Fabrizio De Benedetti
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View article: Interleukin‐18 levels are associated with disease course in patients with Still's disease treated with <scp>IL</scp> ‐1 inhibitors
Interleukin‐18 levels are associated with disease course in patients with Still's disease treated with <span>IL</span> ‐1 inhibitors Open
Objective To evaluate the prognostic utility of circulating Interleukin‐18 (IL‐18) levels in predicting disease activity, macrophage activation syndrome (MAS), and disease course in Still's disease (SD) patients receiving first‐line IL‐1 i…
View article: Sarilumab in Polyarticular‐Course Juvenile Idiopathic Arthritis: Dose‐Finding and One‐Year Analysis of a Phase 2b, Open‐Label, Multicenter Study
Sarilumab in Polyarticular‐Course Juvenile Idiopathic Arthritis: Dose‐Finding and One‐Year Analysis of a Phase 2b, Open‐Label, Multicenter Study Open
Objective This study assessed sarilumab in treating patients with polyarticular‐course juvenile idiopathic arthritis (pcJIA). Methods This phase 2b, open‐label study (NCT02776735) consisted of three sequential parts (each with a core‐treat…
View article: Genome-wide DNA methylation analysis identifies kidney epigenetic dysregulation in a cystinosis mouse model
Genome-wide DNA methylation analysis identifies kidney epigenetic dysregulation in a cystinosis mouse model Open
Introduction Nephropathic cystinosis is a rare genetic disorder characterized by cystine accumulation in lysosomes that causes early renal dysfunction and progressive chronic kidney disease. Although several metabolic pathways, including o…
View article: Innate Lymphoid Cell Phenotypic and Functional Alterations in Patients With Systemic Juvenile Idiopathic Arthritis
Innate Lymphoid Cell Phenotypic and Functional Alterations in Patients With Systemic Juvenile Idiopathic Arthritis Open
Objective Systemic juvenile idiopathic arthritis (sJIA) is a chronic childhood disease classically attributed to innate immune cell dysregulation. This study aimed to elucidate the role of innate lymphoid cells (ILCs), including natural ki…
View article: Investigating amygdala habituation in major depressive disorder: an fMRI study in UK Biobank
Investigating amygdala habituation in major depressive disorder: an fMRI study in UK Biobank Open
Introduction Major depressive disorder (MDD) is a severe psychiatric condition with a high risk of suicide. Research on MDD and suicidality has identified structural and functional abnormalities in the cortico-limbic network as candidate b…
View article: Significant reduction of cognitive distortions after total sleep deprivation in patients with major depression: a preliminary study
Significant reduction of cognitive distortions after total sleep deprivation in patients with major depression: a preliminary study Open
Introduction Cognitive distortion is a central feature of depression, encompassing dysfunctional personality styles and attitudes, and negative thinking. One of the cognitive schemas characteristics of depressed individuals is the tendency…
View article: Combining structural MRI with Polygenic Risk Scores to disentangle unipolar and bipolar depression: a multimodal machine learning study
Combining structural MRI with Polygenic Risk Scores to disentangle unipolar and bipolar depression: a multimodal machine learning study Open
Introduction The differential diagnosis between Major Depressive Disorder (MDD) and Bipolar Disorder (BD) heavily relies on clinical observation. However, the two disorders often show similar symptomatologic profiles, leading to high misdi…
View article: Different patterns of longitudinal changes in antinuclear antibodies titers in children with systemic lupus erythematosus and Sjögren's syndrome
Different patterns of longitudinal changes in antinuclear antibodies titers in children with systemic lupus erythematosus and Sjögren's syndrome Open
Objective to investigate the trend of autoantibody titers during a 2-year follow-up in pediatric systemic lupus erythematosus (pSLE) and pediatric Sjögren's syndrome (pSS). Methods Autoantibodies testing was performed every 3-4 months duri…
View article: Reliability of a generative artificial intelligence tool for pediatric familial Mediterranean fever: insights from a multicentre expert survey
Reliability of a generative artificial intelligence tool for pediatric familial Mediterranean fever: insights from a multicentre expert survey Open
AI has promising implications in pediatric rheumatology, including early diagnosis and management optimization, but challenges persist due to uncertain information reliability and the lack of expert validation. Our survey revealed consider…
View article: Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project
Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project Open
Objective To assess current treatment in macrophage activation syndrome (MAS) worldwide and to highlight any areas of major heterogeneity of practice. Methods A systematic literature search was performed in both EMBASE and PubMed databases…
View article: Long-term use of interleukin-1 inhibitors reduce flare activity in patients with fibrodysplasia ossificans progressiva
Long-term use of interleukin-1 inhibitors reduce flare activity in patients with fibrodysplasia ossificans progressiva Open
Objectives Fibrodysplasia ossificans progressiva (FOP) is one of the most catastrophic forms of genetic heterotopic ossification (HO). FOP is characterized by severe, progressive inflammatory flare-ups, that often lead to HO. The flare-ups…
View article: Defining Criteria for Disease Activity States in Systemic Juvenile Idiopathic Arthritis Based on the Systemic Juvenile Arthritis Disease Activity Score
Defining Criteria for Disease Activity States in Systemic Juvenile Idiopathic Arthritis Based on the Systemic Juvenile Arthritis Disease Activity Score Open
Objective Our objective was to develop and validate cutoff values in the systemic Juvenile Arthritis Disease Activity Score 10 (sJADAS10) that distinguish the states of inactive disease (ID), minimal disease activity (MDA), moderate diseas…
View article: Nlrp2 deletion ameliorates kidney damage in a mouse model of cystinosis
Nlrp2 deletion ameliorates kidney damage in a mouse model of cystinosis Open
Cystinosis is a rare autosomal recessive disorder caused by mutations in the CTNS gene that encodes cystinosin, a ubiquitous lysosomal cystine/H + antiporter. The hallmark of the disease is progressive accumulation of cystine and cystine c…
View article: Long-term efficacy and safety of subcutaneous tocilizumab in clinical trials of polyarticular or systemic juvenile idiopathic arthritis
Long-term efficacy and safety of subcutaneous tocilizumab in clinical trials of polyarticular or systemic juvenile idiopathic arthritis Open
Objective To investigate the safety and efficacy of subcutaneous tocilizumab (SC-TCZ) treatment in a long-term extension (LTE) of clinical trials in polyarticular or systemic juvenile idiopathic arthritis (pJIA or sJIA). Methods Patients w…
View article: P101 Anti-CD19 CAR-T cell therapy for refractory childhood-onset systemic lupus erythematosus
P101 Anti-CD19 CAR-T cell therapy for refractory childhood-onset systemic lupus erythematosus Open
Objective To report the first case of a patient with refractory childhood-onset SLE successfully treated with anti-CD19 CAR-T cell therapy. Methods A single infusion of fresh, autologous second-generation anti-CD19 CAR-T product (lentiviru…
View article: Hyperactivation and altered selection of B cells in patients with paediatric Sjogren’s syndrome
Hyperactivation and altered selection of B cells in patients with paediatric Sjogren’s syndrome Open
Objectives Paediatric Sjögren’s syndrome (pSS) is a rare chronic autoimmune disorder, characterised by inflammation of exocrine glands. B cell hyperactivation plays a central role in adult-onset Sjogren. This study was designed to analyse …