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39 results on '"De Marchi, Ginevra"'

1. Anifrolumab in Refractory Systemic Lupus Erythematosus: A Real-World, Multicenter Study.

Author

Tani, Chiara, Cardelli, Chiara, Zen, Margherita, Moroni, Luca, Piga, Matteo, Ceccarelli, Fulvia, Fasano, Serena, De Marchi, Ginevra, Coladonato, Laura, Emmi, Giacomo, Gatto, Mariele, Trentin, Francesca, Ramirez, Giuseppe A., Chessa, Elisabetta, Gallina, Gabriele, Picciariello, Licia, Patrone, Martina, Urban, Maria L., Biancalana, Edoardo, and Quartuccio, Luca

Published
2024
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2. Safety profile of the interleukin-1 inhibitors anakinra and canakinumab in real-life clinical practice: a nationwide multicenter retrospective observational study

Author

Sota, Jurgen, Vitale, Antonio, Insalaco, Antonella, Sfriso, Paolo, Lopalco, Giuseppe, Emmi, Giacomo, Cattalini, Marco, Manna, Raffaele, Cimaz, Rolando, Priori, Roberta, Talarico, Rosaria, de Marchi, Ginevra, Frassi, Micol, Gallizzi, Romina, Soriano, Alessandra, Alessio, Maria, Cammelli, Daniele, Maggio, Maria Cristina, Gentileschi, Stefano, Marcolongo, Renzo, La Torre, Francesco, Fabiani, Claudia, Colafrancesco, Serena, Ricci, Francesca, Galozzi, Paola, Viapiana, Ombretta, Verrecchia, Elena, Pardeo, Manuela, Cerrito, Lucia, Cavallaro, Elena, Olivieri, Alma Nunzia, Paolazzi, Giuseppe, Vitiello, Gianfranco, Maier, Armin, Silvestri, Elena, Stagnaro, Chiara, Valesini, Guido, Mosca, Marta, de Vita, Salvatore, Tincani, Angela, Lapadula, Giovanni, Frediani, Bruno, De Benedetti, Fabrizio, Iannone, Florenzo, Punzi, Leonardo, Salvarani, Carlo, Galeazzi, Mauro, Angotti, Rossella, Messina, Mario, Tosi, Gian Marco, Rigante, Donato, Cantarini, Luca, and for the “Working Group” of Systemic Autoinflammatory Diseases of SIR (Italian Society of Rheumatology)

Published
2018
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3. Early and Late Response and Glucocorticoid-Sparing Effect of Belimumab in Patients with Systemic Lupus Erythematosus with Joint and Skin Manifestations: Results from the Belimumab in Real Life Setting Study—Joint and Skin (BeRLiSS-JS)

Author

Zen, Margherita, primary, Gatto, Mariele, additional, Depascale, Roberto, additional, Regola, Francesca, additional, Fredi, Micaela, additional, Andreoli, Laura, additional, Franceschini, Franco, additional, Urban, Maria Letizia, additional, Emmi, Giacomo, additional, Ceccarelli, Fulvia, additional, Conti, Fabrizio, additional, Bortoluzzi, Alessandra, additional, Govoni, Marcello, additional, Tani, Chiara, additional, Mosca, Marta, additional, Ubiali, Tania, additional, Gerosa, Maria, additional, Bozzolo, Enrica P., additional, Canti, Valentina, additional, Cardinaletti, Paolo, additional, Gabrielli, Armando, additional, Tanti, Giacomo, additional, Gremese, Elisa, additional, De Marchi, Ginevra, additional, De Vita, Salvatore, additional, Fasano, Serena, additional, Ciccia, Francesco, additional, Pazzola, Giulia, additional, Salvarani, Carlo, additional, Negrini, Simone, additional, Di Matteo, Andrea, additional, De Angelis, Rossella, additional, Orsolini, Giovanni, additional, Rossini, Maurizio, additional, Faggioli, Paola, additional, Laria, Antonella, additional, Piga, Matteo, additional, Cauli, Alberto, additional, Scarpato, Salvatore, additional, Rossi, Francesca Wanda, additional, De Paulis, Amato, additional, Brunetta, Enrico, additional, Ceribelli, Angela, additional, Selmi, Carlo, additional, Prete, Marcella, additional, Racanelli, Vito, additional, Vacca, Angelo, additional, Bartoloni, Elena, additional, Gerli, Roberto, additional, Zanatta, Elisabetta, additional, Larosa, Maddalena, additional, Saccon, Francesca, additional, Doria, Andrea, additional, and Iaccarino, Luca, additional

Published
2023
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8. Inborn Errors of Immunity in Children with Autoimmune and Allergic Complaints: A Single Center Experience from Diagnosis to Treatment.

