Article
Immunology
Anna E. Patrick, Kayla Shoaff, Tashawna Esmond, David M. Patrick, David K. Flaherty, T. Brent Graham, Philip S. Crooke, Susan Thompson, Thomas M. Aune
Summary: In juvenile idiopathic arthritis (JIA), polarized T1 cells incorrectly produce multiple cell subtypes, including Th1, Th17, and Th1.17. The generation and activation of these cells are important for the pathogenesis of the disease and may serve as potential drug targets and diagnostic markers.
FRONTIERS IN IMMUNOLOGY
(2022)
Article
Immunology
Sun-Hee Hwang, Jin Seok Woo, Jeonghyeon Moon, SeungCheon Yang, Jin-Sil Park, JaeSeon Lee, JeongWon Choi, Kun Hee Lee, Seung-Ki Kwok, Sung-Hwan Park, Mi-La Cho
Summary: The study found that the increase of IL-17 is associated with aging and metabolic abnormalities, and may play a key role in the onset and progression of SS.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Medicine, General & Internal
Marino Paroli, Luca Spadea, Rosalba Caccavale, Leopoldo Spadea, Maria Pia Paroli, Nicola Nante
Summary: Interleukin-17A plays a central role in the pathogenesis of juvenile idiopathic arthritis (JIA) and blocking its production can effectively treat JIA. The use of the human anti-IL17A monoclonal antibody secukinumab has been approved in JIA treatment and has shown improvement in the course of the disease.
MEDICINA-LITHUANIA
(2022)
Article
Biology
Dong Hyun Kim, Hee Young Kim, Sunjung Cho, Su-Jin Yoo, Won-Ju Kim, Hye Ran Yeon, Kyungho Choi, Je-Min Choi, Seong Wook Kang, Won-Woo Lee
Summary: The study demonstrates the crucial role of IL-1RII receptor in regulating IL-17 responses, affecting the functionality of CD4(+) T cells by limiting IL-1 beta responsiveness.
Article
Immunology
Li-Chin Liao, Yi-Hsiu Fu, Chieh-Mao Chuang, Pei-Lun Liao, James Cheng-Chung Wei, Yun-Ching Fu
Summary: Recent cohort study in Taiwan showed that children with Kawasaki disease have a significantly higher risk of juvenile idiopathic arthritis compared to those without KD. Further investigations are needed to identify the pathological mechanisms linking these two conditions.
FRONTIERS IN IMMUNOLOGY
(2022)
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
Mao Mizuta, Masaki Shimizu, Natsumi Inoue, Yasuhiro Ikawa, Yasuo Nakagishi, Ryuhei Yasuoka, Naomi Iwata, Akihiro Yachie
Summary: The study showed that serum IL-18 levels can be used to differentiate between s-JIA and other diseases, and monitoring IL-18 levels can help predict the disease course and assess remission in s-JIA.
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
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
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
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)
Review
Biochemistry & Molecular Biology
Iwona Sudol-Szopinska, Nele Herregods, Andrea S. Doria, Mihra S. Taljanovic, Piotr Gietka, Nikolay Tzaribachev, Andrea Sabine Klauser
Summary: Over the past decade, imaging techniques for inflammatory arthritis in juvenile arthropathies have significantly improved, allowing for more accurate assessment and diagnosis. Ultrasound and magnetic resonance imaging have seen advancements in resolution, sensitivity, and diagnostic possibilities.
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)
Article
Medicine, General & Internal
Pierre Quartier
Summary: This review explores the important questions regarding disease heterogeneity and treatment approaches for SJIA/Pediatric Still's disease. The study identifies specific treatments such as IL-1 inhibition and innovative approaches like JAK inhibitors.
JOURNAL OF CLINICAL MEDICINE
(2022)
Review
Nutrition & Dietetics
Renata Puppin Zandonadi
Summary: Nutrition plays a crucial role in the treatment, health, and life of patients with juvenile idiopathic arthritis (JIA). Suboptimal nutrition can adversely affect the long-term outcome of JIA patients, while correct nutritional status helps with recovery and inflammation reduction.
Letter
Rheumatology
Ilaria Maccora, Gabriele Simonini
Article
Rheumatology
Maria Vincenza Mastrolia, Alessandra Bettiol, Edoardo Marrani, Ilaria Maccora, Emilia Taddei, Ilaria Pagnini, Maria Canfora, Giacomo Emmi, Elena Silvestri, Domenico Prisco, Gabriele Simonini
Summary: This study observed significant differences between juvenile-onset and adult-onset Behcet's syndrome in terms of demographic, clinical features, and treatment approach. Juvenile-onset patients were more likely to have a familial predisposition while adult-onset patients had a higher use of DMARDs for treatment.
