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1. Long-term use of interleukin-1 inhibitors reduce flare activity in patients with fibrodysplasia ossificans progressiva.

Author

Haviv, Ruby, Zeitlin, Leonid, Moshe, Veronica, Ziv, Amit, Rabinowicz, Noa, Benedetti, Fabrizio De, Prencipe, Giusi, Matteo, Valentina, Cunto, Carmen Laura De, Hsiao, Edward C, and Uziel, Yosef

Subjects
FIBRODYSPLASIA ossificans progressiva, DISEASE exacerbation, RESEARCH funding, HEALTH status indicators, VISUAL analog scale, QUESTIONNAIRES, TREATMENT duration, TREATMENT effectiveness, BLOOD plasma, PAIN, RESEARCH, CASE studies, DISEASE relapse, HEALTH outcome assessment, INTERLEUKIN-1, GLUCOCORTICOIDS, INTERLEUKINS, WELL-being, CHEMICAL inhibitors
Abstract

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 are associated with increased inflammatory cytokine production, suggesting auto-inflammatory features driven by IL-1β. This study describes the short- and long-term responses of FOP patients to anti-IL-1 therapy. Methods Previously, we reported that a patient with FOP treated with anti-IL-1 agents showed dramatically lower rates of flare-ups, improved flare-up symptoms, decreased use of glucocorticoids and apparently decreased size of residual lesions. Plasma analyses also showed marked elevation in IL-1β levels during a FOP flare, further supporting a role of IL-1β in the pathogenesis of FOP flares. Here, we report results from long-term therapy with IL-1 inhibitors in that patient and describe 3 additional patients, from two medical centres. Results All 4 patients showed persistent improvement in flare activity during treatment with IL-1 inhibitors, with minimal formation of new HO sites. Two patients who stopped therapy experienced a resurgence of flare activity that was re-suppressed upon re-initiation. These patients had IL-1β levels comparable to those in IL-1β-driven diseases. Child Health Assessment Questionnaires confirmed extensive subjective improvements in the pain and general health visual analogue scales. Conclusion This case series demonstrates significant benefits from IL-1 inhibitors for reducing flare activity and improving the general health of patients with FOP. These data provide strong support for additional studies to better understand the function of IL-1 inhibition, primarily in reducing the formation of new HO. Funding RH received support from the International FOP Association ACT grant; ECH received support from NIH/NIAMS R01AR073015 and the UCSF Robert Kroc Chair in Connective Tissue and Rheumatic Diseases III. [ABSTRACT FROM AUTHOR]

Published
2024
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2. Long-term efficacy and safety of subcutaneous tocilizumab in clinical trials of polyarticular or systemic juvenile idiopathic arthritis.

Author

Brunner, Hermine I, Ruperto, Nicolino, Ramanan, Athimalaipet V, Horneff, Gerd, Minden, Kirsten, Penades, Inmaculada Calvo, Alexeeva, Ekaterina, Cleary, Gavin, Stern, Sara M, Kone-Paut, Isabelle, Velázquez, María del Rocío Maldonado, Rabinovich, C Egla, Remesal, Agustin, Silva, Clovis Artur, Nikishina, Irina, Zucchetto, Mauro, Brockwell, Laura, Gordon, Oliver, Nagel, Sandra, and Benedetti, Fabrizio De

Subjects
THERAPEUTIC use of monoclonal antibodies, JUVENILE idiopathic arthritis, PATIENT safety, RESEARCH funding, CLINICAL trials, DESCRIPTIVE statistics, MONOCLONAL antibodies, DRUG efficacy, TOCILIZUMAB, SUBCUTANEOUS injections, INTERLEUKINS, EVALUATION, CHILDREN
Abstract

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 with pJIA or sJIA from two open-label, 52-week phase 1b core trials of SC-TCZ who had adequate response per investigator assessment entered the LTE and continued SC-TCZ treatment according to body weight–based dosing regimens until commercial availability or up to 5 years. Pharmacokinetics, pharmacodynamics, and efficacy were assessed for up to 3 years, and safety for up to 5 years in the LTE. Results Forty-four patients with pJIA and 38 patients with sJIA entered the LTE. Tocilizumab trough concentrations were maintained within the range expected to provide clinical benefit (mean values: pJIA, ∼10 μg/ml; sJIA, ∼75 μg/ml over 3 years). Pharmacodynamic parameters (interleukin-6, soluble interleukin-6 receptor, erythrocyte sedimentation rate, C-reactive protein) were maintained throughout the LTE at levels achieved in the core trials. Inactive disease per American College of Rheumatology provisional criteria was reported for 90% (17/19) and 53% (8/15) of patients with pJIA and 91% (10/11) and 92% (12/13) of patients with sJIA in the <30 and ≥30 kg body weight groups, respectively. Serious adverse events in the LTE were reported in six patients with pJIA (13.6%; five serious infections) and five patients with sJIA (13.2%; one serious infection). Conclusion Patients with pJIA or sJIA experienced long-term disease control with SC-TCZ treatment. Long-term safety was consistent with the known tocilizumab safety profile. Clinical trial registration clinicaltrials.gov, NCT02165345 [ABSTRACT FROM AUTHOR]

Published
2024
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4. Contributors