Author

Boz, Valentina, Tesser, Alessandra, Girardelli, Martina, Burlo, Francesca, Pin, Alessia, Severini, Giovanni Maria, De Marchi, Ginevra, Verzegnassi, Federico, Naviglio, Samuele, Tommasini, Alberto, and Valencic, Erica

Subjects
DIAGNOSIS, DELAYED diagnosis, GENETIC disorder diagnosis, LYMPHOCYTE subsets, SYMPTOMS
Abstract

Inborn errors of immunity (IEI) associated with immune dysregulation are not sufficiently addressed in shared recommendation, resulting in delayed diagnosis and high morbidity. The availability of precision medicine for some of these immune defects makes it urgent to evaluate effective strategies to diagnose and treat such defects before the occurrence of severe complications. A diagnosis of an IEI in these patients enabled the use of a more specific treatment in most cases, and these have the potential to prevent further disease progression. We studied immune dysregulation diseases in 30 patients with autoimmune or allergic phenotypes, exploiting data from clinics and immunophenotype, genetic and transcriptome investigations, and 6 of them were diagnosed with a monogenic disorder. Our results confirm that a non-negligible number of children with IEIs may present with signs and symptoms of immune dysregulation and share many features with common multifactorial immune conditions. Reaching a genetic diagnosis becomes more likely in the presence of multiple clinical manifestations, especially when in association with abnormalities of lymphocytes subsets and/or immunoglobulins levels. Moreover, 5 of 6 patients that obtained a diagnosis of monogenic disorder received precision therapy, in four cases with a good or moderate response. [ABSTRACT FROM AUTHOR]

Published
2023
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10. Early disease and low baseline damage predict response to belimumab in patients with systemic lupus erythematosus

Author

Gatto, Mariele, Saccon, Francesca, Zen, Margherita, Regola, Francesca, Fredi, Micaela, Andreoli, Laura, Tincani, Angela, Urban, Maria Letizia, Emmi, Giacomo, Ceccarelli, Fulvia, Conti, Fabrizio, Bortoluzzi, Alessandra, Govoni, Marcello, Tani, Chiara, Mosca, Marta, Ubiali, Tania, Gerosa, Maria, Bozzolo, Enrica, Canti, Valentina, Cardinaletti, Paolo, Gabrielli, Armando, Tanti, Giacomo, Gremese, Elisa, De Marchi, Ginevra, De Vita, Salvatore, Fasano, Serena, Ciccia, Francesco, Pazzola, Giulia, Salvarani, Carlo, Negrini, Simone, Puppo, Francesco, Di Matteo, Andrea, De Angelis, Rossella, Orsolini, Giovanni, Rossini, Maurizio, Faggioli, Paola, Laria, Antonella, Piga, Matteo, Mathieu, Alessandro, Scarpato, Salvatore, Rossi, Francesca W, de Paulis, Amato, Brunetta, Enrico, Ceribelli, Angela, Selmi, Carlo, Prete, Marcella, Racanelli, Vito, Vacca, Angelo, Bartoloni, Elena, Gerli, Roberto, Larosa, Maddalena, Iaccarino, Luca, Doria, Andrea, Gatto, Mariele, Saccon, Francesca, Zen, Margherita, Regola, Francesca, Fredi, Micaela, Andreoli, Laura, Tincani, Angela, Urban, Maria Letizia, Emmi, Giacomo, Ceccarelli, Fulvia, Conti, Fabrizio, Bortoluzzi, Alessandra, Govoni, Marcello, Tani, Chiara, Mosca, Marta, Ubiali, Tania, Gerosa, Maria, Bozzolo, Enrica, Canti, Valentina, Cardinaletti, Paolo, Gabrielli, Armando, Tanti, Giacomo, Gremese, Elisa, De Marchi, Ginevra, De Vita, Salvatore, Fasano, Serena, Ciccia, Francesco, Pazzola, Giulia, Salvarani, Carlo, Negrini, Simone, Puppo, Francesco, Di Matteo, Andrea, De Angelis, Rossella, Orsolini, Giovanni, Rossini, Maurizio, Faggioli, Paola, Laria, Antonella, Piga, Matteo, Mathieu, Alessandro, Scarpato, Salvatore, Rossi, Francesca W, de Paulis, Amato, Brunetta, Enrico, Ceribelli, Angela, Selmi, Carlo, Prete, Marcella, Racanelli, Vito, Vacca, Angelo, Bartoloni, Elena, Gerli, Roberto, Larosa, Maddalena, Iaccarino, Luca, and Doria, Andrea