Article
Rheumatology
Ivan Foeldvari, Ilaria Maccora, Harry Petrushkin, Najiha Rahman, Jordi Anton, Joke de Boer, Joan Calzada-Hernandez, Elisa Carreras, Jesus Diaz, Clive Edelsten, Sheila T. Angeles-Han, Arnd Heiligenhaus, Elisabetta Miserocchi, Susan Nielsen, Rotraud K. Saurenmann, Nicole Stuebiger, Karoline Baquet-Walscheid, Daniel Furst, Gabriele Simonini
Summary: The Multinational Interdisciplinary Working Group for Uveitis in Childhood recognized the need to update current guidelines for managing children with juvenile idiopathic arthritis-associated uveitis (JIAU) and idiopathic chronic anterior uveitis (CAU). After analyzing literature and conducting a consensus meeting, 14 recommendations for JIAU and CAU treatment were accepted with over 90% agreement. This evidence-based consensus process aims to provide an updated guideline to support clinicians in caring for children and young people with CAU.
ARTHRITIS CARE & RESEARCH
(2023)
Review
Immunology
Ilaria Maccora, Athimalaipet Ramanan, Daniel Wiseman, Edoardo Marrani, Maria Mastrolia, Gabriele Simonini
Summary: SIFD syndrome is a novel rare autoinflammatory multisystem disorder characterized by anemia, fever, immunodeficiency, and neurodevelopmental delay. Treatment with anti-TNF alpha and hematopoietic cell stem transplantation can potentially modify the clinical course of this syndrome.
JOURNAL OF CLINICAL IMMUNOLOGY
(2023)
Review
Immunology
Silvia Grazzini, Chiara Rizzo, Edoardo Conticini, Roberto D'Alessandro, Lidia La Barbera, Miriana D'Alessandro, Paolo Falsetti, Elena Bargagli, Giuliana Guggino, Luca Cantarini, Bruno Frediani
Summary: Idiopathic inflammatory myopathies (IIM) are a group of diseases that affect striate muscle, lung, joints, skin, and gastrointestinal tract. The current treatment mainly involves glucocorticoids and immunosuppressants. However, for refractory cases, the use of biologic disease modifying drugs (bDMARDs) has shown promising results.
AUTOIMMUNITY REVIEWS
(2023)
Review
Immunology
Antonio Vitale, Valeria Caggiano, Virginia Berlengiero, Maria Orsetta Perfetti, Jurgen Sota, Gian Marco Tosi, Bruno Frediani, Luca Cantarini, Claudia Fabiani
Summary: Behcet's disease (BD) associated uveitis occurs in a significant percentage of patients and can lead to severe visual impairment. Treatment with tumor necrosis factor (TNF) inhibitors and other biologic agents shows promise, but more evidence is needed. The Auto-Inflammatory Disease Alliance (AIDA) Network is working on an international registry to address the unmet needs in the clinical practice.
EXPERT REVIEW OF CLINICAL IMMUNOLOGY
(2023)
Review
Medicine, General & Internal
Antonio Vitale, Valeria Caggiano, Antonio Bimonte, Federico Caroni, Gian Marco Tosi, Alessandra Fabbiani, Alessandra Renieri, Monica Bocchia, Bruno Frediani, Claudia Fabiani, Luca Cantarini
Summary: VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) syndrome is an acquired monogenic autoinflammatory disease caused by somatic mutations of the UBA1 gene, leading to systemic inflammation. It has a heterogeneous clinical picture resembling various other systemic rheumatic diseases coexisting with hematological disorders, especially myelodysplastic syndrome. This syndrome represents a fascinating clinical condition of adult-onset monogenic autoinflammatory diseases caused by genetic mosaicism originating from bone marrow disorders. It should be considered in patients with unexplained systemic inflammatory conditions, particularly when accompanied by myelodysplastic syndrome or other hematological disorders.
INTERNAL AND EMERGENCY MEDICINE
(2023)
Article
Medicine, General & Internal
Vitale Antonio, Virginia Berlengiero, Valeria Caggiano, Sara Barneschi, Mariam Mourabi, Jurgen Sota, Stefano Gentileschi, Maria Cristina Maggio, Carla Gaggiano, Maria Tarsia, Gian Marco Tosi, Giuseppe Lopalco, Claudia Fabiani, Bruno Frediani, Luca Cantarini
Summary: The aim of the study was to evaluate the frequency and features of positive pathergy test (PPT) in Italy and its role in the diagnosis of Behcet's disease (BD). The results showed that the occurrence of skin erythema at PPT could be sufficient to unveil the hyper-reactivity of the innate immune system in BD patients from Western Europe. The sensitivity and specificity of erythema at self-saliva prick test were also determined.