Author

Akikusa, Jonathan, primary, Albani, Salvatore, additional, Allen, Roger, additional, Alsaeid, Khaled, additional, Avčin, Tadej, additional, Babyn, Paul S., additional, Bagga, Arvind, additional, Barron, Karyl S., additional, Becker, Mara L., additional, Benseler, Susanne M., additional, Beukelman, Timothy, additional, Brogan, Paul, additional, Brunner, Hermine I., additional, Burgos-Vargas, Rubèn, additional, Buyon, Jill, additional, Cabral, David A., additional, Choo, Sharon, additional, Cimaz, Rolando, additional, Colbert, Robert Allen, additional, Cole, William G., additional, Davidson, Iris, additional, Benedetti, Fabrizio De, additional, Doria, Andrea S., additional, Dressler, Frank, additional, Duffy, Ciarán M., additional, Eleftheriou, Despina, additional, Feldman, Brian M., additional, Ferguson, Polly J., additional, Fuhlbrigge, Robert, additional, Gattorno, Marco, additional, Grom, Alexei A., additional, Hashkes, Philip J., additional, Houghton, Kristin, additional, Huppertz, Hans-Iko, additional, Ilowite, Norman T., additional, Jaeggi, Edgar, additional, Kastner, Daniel L., additional, Kirton, Adam, additional, Klein-Gitelman, Marisa, additional, Kuchta, Gay, additional, Lane, Jerome Charles, additional, Laxer, Ronald M., additional, LeBlanc, Claire, additional, Leeder, Steven J., additional, Legger, G. Elizabeth, additional, Li, Suzanne C., additional, Lindsley, Carol B., additional, Lovell, Dan, additional, Makitie, Outi, additional, Martini, Alberto, additional, Miller, Frederick W., additional, Morishita, Kimberly, additional, Nigrovic, Peter A., additional, Oen, Kiem G., additional, O'Neil, Kathleen M., additional, Ozen, Seza, additional, Pepmueller, Peri H., additional, Petty, Ross E., additional, Pope, Elena, additional, Prahalad, Sampath, additional, Prakken, Berent, additional, Rapoff, Michael, additional, Rider, Lisa G., additional, Rosé, Carlos Daniel, additional, Rosenbaum, James T., additional, Rosenberg, Alan M., additional, Roth, Johannes, additional, Guillermo, Ricardo Alberto, additional, Schneider, Rayfel, additional, Scott, Christiaan, additional, Sherry, David D., additional, Silverman, Earl, additional, Son, Mary Beth, additional, Sundel, Robert P., additional, Thompson, Susan D., additional, Tiedemann, Karin, additional, Tse, Shirley M.L., additional, Tucker, Lori, additional, Uziel, Yosef, additional, van Montfrans, Joris, additional, Vazques-Mellado, Janitzia, additional, Ward, Leanne, additional, Wedderburn, Lucy R., additional, Wouters, Carine, additional, Wright, James, additional, Wulffraat, Nico M., additional, Zemel, Lawrence, additional, and Zulian, Francesco, additional

Published
2016
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5. Efficacy and safety of tocilizumab for polyarticular-course juvenile idiopathic arthritis in the open-label two-year extension of a phase III trial

Author

Brunner, Hermine I., Xavier, Ricardo Machado, and Benedetti, Fabrizio de

Subjects
Estudo clínico, Artrite, Eficácia, Adolescente
Abstract

Objective. To report the 2-year efficacy and safety of tocilizumab (TCZ) in patients with polyarticular-course juvenile idiopathic arthritis (JIA). Methods. Patients ages 2–17 years with active polyarticular-course JIA, in whom treatment with methotrexate was unsuccessful, received 16 weeks of open-label intravenous TCZ in part 1 (once every 4 weeks: 8 mg/kg or 10 mg/kg for body weight [BW] 1 of the remaining JIA CRVs by >30%) at week 16 were randomly assigned (1:1) to receive TCZ or placebo in part 2. Patients remained in part 2 until either week 40 or the occurrence of JIA flare. Upon starting part 3, all patients received open-label TCZ. At week 104 of the study, efficacy was assessed using JIA-ACR50/70/90 response rates (defined as 50%, 70%, or 90% improvement, respectively), achievement of inactive disease, and the Juvenile Arthritis Disease Activity Score in 71 joints (JADAS-71). Safety was assessed in the all-exposure population per 100 patient-years of exposure. Results. Overall, 188 patients entered part 1, 166 patients entered part 2, and 160 patients entered part 3. By week 104, among the 188 patients in the modified intent-to-treat group who received TCZ, JIA-ACR50/70/90 response rates were 80.3%/77.1%/59.6%, respectively, the median JADAS-71 score decreased from 3.6 at week 40 to 0.7 at week 104, 51.1% of patients had achieved inactive disease, and 31 of 66 patients who had been receiving glucocorticoids discontinued them. Adverse event (AE) and serious AE rates were 406.5 per 100 patient-years and 11.1 per 100 patient-years, respectively. The infection rate was 151.4 per 100 patient-years, and the serious infection rate was 5.2 per 100 patient-years. Conclusion. Patients treated with TCZ for polyarticular-course JIA showed high-level disease control for up to 2 years. The TCZ safety profile was consistent with that previously reported.