Subjects
remission, immune system diseases, LDA, SLE, skin and connective tissue diseases, belimumab, damage
Abstract

To investigate predictors of response, remission, low disease activity (LDA), damage and drug discontinuation in patients with systemic lupus erythematosus (SLE) treated with belimumab.

Published
2020

11. Durable renal response and safety with add-on belimumab in patients with lupus nephritis in real-life setting (BeRLiSS-LN). Results from a large, nationwide, multicentric cohort

Author

Gatto, Mariele, primary, Saccon, Francesca, additional, Andreoli, Laura, additional, Bartoloni, Elena, additional, Benvenuti, Francesco, additional, Bortoluzzi, Alessandra, additional, Bozzolo, Enrica, additional, Brunetta, Enrico, additional, Canti, Valentina, additional, Cardinaletti, Paolo, additional, Ceccarelli, Fulvia, additional, Ciccia, Francesco, additional, Conti, Fabrizio, additional, De Marchi, Ginevra, additional, de Paulis, Amato, additional, De Vita, Salvatore, additional, Emmi, Giacomo, additional, Faggioli, Paola, additional, Fasano, Serena, additional, Fredi, Micaela, additional, Gabrielli, Armando, additional, Gasparotto, Michela, additional, Gerli, Roberto, additional, Gerosa, Maria, additional, Govoni, Marcello, additional, Gremese, Elisa, additional, Laria, Antonella, additional, Larosa, Maddalena, additional, Mosca, Marta, additional, Orsolini, Giovanni, additional, Pazzola, Giulia, additional, Petricca, Luca, additional, Ramirez, Giuseppe A., additional, Regola, Francesca, additional, Rossi, Francesca W., additional, Rossini, Maurizio, additional, Salvarani, Carlo, additional, Scarpato, Salvatore, additional, Tani, Chiara, additional, Tincani, Angela, additional, Ubiali, Tania, additional, Urban, Maria Letizia, additional, Zen, Margherita, additional, Doria, Andrea, additional, and Iaccarino, Luca, additional

Published
2021
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13. Psychological Effects of Lockdown Measures for the COVID-19 Outbreak in Patients with Systemic Lupus Erythematosus

Author

Quartuccio, Luca, primary, De Marchi, Ginevra, additional, Azzolina, Danila, additional, Maresio, Elisabetta, additional, Colatutto, Donatella, additional, Binutti, Marco, additional, Monte, Marinella, additional, Gallipoli, Silvia, additional, Zobec, Federica, additional, Lanera, Corrado, additional, Lorenzoni, Giulia, additional, Palese, Alvisa, additional, Silano, Marco, additional, De Vita, Salvatore, additional, and Gregori, Dario, additional

Published
2021
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14. Psychological Effects of Lockdown Measures for the COVID-19 Outbreak in Patients with Systemic Lupus Erythematosus

Author

Quartuccio,Luca, De Marchi,Ginevra, Azzolina,Danila, Maresio,Elisabetta, Colatutto,Donatella, Binutti,Marco, Monte,Marinella, Gallipoli,Silvia, Zobec,Federica, Lanera,Corrado, Lorenzoni,Giulia, Palese,Alvisa, Silano,Marco, De Vita,Salvatore, Gregori,Dario, Quartuccio,Luca, De Marchi,Ginevra, Azzolina,Danila, Maresio,Elisabetta, Colatutto,Donatella, Binutti,Marco, Monte,Marinella, Gallipoli,Silvia, Zobec,Federica, Lanera,Corrado, Lorenzoni,Giulia, Palese,Alvisa, Silano,Marco, De Vita,Salvatore, and Gregori,Dario