INTERNAL AND EMERGENCY MEDICINE
(2023)
Article
Rheumatology
Ilaria Maccora, Teodoro Oliverio, Ilaria Pagnini, Edoardo Marrani, Maria Vincenza Mastrolia, Gabriele Simonini
ANNALS OF THE RHEUMATIC DISEASES
(2023)
Article
Immunology
Anna Vanni, Alessio Mazzoni, Roberto Semeraro, Manuela Capone, Patrick Maschmeyer, Giulia Lamacchia, Lorenzo Salvati, Alberto Carnasciali, Parham Farahvachi, Teresa Giani, Gabriele Simonini, Giovanni Filocamo, Micol Romano, Francesco Liotta, Mir-Farzin Mashreghi, Lorenzo Cosmi, Rolando Cimaz, Alberto Magi, Laura Maggi, Francesco Annunziato
Summary: Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic condition in childhood, with T cells playing a key role in its pathogenesis. The expression of PD-1 on CD4+ and CD8+ T cells in JIA indicates their active contribution in sustaining chronic inflammation. Targeting PD-1 could be an effective therapeutic strategy for JIA.
EUROPEAN JOURNAL OF IMMUNOLOGY
(2023)
Review
Immunology
Ilaria Maccora, Edoardo Marrani, Ilaria Pagnini, Maria Vincenza Mastrolia, Cinzia de Libero, Roberto Caputo, Gabriele Simonini
Summary: Childhood uveitis is a serious condition that can lead to blindness if not properly recognized and treated. This review discusses the main causes, diagnostic approach, risk factors, and difficulties in eye examination for childhood uveitis. The treatment options for childhood uveitis are also discussed in terms of choice, timing, and withdrawal.
EXPERT REVIEW OF CLINICAL IMMUNOLOGY
(2023)
Article
Biochemistry & Molecular Biology
Paola Triggianese, Mauro Fatica, Francesco Caso, Luisa Costa, Arianna D'Antonio, Marco Tasso, Elisabetta Greco, Paola Conigliaro, Alberto Bergamini, Claudia Fabiani, Luca Cantarini, Maria Sole Chimenti
Summary: Non-infectious uveitis (NIU) is often the initial manifestation of systemic rheumatic diseases, and rheumatologists play a crucial role in its diagnosis and treatment. This study evaluated 130 patients with NIU and found that anterior uveitis (AU) was the most common type, predominantly associated with spondyloarthritis (SpA). HLA-B27(+) patients had a higher prevalence of AU with an acute course, while HLA-B51(+) patients had mostly posterior uveitis (PU) with a recurrent course. Rheumatologic referral significantly influenced the treatment strategies for NIU.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Biochemistry & Molecular Biology
Vincenzo Venerito, Pasquale Stefanizzi, Luca Cantarini, Marlea Lavista, Maria Grazia Galeone, Antonio Di Lorenzo, Florenzo Iannone, Silvio Tafuri, Giuseppe Lopalco
Summary: RA patients treated with JAK inhibitors or anti-cellular bDMARDS have an increased risk of herpes zoster (HZ). This prospective study assessed the immunogenicity and safety of the Adjuvanted Recombinant Zoster Vaccine (RZV) in RA patients on JAK inhibitors or anti-cellular bDMARDS. The results showed that RZV vaccination induced a similar immune response in RA patients compared to healthy controls, regardless of the treatment.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Pediatrics
Valerio Maniscalco, Rachele Niccolai, Edoardo Marrani, Ilaria Maccora, Federico Bertini, Ilaria Pagnini, Gabriele Simonini, Donatella Lasagni, Sandra Trapani, Maria Vincenza Mastrolia
Summary: Multisystem Inflammatory Syndrome in Children (MIS-C) is a systemic hyperinflammatory disorder associated with a hypercoagulable state and higher risk of thrombotic events (TEs). This case report discusses a severe case of a 9-year-old MIS-C patient who developed a massive pulmonary embolism, successfully treated with heparin. A literature review of 60 MIS-C cases reveals that 91.7% of patients had at least one risk factor for thrombosis, with pediatric intensive care unit hospitalization, central venous catheter, and age >12 years being the most frequently observed risk factors. Despite antithrombotic prophylaxis, 40% of MIS-C patients developed TEs, leading to permanent disability or death in some cases.
Article
Medicine, General & Internal
Edoardo Marrani, Giovanni Fulvio, Camilla Virgili, Rossana Izzetti, Valentina Dini, Teresa Oranges, Chiara Baldini, Gabriele Simonini
Summary: This study evaluated the potential role of ultrahigh-frequency ultrasound (UHFUS) in diagnosing pediatric Sjögren's disease. Intraoral UHFUS scan of the lip mucosa was performed on 12 pediatric patients, and it was found that all patients had salivary gland alterations. Preliminary results suggest that UHFUS could be a feasible technique for diagnosing pediatric Sjögren's disease.