Published
2021

6. IL-1 BLOCKADE IN PEDIATRIC RECURRENT PERICARDITIS: A MULTICENTRIC RETROSPECTIVE STUDY ON THE ITALIAN COHORT

Author

Caorsi, Roberta, Insalaco, Antonella, Longo, Chiara, Martini, Giorgia, Cattalini, Marco, Consolini, Rita, Filocamo, Giovanni, Rimini, Alessandro, Federici, Silvia, Celani, Camilla, Maggio, Maria Cristina, Romano, Micol, Teruzzi, Barbara Lia, Taddio, Andrea, Minaici, Angela, Martino, Silvana, Torre, Francesco La, Fanti, Alessandro De, Dagna, Lorenzo, Bigucci, Barbara, Brucato, Antonio, Benedetti, Fabrizio De, Gattorno, Marco, Caorsi, Roberta, Insalaco, Antonella, Longo, Chiara, Martini, Giorgia, Cattalini, Marco, Consolini, Rita, Filocamo, Giovanni, Rimini, Alessandro, Federici, Silvia, Celani, Camilla, Maggio, Maria Cristina, Romano, Micol, Teruzzi, Barbara Lia, Taddio, Andrea, Minaici, Angela, Martino, Silvana, Torre, Francesco La, Fanti, Alessandro De, Dagna, Lorenzo, Bigucci, Barbara, Brucato, Antonio, Benedetti, Fabrizio De, and Gattorno, Marco

Subjects
Acute pericarditis, colchicine, anakinra, Settore MED/38 - Pediatria Generale E Specialistica
Abstract

Background: Acute pericarditis is an inflammatory condition causing the occurrence of pericardial effusion. In a third of patients, the disease is recurrent. Most of the cases are idiopathic or occur after a pericardial procedure. First line treatment of idiopathic pericarditis consists in NSAIDs and colchicine; glucocorticoids represent the second line treatment in resistant or intolerant cases. The use of different biologics and immunosoppressant has been reported, with variable responses. A recent clinical trial has enlightened the effectiveness of anakinra in patients with colchicine-resistant recurrent pericarditis. Objectives: To describe the clinical characteristics and response to treatment in a cohort of paediatric patients with recurrent pericarditis treated with IL inhibitors. Methods: Pediatric patients with recurrent pericarditis followed at 19 Italian centers of paediatric rheumatology or cardiology and treated with IL1 inhibitors were included in the study. Demographic, clinical and response to treatment data were retrospectively collected. Results: 55 patients were included in the study. The mean age at onset of the first episode of pericarditis was 12.53 years. The mean number of relapses of pericarditis before the beginning of treatment with IL1 inhibitors was of 3.4. 53 out of 55 patients had previously received treatment with NSAIDS and 44 colchicine. 44 patients received steroidal treatment: 2 of them displayed a steroidal-resistance and 39 steroidal-dependence with reoccurrence of the symptoms following any attempt to reduce or withdraw this treatment. Anakinra (mean dosage of 1.67 mg/kg/day) was used as first Il-1 inhibitor in 54 of the 55 patients, Canakinumab (150 mg every 4 weeks) in one. 53 out of 54 patients treated with anakinra displayed a complete clinical response to treatment within a mean of 2 days: NSAIDs, glucocorticoids and colchicine were withdraw in 25 out of 26, 31 out of 35 and 15 out of 32 patients respectively. 50 of 54 patients displayed a complete response; among these, three were switched to Canakinumab, 17 patients continued treatment at the same dosage while in 30 patients a reduction of treatment was attempted. 12 patients presented, during anakinra tapering, a disease flare, promptly resolved after an increasing of the dosage. The remaining 18 patients did not present any flare despite the reduction of the drug. Anakinra was withdrawn in 16 patients, with recurrence of the symptoms in 11 (9 restarted anakinra, 2 were treated with glucocorticoids and colchicine, with good response). 5 patients were treated with Canakinumab: 1 as first anti-IL1 drug, 4 were switched from anakinra (two for poor compliance, one for side effects and one for incomplete control of the disease). 2 out of five patients had a complete control of the diseases, 2 patients discontinued the treatment because of inefficacy and 1 patient required low dose of glucocorticoids to control the disease. At last follow-up 34 patients were on anakinra, 7 on anakinra and colchicine, 2 on canakinumab, 1 on canakinumab plus colchicine and NSAIDs. In 9 patients all treatments were withdrawn for complete control of the disease. Conclusion: This study confirm the effectiveness of IL-1 blockade in paediatric patients with recurrent pericarditis. However, most of the patients require prolonged treatment to maintain clinical remission. Moreover, in our cohort of patients the rate of response was higher for anakinra then for canakinumab, suggesting a possible role of IL1a in the pathogenesis of this condition.

Published
2019

7. Additional file 1 of Tocilizumab may slow radiographic progression in patients with systemic or polyarticular-course juvenile idiopathic arthritis: post hoc radiographic analysis from two randomized controlled trials

Author

Malattia, Clara, Ruperto, Nicolino, Pederzoli, Silvia, Palmisani, Elena, Pistorio, Angela, Wouters, Carine, Dolezalova, Pavla, Flato, Berit, Garay, Stella, Giancane, Gabriella, Wells, Chris, Douglass, Wendy, Brunner, Hermine I., Benedetti, Fabrizio De, and Ravelli, Angelo

Subjects
respiratory system, respiratory tract diseases
Abstract

Additional file 1 Supplemental Methods. Inter-reader reliability methods for assessment of radiographs. Radiographic progression using cutoff of zero.

Published
2020
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8. Long-term efficacy and safety of canakinumab in patients with mevalonate kinase deficiency: results from the randomised Phase 3 CLUSTER trial.