Abstract

Luca Quartuccio,1,2,* Ginevra De Marchi,1,* Danila Azzolina,3 Elisabetta Maresio,4 Donatella Colatutto,1,2 Marco Binutti,1,2 Marinella Monte,5 Silvia Gallipoli,6 Federica Zobec,6 Corrado Lanera,3 Giulia Lorenzoni,3 Alvisa Palese,2,7 Marco Silano,8 Salvatore De Vita,1,2 Dario Gregori3 1Rheumatology Clinic, Department of Specialist Medicine, ASUFC, Udine, Italy; 2Department of Medicine (DAME), University of Udine, Udine, Italy; 3Unit of Biostatistics, Epidemiology and Public Health, Department of Cardiology, Thoracic and Vascular Sciences, University of Padova, Padova, Italy; 4Prochild Onlus, Unit of Psychotherapy and Psychology, Trieste, Italy; 5Associazione Malati Reumatici (A.Ma.Re.), Udine, Italy; 6Zeta Research s.r.l., Trieste, Italy; 7School of Nursing, Department of Medicine, University of Udine, Udine, Italy; 8Unit of Human Nutrition and Health, Department of Food Safety Nutrition and Veterinary Public Health, Istituto Superiore di Sanità, Rome, 00161, Italy*These authors contributed equally to this workCorrespondence: Luca QuartuccioDepartment of Medicine (DAME), University of Udine, Udine, ItalyTel +39 0432559352Fax +39 0432559472Email luca.quartuccio@uniud.itObjective: To compare the psychological impact of the lockdown measures contrasting the COVID-19 outbreak between systemic lupus erythematosus (SLE) and general population.Patients and Methods: From July 15th to August 15th 2020, a retrospective survey referring to the period March 9th to May 18th 2020 was administered to SLE patients and the results of the survey, called LEPRE (Lupus Erythematosus PREsto) study, were compared with those from the PRESTO (imPact of quaRantine mEasures againST cOvid19) project, the same survey provided to the general population. Consecutive patients > 18 years old affected by SLE and regularly followed in a single rheumatologic centre were involved. Primary outcome was to compare the scores of the Impact of Events Scale-Revised (IES-R), the General Healt

Published
2021

15. Early Disease and Low Baseline Damage as Predictors of Response to Belimumab in Patients With Systemic Lupus Erythematosus in a Real‐Life Setting

Author

Gatto, Mariele, primary, Saccon, Francesca, additional, Zen, Margherita, additional, Regola, Francesca, additional, Fredi, Micaela, additional, Andreoli, Laura, additional, Tincani, Angela, additional, Urban, Maria Letizia, additional, Emmi, Giacomo, additional, Ceccarelli, Fulvia, additional, Conti, Fabrizio, additional, Bortoluzzi, Alessandra, additional, Govoni, Marcello, additional, Tani, Chiara, additional, Mosca, Marta, additional, Ubiali, Tania, additional, Gerosa, Maria, additional, Bozzolo, Enrica, additional, Canti, Valentina, additional, Cardinaletti, Paolo, additional, Gabrielli, Armando, additional, Tanti, Giacomo, additional, Gremese, Elisa, additional, De Marchi, Ginevra, additional, De Vita, Salvatore, additional, Fasano, Serena, additional, Ciccia, Francesco, additional, Pazzola, Giulia, additional, Salvarani, Carlo, additional, Negrini, Simone, additional, Puppo, Francesco, additional, Di Matteo, Andrea, additional, De Angelis, Rossella, additional, Orsolini, Giovanni, additional, Rossini, Maurizio, additional, Faggioli, Paola, additional, Laria, Antonella, additional, Piga, Matteo, additional, Mathieu, Alessandro, additional, Scarpato, Salvatore, additional, Rossi, Francesca W., additional, Paulis, Amato, additional, Brunetta, Enrico, additional, Ceribelli, Angela, additional, Selmi, Carlo, additional, Prete, Marcella, additional, Racanelli, Vito, additional, Vacca, Angelo, additional, Bartoloni, Elena, additional, Gerli, Roberto, additional, Larosa, Maddalena, additional, Iaccarino, Luca, additional, and Doria, Andrea, additional

