaafar Ragab, Emma Aragona, Ewa Wiesik-Szewczyk, Djouher Ait-Idir, Giovanni Conti, Ludovica Iezzi, Maria Cristina Maggio, Marco Cattalini, Francesco La Torre, Giuseppe Lopalco, Elena Verrecchia, Amato de Paulis, Ali Sahin, Antonella Insalaco, Petros P. Sfikakis, Achille Marino, Micol Frassi, Benson Ogunjimi, Daniela Opris-Belinski, Paola Parronchi, Giacomo Emmi, Farhad Shahram, Francesco Ciccia, Matteo Piga, Jose Hernandez-Rodriguez, Rosa Maria R. Pereira, Maria Alessio, Roberta Naddei, Alma Nunzia Olivieri, Emanuela Del Giudice, Paolo Sfriso, Piero Ruscitti, Francesca Li Gobbi, Hamit Kucuk, Jurgen Sota, Mohamed A. Hussein, Giuseppe Malizia, Karina Jahnz-Rozyk, Rawda Sari-Hamidou, Mery Romeo, Francesca Ricci, Fabio Cardinale, Florenzo Iannone, Francesca Della Casa, Marco Francesco Natale, Katerina Laskari, Teresa Giani, Franco Franceschini, Vito Sabato, Derya Yildirim, Valeria Caggiano, Mohamed Tharwat Hegazy, Rosalba Di Marzo, Aleksandra Kucharczyk, Ghalia Khellaf, Maria Tarsia, Ibrahim A. Almaghlouth, Ahmed Hatem Laymouna, Violetta Mastrorilli, Laura Dotta, Luca Benacquista, Salvatore Grosso, Francesca Crisafulli, Veronica Parretti, Heitor F. Giordano, Ayman Abdel-Monem Ahmed Mahmoud, Rossana Nuzzolese, Marta De Musso, Cecilia Beatrice Chighizola, Stefano Gentileschi, Mirella Morrone, Ilenia Di Cola, Veronica Spedicato, Henrique A. Mayrink Giardini, Ibrahim Vasi, Alessandra Renieri, Alessandra Fabbiani, Maria Antonietta Mencarelli, Bruno Frediani, Alberto Balistreri, Gian Marco Tosi, Claudia Fabiani, Merav Lidar, Donato Rigante, Luca Cantarini
Summary: This article describes an international, electronic-based registry platform for mAIDs patients, which aims to collect and share data for research and improve patient management.
FRONTIERS IN MEDICINE
(2022)
Article
Rheumatology
Brian Skaug, Xinjian Guo, Yuanteng Jeff Li, Julio Charles, Kay T. Pham, Jacob Couturier, Dorothy E. Lewis, Claudia Bracaglia, Ivan Caiello, Maureen D. Mayes, Shervin Assassi
Summary: The R206C polymorphism in the coding region of DNASE1L3 gene impairs its ability to digest extracellular DNA, leading to reduced digestion of circulating DNA in plasma from SSc patients.
Article
Rheumatology
Marco Gattorno, Laura Obici, Inmaculada Calvo Penades, Tilmann Kallinich, Susanne Benseler, Elise Dekker, Jeremy Levy, Fabrizio De Benedetti, Helen Lachmann
Summary: This study assessed the efficacy, safety, and tolerability of canakinumab in TRAPS patients during a 72-week long-term, open-label extension. The results showed that long-term canakinumab treatment effectively controlled disease activity and reduced flare incidence in TRAPS patients. No new safety concerns were identified.
ARTHRITIS & 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)