Author

Jeyaratnam, Jerold, Simon, Anna, Calvo, Inmaculada, Constantin, Tamas, Shcherbina, Anna, Hofer, Michael, Gattorno, Marco, Martini, Alberto, Bader-Meunier, Brigitte, Vastert, Bas, Levy, Jeremy, Dekker, Elise, Benedetti, Fabrizio de, and Frenkel, Joost

Subjects
THERAPEUTIC use of monoclonal antibodies, DRUG efficacy, C-reactive protein, ACUTE phase proteins, MONOCLONAL antibodies, INTERLEUKIN-1, MEVALONATE kinase deficiency, RANDOMIZED controlled trials, DESCRIPTIVE statistics, PATIENT safety, AUTOINFLAMMATORY diseases, EVALUATION
Abstract

Objectives To evaluate the long-term efficacy and safety of canakinumab in patients with mevalonate kinase deficiency during the open label extension (weeks 41–113) of the randomized controlled CLUSTER trial. Methods During a 72-week period, patients received open-label canakinumab 150 or 300 mg, every 4 or 8 weeks. The disease activity was evaluated every 8 weeks using physician global assessment and counting the number of flares. Concentrations of CRP and serum amyloid A protein were measured. The safety was studied by determination and classification of observed adverse events. The safety and efficacy were analysed separately in three subgroups of patients receiving a cumulative dose of less than <35 mg/kg, ≥35 to <70 mg/kg or ≥70 mg/kg. Results Of the 74 patients who started the CLUSTER study, 66 entered Epoch 4 and 65 completed it. During the 72-week period, 42 (64%) patients experienced no flares, while 13 (20%) had one flare, as compared with a median of 12 flares per year reported at baseline. Low physician global assessment scores were seen at the end of the study for all groups with >90% reporting minimal disease activity or none at all. Median CRP concentrations were consistently equal or lower than 10 mg/l, while median serum amyloid A concentrations remained only slightly above the normal range of 10 mg/l. The study showed no new or unexpected adverse events. Conclusion Canakinumab proved effective to control disease activity and prevent flares in mevalonate kinase deficiency during the 72-week study period. No new safety concerns were reported. Trial registration NCT02059291. https://clinicaltrials.gov. [ABSTRACT FROM AUTHOR]

Published
2022
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9. Defining colchicine resistance/intolerance in patients with familial Mediterranean fever: a modified-Delphi consensus approach.

Author

Özen, Seza, Sag, Erdal, Ben-Chetrit, Eldad, Gattorno, Marco, Gül, Ahmet, Hashkes, Philip J, Kone-Paut, Isabelle, Lachmann, Helen J, Tsitsami, Elena, Twilt, Marinka, Benedetti, Fabrizio de, and Kuemmerle-Deschner, Jasmin B

Subjects
MEDICAL quality control, CONSENSUS (Social sciences), ONLINE information services, MEDICAL information storage & retrieval systems, MEDICAL databases, INFORMATION storage & retrieval systems, INFLAMMATION, SYSTEMATIC reviews, GENETIC disorders, DRUG resistance, HEALTH outcome assessment, QUALITY of life, QUESTIONNAIRES, COLCHICINE, MEDLINE, DELPHI method
Abstract

Objectives Colchicine is the main treatment for FMF. Although a number of individuals with FMF are intolerant/resistant to colchicine, there is no standard definition of colchicine resistance/intolerance. We developed a set of evidence-based core statements defining colchicine resistance/intolerance in patients with FMF that may serve as a guide for clinicians and health authorities. Methods A set of statements was identified using a modified-Delphi consensus-based approach. The process involved development of an initial colchicine resistance/intolerance-related questionnaire derived from a systematic literature review. The questionnaire, which was completed by an international panel of 11 adult and paediatric rheumatologists with expertise in FMF, was analysed anonymously. The results informed draft consensus statements that were discussed by a round-table expert panel, using a nominal group technique to agree on the selection and wording of the final statements. Results Consensus among the panel was achieved on eight core statements defining colchicine resistance/intolerance in patients with FMF. A definition of resistance was agreed upon that included recurrent clinical attacks (average one or more attacks per month over a 3-month period) or persistent laboratory inflammation in between attacks. Other core statements recognize the importance of assessing treatment adherence, and the impact of active disease and intolerance to colchicine on quality of life. Conclusion Based on expert opinion, a set of evidence-based core statements defining colchicine resistance/intolerance in patients with FMF were identified to help guide clinicians and health authorities in the management of patients with FMF. [ABSTRACT FROM AUTHOR]

Published
2021
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10. Additional file 1: of An international delphi survey for the definition of the variables for the development of new classification criteria for periodic fever aphtous stomatitis pharingitis cervical adenitis (PFAPA)

Author

Vanoni, Federica, Federici, Silvia, AntĂłn, Jordi, Karyl Barron, Brogan, Paul, Benedetti, Fabrizio De, Dedeoglu, Fatma, Demirkaya, Erkan, Hentgen, Veronique, Kallinich, Tilmann, Laxer, Ronald, Russo, Ricardo, Natasa Toplak, Uziel, Yosef, Martini, Alberto, Ruperto, Nicolino, Gattorno, Marco, and Hofer, Michael

Abstract

Table S1. Variables coming from the first survey. Table S2. Variable rank from the second Delphi. (DOCX 662Â kb)

Published
2018
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12. THU0513 WHOLE BLOOD CELLS FROM PATIENTS WITH SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS (SJIA) IN CLINICAL INACTIVE DISEASE DISPLAY A DYSREGULATED RESPONSE TO TLR-4 STIMULATION

Author

Pascarella, Antonia, primary, Pardeo, Manuela, additional, Caiello, Ivan, additional, Bracaglia, Claudia, additional, Rossi, Marianna, additional, Marucci, Giulia, additional, Sacco, Emanuela, additional, Benedetti, Fabrizio De, additional, and Prencipe, Giusi, additional