Published
2020
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16. Comparison of Early vs. Delayed Anakinra Treatment in Patients With Adult Onset Still's Disease and Effect on Clinical and Laboratory Outcomes

Author

Vitale, Antonio, primary, Cavalli, Giulio, additional, Ruscitti, Piero, additional, Sota, Jurgen, additional, Colafrancesco, Serena, additional, Priori, Roberta, additional, Valesini, Guido, additional, Argolini, Lorenza Maria, additional, Baldissera, Elena, additional, Bartoloni, Elena, additional, Cammelli, Daniele, additional, Canestrari, Giovanni, additional, Cavallaro, Elena, additional, Massaro, Maria Grazia, additional, Cipriani, Paola, additional, De Marchi, Ginevra, additional, De Vita, Salvatore, additional, Emmi, Giacomo, additional, Frassi, Micol, additional, Gerli, Roberto, additional, Gremese, Elisa, additional, Iannone, Florenzo, additional, Fornaro, Marco, additional, Paladini, Anna, additional, Lopalco, Giuseppe, additional, Manna, Raffaele, additional, Mathieu, Alessandro, additional, Montecucco, Carlomaurizio, additional, Mosca, Marta, additional, Piazza, Ilaria, additional, Piga, Matteo, additional, Pontikaki, Irene, additional, Romano, Micol, additional, Rossi, Silvia, additional, Rossini, Maurizio, additional, Silvestri, Elena, additional, Stagnaro, Chiara, additional, Talarico, Rosaria, additional, Frediani, Bruno, additional, Tincani, Angela, additional, Viapiana, Ombretta, additional, Vitiello, Gianfranco, additional, Galozzi, Paola, additional, Sfriso, Paolo, additional, Gaggiano, Carla, additional, Grosso, Salvatore, additional, Rigante, Donato, additional, Dagna, Lorenzo, additional, Giacomelli, Roberto, additional, and Cantarini, Luca, additional

Published
2020
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21. Long-Term Retention Rate of Anakinra in Adult Onset Still’s Disease and Predictive Factors for Treatment Response

Author

Vitale, Antonio, primary, Cavalli, Giulio, additional, Colafrancesco, Serena, additional, Priori, Roberta, additional, Valesini, Guido, additional, Argolini, Lorenza Maria, additional, Baldissera, Elena, additional, Bartoloni, Elena, additional, Cammelli, Daniele, additional, Canestrari, Giovanni, additional, Sota, Jurgen, additional, Cavallaro, Elena, additional, Massaro, Maria Grazia, additional, Ruscitti, Piero, additional, Cipriani, Paola, additional, De Marchi, Ginevra, additional, De Vita, Salvatore, additional, Emmi, Giacomo, additional, Ferraccioli, Gianfranco, additional, Frassi, Micol, additional, Gerli, Roberto, additional, Gremese, Elisa, additional, Iannone, Florenzo, additional, Lapadula, Giovanni, additional, Lopalco, Giuseppe, additional, Manna, Raffaele, additional, Mathieu, Alessandro, additional, Montecucco, Carlomaurizio, additional, Mosca, Marta, additional, Piazza, Ilaria, additional, Piga, Matteo, additional, Pontikaki, Irene, additional, Romano, Micol, additional, Rossi, Silvia, additional, Rossini, Maurizio, additional, Silvestri, Elena, additional, Stagnaro, Chiara, additional, Talarico, Rosaria, additional, Tincani, Angela, additional, Viapiana, Ombretta, additional, Vitiello, Gianfranco, additional, Galozzi, Paola, additional, Sfriso, Paolo, additional, Gaggiano, Carla, additional, Rigante, Donato, additional, Dagna, Lorenzo, additional, Giacomelli, Roberto, additional, and Cantarini, Luca, additional

Published
2019
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23. Clinical predictors of response and discontinuation of belimumab in patients with systemic lupus erythematosus in real life setting. Results of a large, multicentric, nationwide study