Published
2019
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13. OP0258 LESSON FROM EUROFEVER REGISTRY AFTER THE FIRST TEN YEARS OF ENROLLMENT

Author

Finetti, Martina, primary, Gueli, Ilaria, additional, Frenkel, Joost, additional, Özen, Seza, additional, Lachmann, Helen J., additional, Benedetti, Fabrizio De, additional, Koné-Paut, Isabelle, additional, Wouters, Carine, additional, Brogan, Paul, additional, Girschick, Hermann, additional, Neven, Benedicte, additional, Martini, Alberto, additional, Ruperto, Nicolino, additional, and Gattorno, Marco, additional

Published
2019
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14. THU0516 LONG-TERM SAFETY OF SUBCUTANEOUS TOCILIZUMAB ADMINISTRATION IN SYSTEMIC AND POLYARTICULAR JUVENILE IDIOPATHIC ARTHRITIS

Author

Benedetti, Fabrizio De, primary, Ruperto, Nicolino, additional, Ramanan, Athimalaipet, additional, Cuttica, Ruben, additional, Weiss, Jennifer E., additional, Henrickson, Michael, additional, Schmeling, Heinrike, additional, Anton, Jordi, additional, Minden, Kirsten, additional, Horneff, Gerd, additional, Gámir, Maria Luz Gámir, additional, Hufnagel, Markus, additional, Douglass, Wendy, additional, Wells, Chris, additional, Mallalieu, Navita L., additional, Martini, Alberto, additional, Lovell, Daniel J, additional, and Brunner, Hermine, additional

Published
2019
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16. FRI0539 WNT6 MUTATION CAUSES AN EARLY ONSET GRANULOMATOSUS INTESTINAL DISEASE WITH RECURRENT HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS (HLH)

Author

Bracaglia, Claudia, primary, Knafelz, Daniela, additional, Bracci, Fiammetta, additional, Insalaco, Antonella, additional, Marucci, Giulia, additional, Pardeo, Manuela, additional, Prencipe, Giusi, additional, Caiello, Ivan, additional, Pascarella, Antonia, additional, Niceta, Marcello, additional, Pantaleoni, Francesca, additional, Ciolfi, Andrea, additional, Papadatou, Bronislava, additional, Tartaglia, Marco, additional, Torre, Giuliano, additional, and Benedetti, Fabrizio De, additional

Published
2019
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17. AB0185 INTERFERON-Γ AMPLIFIES IMMUNE RESPONSE MEDIATED BY TYPE I INTERFERONS IN PAEDIATRIC SYSTEMIC LUPUS ERYTHEMATOSUS AND CORRELATES WITH DISEASE ACTIVITY

Author

Moneta, Gian Marco, primary, Bracaglia, Claudia, additional, Caiello, Ivan, additional, Pecoraro, Raffaele, additional, Farroni, Chiara, additional, Basta, Fabio, additional, Bracci-Laudiero, Luisa, additional, Carsetti, Rita, additional, Benedetti, Fabrizio De, additional, and Marasco, Emiliano, additional

Published
2019
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20. THU0517 THE LONGITUDINAL EUROFEVER PROJECT: AN UPDATE ON ENROLLMENT

Author

Gueli, Ilaria, primary, Finetti, Martina, additional, Benedetti, Fabrizio De, additional, Anton, Jordi, additional, Alessio, Maria, additional, Frenkel, Joost, additional, Cantarini, Luca, additional, Gallizzi, Romina, additional, Manubens, Judith Sanchez, additional, Cattalini, Marco, additional, Papadopoulou-Alataki, Efimia, additional, Cimaz, Rolando, additional, Rigante, Donato, additional, Olivieri, Alma Nunzia, additional, Dolezalova, Pavla, additional, Martini, Alberto, additional, Ruperto, Nicolino, additional, and Gattorno, Marco, additional

Published
2019
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23. FRI0540 A NOVEL AUTOINFLAMMATORY DISEASE CHARACTERIZED BY NEONATAL-ONSET CYTOPENIA WITH AUTOINFLAMMATION, RASH, AND HEMOPHAGOCYTOSIS (NOCARH) DUE TO ABERRANT CDC42 FUNCTION

Author

Lam, Michael T., primary, Coppola, Simona, additional, Krumbach, Oliver H., additional, Prencipe, Giusi, additional, Insalaco, Antonella, additional, Cifaldi, Cristina, additional, Brigida, Immacolata, additional, Scala, Serena, additional, Niceta, Marcello, additional, Ciolfi, Andrea, additional, Carisey, Alexandre, additional, Akbarzadeh, Mohammad, additional, Finocchi, Andrea, additional, Locatelli, Franco, additional, Cancrini, Caterina, additional, Aiuti, Alessandro, additional, Ahmadian, Reza, additional, Orange, Jordan S., additional, Benedetti, Fabrizio De, additional, and Tartaglia, Marco, additional

Published
2019
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26. OP0204 EMAPALUMAB, AN INTERFERON GAMMA (IFN-Y)-BLOCKING MONOCLONAL ANTIBODY, IN PATIENTS WITH MACROPHAGE ACTIVATION SYNDROME (MAS) COMPLICATING SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS (SJIA)

Author

Benedetti, Fabrizio De, primary, Brogan, Paul, additional, Grom, Alexei, additional, Quartier, Pierre, additional, Schneider, Rayfel, additional, Graaf, Kathy De, additional, Jacqmin, Philippe, additional, Ballabio, Maria, additional, and Min, Cristina de, additional

Published
2019
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27. OP0255 MICROBIOTA TRANSPLANT TO CONTROL INFLAMMATION IN A NLRC4-RELATED DISEASE PATIENT WITH RECURRENT HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS (HLH)

Author

Bracaglia, Claudia, primary, Marucci, Giulia, additional, Chierico, Federica Del, additional, Russo, Alessandra, additional, Pardeo, Manuela, additional, Insalaco, Antonella, additional, Prencipe, Giusi, additional, Caiello, Ivan, additional, Dolezalova, Pavla, additional, Fingerhutova, Sarka, additional, Benedetti, Fabrizio De, additional, and Putignani, Lorenza, additional

Published
2019
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28. AB0940 OBESITY AND NUMBER OF ACTIVE JOINTS AT TUMOR NECROSIS FACTOR-INHIBITOR START ARE ASSOCIATED WITH SHORTER REMISSION DURATION AFTER WITHDRAWAL IN PATIENTS WITH JUVENILE IDIOPATHIC ARTHRITIS: PRELIMINARY RESULTS FROM A MONOCENTRIC COHORT

Author

Basta, Fabio, primary, Jadoun, Hanan, additional, Petrone, Maria Isabella, additional, Aquilani, Angela, additional, Uva, Andrea, additional, Nicolai, Rebecca, additional, Benedetti, Fabrizio De, additional, and Magni-Manzoni, Silvia, additional

Published
2019
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29. AB1062 INTER-LABORATORY COMPARISON OF TYPE I INTERFERON SIGNATURE ANALYSES: PAVING THE WAY TO SHARE RECOMMENDATIONS.

Author

Tesser, Alessandra, primary, Moneta, Gian Marco, additional, Insalaco, Antonella, additional, Nicolai, Rebecca, additional, Bracaglia, Claudia, additional, Moressa, Valentina, additional, Pastore, Serena, additional, Taddio, Andrea, additional, Tommasini, Alberto, additional, Bocca, Paola, additional, Gattorno, Marco, additional, Benedetti, Fabrizio De, additional, and Stefano, Volpi, additional

Published
2019
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31. FRI0572 DISABILITY AND HEALTH-RELATED QUALITY OF LIFE OUTCOMES IN PATIENTS WITH SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS TREATED WITH TOCILIZUMAB IN A PHASE 3 RANDOMIZED CONTROLLED TRIAL

Author

Ruperto, Nicolino, primary, Chen, Chen, additional, Martini, Alberto, additional, Espada, Graciela, additional, Joos, Rik, additional, Akikusa, Jonathan, additional, Chaitow, Jeffrey, additional, Maria Luz, Gámir Gámir, additional, Kimura, Yukiko, additional, Rietschel, Christoph, additional, Siri, Daniel, additional, Smolewska, Elzbieta, additional, Schmeling, Heinrike, additional, Brown, Diane, additional, Benedetti, Fabrizio De, additional, Lovell, Daniel J., additional, Huang, Bin, additional, and Brunner, Hermine, additional

Published
2019
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32. SAT0490 IL-1 BLOCKADE IN PEDIATRIC RECURRENT PERICARDITIS: A MULTICENTRIC RETROSPECTIVE STUDY ON THE ITALIAN COHORT

Author

Caorsi, Roberta, primary, Insalaco, Antonella, additional, Longo, Chiara, additional, Martini, Giorgia, additional, Cattalini, Marco, additional, Consolini, Rita, additional, Filocamo, Giovanni, additional, Rimini, Alessandro, additional, Federici, Silvia, additional, Celani, Camilla, additional, Maggio, Maria Cristina, additional, Romano, Micol, additional, Teruzzi, Barbara Lia, additional, Taddio, Andrea, additional, Minaici, Angela, additional, Martino, Silvana, additional, Torre, Francesco La, additional, Fanti, Alessandro De, additional, Dagna, Lorenzo, additional, Bigucci, Barbara, additional, Brucato, Antonio, additional, Benedetti, Fabrizio De, additional, and Gattorno, Marco, additional

Published
2019
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34. FRI0549 SARILUMAB, A HUMAN MONOCLONAL ANTIBODY TO THE INTERLEUKIN-6 (IL-6) RECEPTOR, IN POLYARTICULAR-COURSE JUVENILE IDIOPATHIC ARTHRITIS (PCJIA): A 12-WEEK MULTINATIONAL OPEN-LABEL DOSE-FINDING STUDY

Author

Benedetti, Fabrizio De, primary, Penadés, Inmaculada Calvo, additional, Pérez, Nadina E. Rubio, additional, Maschan, Alexey, additional, Quartier, Pierre, additional, Żuber, Zbigniew, additional, Stanislav, Marina, additional, Barria, Raul, additional, Garulo, Daniel Clemente, additional, Cornejo, Gabriel Vega, additional, Liu, Nancy, additional, Xu, Christine, additional, Giannelou, Angeliki, additional, Akinlade, Bolanle, additional, and Baret-Cormel, Lydie, additional

Published
2019
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37. OP0254 CANAKINUMAB IMPROVES PATIENT-REPORTED OUTCOMES IN PATIENTS WITH RECURRENT FEVER SYNDROMES: RESULTS FROM A PHASE 3 TRIAL (CLUSTER)

Author

Lachmann, Helen J., primary, Lauwerys, Bernard, additional, Miettunen, Paivi, additional, Kallinich, Tilmann, additional, Horneff, Gerd, additional, Brik, Riva, additional, Manna, Rafaelle, additional, Murias, Sara, additional, Savic, Sinisa, additional, Smeets, Serge, additional, Benedetti, Fabrizio De, additional, and Simon, Anna, additional