Author

Iaccarino, Luca, primary, Andreoli, Laura, additional, Bocci, Elena Bartoloni, additional, Bortoluzzi, Alessandra, additional, Ceccarelli, Fulvia, additional, Conti, Fabrizio, additional, De Angelis, Rossella, additional, De Marchi, Ginevra, additional, De Vita, Salvatore, additional, Di Matteo, Andrea, additional, Emmi, Giacomo, additional, Emmi, Lorenzo, additional, Gatto, Mariele, additional, Gerli, Roberto, additional, Gerosa, Maria, additional, Govoni, Marcello, additional, Larosa, Maddalena, additional, Meroni, Pier Luigi, additional, Mosca, Marta, additional, Pazzola, Giulia, additional, Reggia, Rossella, additional, Saccon, Francesca, additional, Salvarani, Carlo, additional, Tani, Chiara, additional, Zen, Margherita, additional, Frigo, Anna Chiara, additional, Tincani, Angela, additional, and Doria, Andrea, additional

Published
2018
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24. Response to interleukin-1 inhibitors in 140 Italian patients with adult-onset still's disease: A multicentre retrospective observational study

Author

Colafrancesco, Serena, Priori, Roberta, Valesini, Guido, Argolini, Lorenza, Baldissera, Elena, Bartoloni, Elena, Cammelli, Daniele, Canestrari, Giovanni Battista, Cantarini, Luca, Cavallaro, Elena, Cavalli, Giulio, Cerrito, Lucia, Cipriani, Paola, Dagna, Lorenzo, De Marchi, Ginevra, De Vita, Salvatore, Emmi, Giacomo, Ferraccioli, Gianfranco, Frassi, Micol, Galeazzi, Mauro, Gerli, Roberto, Giacomelli, Roberto, Gremese, Elisa, Iannone, Florenzo, Lapadula, Giovanni, Lopalco, Giuseppe, Manna, Raffaele, Mathieu, Alessandro, Montecucco, Carlomaurizio, Mosca, Marta, Piazza, Ilaria, Piga, Matteo, Pontikaki, Irene, Romano, Micol, Rossi, Silvia, Rossini, Maurizio, Ruscitti, Piero, Silvestri, Elena, Stagnaro, Chiara, Talarico, Rosaria, Tincani, Angela, Viapiana, Ombretta, Vitiello, Gianfranco, Fabris, Francesca, Bindoli, Sara, Punzi, Leonardo, Galozzi, Paola, Sfriso, Paolo, Canestrari, Giovanni, Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), Gremese, Elisa (ORCID:0000-0002-2248-1058), Manna, Raffaele (ORCID:0000-0003-1560-3907), Colafrancesco, Serena, Priori, Roberta, Valesini, Guido, Argolini, Lorenza, Baldissera, Elena, Bartoloni, Elena, Cammelli, Daniele, Canestrari, Giovanni Battista, Cantarini, Luca, Cavallaro, Elena, Cavalli, Giulio, Cerrito, Lucia, Cipriani, Paola, Dagna, Lorenzo, De Marchi, Ginevra, De Vita, Salvatore, Emmi, Giacomo, Ferraccioli, Gianfranco, Frassi, Micol, Galeazzi, Mauro, Gerli, Roberto, Giacomelli, Roberto, Gremese, Elisa, Iannone, Florenzo, Lapadula, Giovanni, Lopalco, Giuseppe, Manna, Raffaele, Mathieu, Alessandro, Montecucco, Carlomaurizio, Mosca, Marta, Piazza, Ilaria, Piga, Matteo, Pontikaki, Irene, Romano, Micol, Rossi, Silvia, Rossini, Maurizio, Ruscitti, Piero, Silvestri, Elena, Stagnaro, Chiara, Talarico, Rosaria, Tincani, Angela, Viapiana, Ombretta, Vitiello, Gianfranco, Fabris, Francesca, Bindoli, Sara, Punzi, Leonardo, Galozzi, Paola, Sfriso, Paolo, Canestrari, Giovanni, Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), Gremese, Elisa (ORCID:0000-0002-2248-1058), and Manna, Raffaele (ORCID:0000-0003-1560-3907)