Published
2019
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38. SAT0511 CANAKINUMAB IN SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS: CLINICAL INACTIVE DISEASE RATE AND SAFETY IN ITALIAN PATIENTS

Author

Pardeo, Manuela, primary, Bracaglia, Claudia, additional, Piscitelli, Anna Lucia, additional, Matteis, Arianna De, additional, Tibaldi, Jessica, additional, Alessio, Maria, additional, Marino, Achille, additional, Conti, Giovanni, additional, Maggio, Maria Cristina, additional, Alizzi, Clotilde, additional, Licciardi, Francesco, additional, Olivieri, Alma Nunzia, additional, Filocamo, Giovanni, additional, Orlando, Francesca, additional, Martino, Silvana, additional, Cimaz, Rolando, additional, Ravelli, Angelo, additional, and Benedetti, Fabrizio De, additional

Published
2019
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40. 170 Safety and efficacy of subcutaneous tocilizumab in patients with systemic and polyarticular juvenile idiopathic arthritis

Author

Ramanan, Athimalaipet V, primary, Brunner, Hermine I, additional, Ruperto, Nicola, additional, Martini, Alberto, additional, Cuttica, Rubén J, additional, Weiss, Jennifer E, additional, Henrickson, Michael, additional, Schmeling, Heinrike, additional, Antón, Jordi, additional, Minden, Kirsten, additional, Horneff, Gerd, additional, Gámir-Gámir, María Luz, additional, Hufnagel, Markus, additional, Douglass, Wendy, additional, Wells, Chris, additional, Wimalasundera, Sunethra, additional, Mallalieu, Navita L, additional, Lovell, Daniel J, additional, and Benedetti, Fabrizio De, additional

Published
2019
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41. Correlation of serum interleukin-6 levels with joint involvement and thrombocytosis in systemic juvenile rheumatoid arthritis

Author

Benedetti, Fabrizio De, Massa, Margherita, Robbioni, Piera, Ravelli, Angelo, Burgio, Giuseppe R., and Martini, Alberto

Subjects
Interleukin-6 -- Measurement, Rheumatoid arthritis in children -- Physiological aspects, Health
Abstract

In one form of juvenile rheumatoid arthritis (JRA), affected children develop systemic symptoms such as high fever, skin rash, and enlargement of liver and spleen and of lymph nodes, in addition to chronic arthritis. Laboratory tests indicate the presence of inflammation, including elevations in levels of disease-associated proteins such as acute-phase protein, and increased levels of platelets and neutrophils (a type of white blood cell important in fighting infection). Many of these features can be induced by interleukin-6 (IL-6), a protein hormone which affects the function of immune and other cells. IL-6 is produced by many types of cells, including some usually found in joints, and adults with rheumatoid arthritis (RA) have elevated IL-6 levels in blood and synovial (joint) fluid. Blood levels of IL-6 were studied in 25 patients with systemic-onset JRA and compared with those in seven patients with a systemic illness like JRA but without arthritis, and in 17 healthy control subjects. Blood levels of IL-6 were significantly higher in JRA patients with active systemic disease than in control children, but not in patients in remission. Levels in children with active disease were much higher than those found in adults with RA. Levels did not vary according to type of medication used by patients. IL-6 levels peaked at the same time as fever in three patients who were tested. IL-6 levels were similar in children with systemic JRA regardless of whether fever was present, and were significantly higher than in children with systemic-like illness. In children with active illness, IL-6 levels correlated with arthritis severity and with blood platelet levels. (Consumer Summary produced by Reliance Medical Information, Inc.)

Published
1991

42. Dynamic Contrast-Enhanced MRI Confirms Rapid And Sustained Improvement Of Rheumatoid Arthritis Induced By Tocilizumab Treatment: An Italian Multicentre Study.

Author

Cimmino, Marco A, Parodi, Massimiliano, Barbieri, Francesca, Bombardieri, Stefano, Zampogna, Giuseppe, Iagnocco, Annamaria, Batticciotto, Alberto, Sconfienza, Luca Maria, Sinigaglia, Luigi, Benedetti, Fabrizio De, Atzeni, Fabiola, and Sarzi-Puttini, Piercarlo

Subjects
CONTRAST-enhanced magnetic resonance imaging, RHEUMATOID arthritis, TOCILIZUMAB, THERAPEUTICS
Published
2020
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44. Anakinra in children and adults with Still's disease.

Author

Vastert, Sebastiaan J, Jamilloux, Yvan, Quartier, Pierre, Ohlman, Sven, Koskinen, Lisa Osterling, Kullenberg, Torbjörn, Franck-Larsson, Karin, Fautrel, Bruno, and Benedetti, Fabrizio de

Subjects
ANTIRHEUMATIC agents, GLUCOCORTICOIDS, INTERLEUKIN-1, PATIENT safety, PROTEINS, RHEUMATOID arthritis, STILL'S disease, TREATMENT effectiveness, EARLY medical intervention, CHEMICAL inhibitors
Abstract

Systemic juvenile idiopathic arthritis and adult-onset Still's disease are rare autoinflammatory disorders with common features, supporting the recognition of these being one disease—Still's disease—with different ages of onset. Anakinra was recently approved by the European Medicines Agency for Still's disease. In this review we discuss the reasoning for considering Still's disease as one disease and present anakinra efficacy and safety based on the available literature. The analysis of 27 studies showed that response to anakinra in Still's disease was remarkable, with clinically inactive disease or the equivalent reported for 23–100% of patients. Glucocorticoid reduction and/or stoppage was reported universally across the studies. In studies on paediatric patients where anakinra was used early or as first-line treatment, clinically inactive disease and successful anakinra tapering/stopping occurred in >50% of patients. Overall, current data support targeted therapy with anakinra in Still's disease since it improves clinical outcome, especially if initiated early in the disease course. [ABSTRACT FROM AUTHOR]

Published
2019
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45. A patient with stimulator of interferon genes–associated vasculopathy with onset in infancy without skin vasculopathy.

Author

Raffaele, Carmela Gerarda Luana, Messia, Virginia, Moneta, Gianmarco, Caiello, Ivan, Federici, Silvia, Pardeo, Manuela, Bracaglia, Claudia, Benedetti, Fabrizio De, and Insalaco, Antonella

Subjects
VASCULAR disease diagnosis, BLOOD sedimentation, VASCULAR diseases, C-reactive protein, CLINICAL pathology, GENE expression, INTERFERONS, NEUROTRANSMITTER uptake inhibitors, PROTEINS, JOINT pain, JANUS kinases, CHILDREN
Abstract

The article discusses a study which has found that skin involvement is not necessary for the diagnosis of stimulator of interferon genes (STING)-associated vasculopathy with onset in infancy (SAVI) syndrome. It describes the case of a 4-year-old girl with SAVI syndrome, whose only presenting features were lung involvement and recurrent fever with inflammation.

Published
2020
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46. Prediction of inactive disease in juvenile idiopathic arthritis: a multicentre observational cohort study.

Author

Dijkhuizen, Evert H Pieter van, Aidonopoulos, Orfeas, Haar, Nienke M ter, Marafon, Denise Pires, Magni-Manzoni, Silvia, Ioannidis, Yannis E, Putignani, Lorenza, Vastert, Sebastiaan J, Malattia, Clara, and Benedetti, Fabrizio De

Subjects
COMORBIDITY, ALGORITHMS, CYTOKINES, HEMOGLOBINS, INFLAMMATORY mediators, JOINTS (Anatomy), LONGITUDINAL method, MEDICAL cooperation, SCIENTIFIC observation, RESEARCH, RESEARCH evaluation, JUVENILE idiopathic arthritis, RISK assessment, GUT microbiome, SYMPTOMS, DISEASE duration, DISEASE complications
Abstract

Objectives To predict the occurrence of inactive disease in JIA in the first 2 years of disease. Methods An inception cohort of 152 treatment-naïve JIA patients with disease duration <6 months was analysed. Potential predictors were baseline clinical variables, joint US, gut microbiota composition and a panel of inflammation-related compounds in blood plasma. Various algorithms were employed to predict inactive disease according to Wallace criteria at 6-month intervals in the first 2 years. Performance of the models was evaluated using the split-cohort technique. The cohort was analysed in its entirety, and separate models were developed for oligoarticular patients, polyarticular RF negative patients and ANA positive patients. Results All models analysing the cohort as a whole showed poor performance in test data [area under the curve (AUC): <0.65]. The subgroup models performed better. Inactive disease was predicted by lower baseline juvenile arthritis DAS (JADAS)-71 and lower relative abundance of the operational taxonomic unit Mogibacteriaceae for oligoarticular patients (AUC in test data: 0.69); shorter duration of morning stiffness, higher haemoglobin and lower CXCL-9 levels at baseline for polyarticular RF negative patients (AUC in test data: 0.69); and shorter duration of morning stiffness and higher baseline haemoglobin for ANA positive patients (AUC in test data: 0.72). Conclusion Inactive disease could not be predicted with satisfactory accuracy in the whole cohort, likely due to disease heterogeneity. Interesting predictors were found in more homogeneous subgroups. These need to be validated in future studies. [ABSTRACT FROM AUTHOR]

Published
2018
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47. It is about time: the first validated biomarker for early diagnosis of systemic juvenile idiopathic arthritis.

Author

Pardeo, Manuela, Vastert, Sebastiaan J, and Benedetti, Fabrizio De

Subjects
BIOMARKERS, SERIAL publications, JUVENILE idiopathic arthritis
Abstract

The author reflects on the approved biomarkers for the early diagnosis of systemic juvenile idiopathic arthritis (sJIA), which is a rare childhood polygenic autoinflammatory disease. Other topics include the sJIA symptoms like arthralgia, fever and rashes, the study which assessed the performance of myeloid-related protein 8/14 (MRP8/14) as diagnostic biomarker of sJIA, and the effectiveness of the interleukin 1 (IL-1) and IL-6 inhibitors in managing patients with sJIA.

Published
2022
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49. A45: Neutropenia With Tocilizumab Treatment Is Not Associated With Increased Infection Risk in Patients With Polyarticular-Course Juvenile Idiopathic Arthritis

Author

Benedetti, Fabrizio De, primary, Rubio-Pérez, Nadina, additional, Salazar, Carolina Duarte, additional, Goodman, Steven, additional, Job-Deslandre, Chantal, additional, Joos, Rik, additional, Kone-Paut, Isabelle, additional, Minden, Kirsten, additional, Onel, Karen, additional, Porter-Brown, Benjamin, additional, Bharucha, Kamal, additional, Wang, Jianmei, additional, Martini, Alberto, additional, Lovell, Daniel J., additional, and Brunner, Hermine I., additional

Published
2014
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