Abstract

Background: Interleukin (IL)-1 plays a crucial role in the pathogenesis of Adult onset Still's disease (AOSD). Objectives: To evaluate the efficacy and safety of anakinra (ANA) and canakinumab (CAN) in a large group of AOSD patients. Methods: Data on clinical, serological features, and concomitant treatments were retrospectively collected at baseline and after 3, 6, and 12 months from AOSD patients (Yamaguchi criteria) referred by 18 Italian centers. Pouchot's score was used to evaluate disease severity. Results: One hundred forty patients were treated with ANA; 4 were subsequently switched to CAN after ANA failure. The systemic pattern of AOSD was identified in 104 (74.2%) of the ANA-treated and in 3 (75%) of the CAN-treated groups; the chronic-articular type of AOSD was identified in 48 (25.8%) of the ANA-treated and in 1 (25%) of the CAN-treated groups. Methotrexate (MTX) was the most frequent disease modifying anti-rheumatic drug (DMARD) used before beginning ANA or CAN [91/140 (75.8%), 2/4 (50%), respectively]. As a second-line biologic DMARD therapy in 29/140 (20.7%) of the patients, ANA was found effective in improving all clinical and serological manifestations (p < 0.0001), and Pouchot's score was found to be significantly reduced at all time points (p < 0.0001). No differences in treatment response were identified in the ANA-group when the patients were stratified according to age, sex, disease pattern or mono/combination therapy profile. ANA primary and secondary inefficacy at the 12-month time point was 15/140 (10.7%) and 11/140 (7.8%), respectively. Adverse events (AEs) [mainly represented by in situ (28/47, 59.5%) or diffuse (12/47, 25.5%) skin reactions and infections (7/47, 14.8%)] were the main causes for discontinuation. Pouchot's score and clinical and serological features were significantly ameliorated at all time points (p < 0.0001) in the CAN-group, and no AEs were registered during CAN therapy. Treatment was suspended for loss of effi

Published
2017

25. A Snapshot on the On-Label and Off-Label Use of the Interleukin-1 Inhibitors in Italy among Rheumatologists and Pediatric Rheumatologists: A Nationwide Multi-Center Retrospective Observational Study

Author

Vitale, Antonio, primary, Insalaco, Antonella, additional, Sfriso, Paolo, additional, Lopalco, Giuseppe, additional, Emmi, Giacomo, additional, Cattalini, Marco, additional, Manna, Raffaele, additional, Cimaz, Rolando, additional, Priori, Roberta, additional, Talarico, Rosaria, additional, Gentileschi, Stefano, additional, de Marchi, Ginevra, additional, Frassi, Micol, additional, Gallizzi, Romina, additional, Soriano, Alessandra, additional, Alessio, Maria, additional, Cammelli, Daniele, additional, Maggio, Maria C., additional, Marcolongo, Renzo, additional, La Torre, Francesco, additional, Fabiani, Claudia, additional, Colafrancesco, Serena, additional, Ricci, Francesca, additional, Galozzi, Paola, additional, Viapiana, Ombretta, additional, Verrecchia, Elena, additional, Pardeo, Manuela, additional, Cerrito, Lucia, additional, Cavallaro, Elena, additional, Olivieri, Alma N., additional, Paolazzi, Giuseppe, additional, Vitiello, Gianfranco, additional, Maier, Armin, additional, Silvestri, Elena, additional, Stagnaro, Chiara, additional, Valesini, Guido, additional, Mosca, Marta, additional, de Vita, Salvatore, additional, Tincani, Angela, additional, Lapadula, Giovanni, additional, Frediani, Bruno, additional, De Benedetti, Fabrizio, additional, Iannone, Florenzo, additional, Punzi, Leonardo, additional, Salvarani, Carlo, additional, Galeazzi, Mauro, additional, Rigante, Donato, additional, and Cantarini, Luca, additional

Published
2016
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27. Controversies on Rituximab Therapy in Sjögren Syndrome-Associated Lymphoproliferation

Author

Quartuccio, Luca, Fabris, Martina, Salvin, Sara, Maset, Marta, De Marchi, Ginevra, and De Vita, Salvatore

Subjects
Article Subject, immune system diseases, hemic and lymphatic diseases
Abstract

Sjögren's syndrome (SS) is a systemic autoimmune disease characterized by chronic inflammation of salivary and lachrymal glands, and frequently accompanied by systemic symptoms. A subgroup of SS patients develops malignant B cell non-Hodgkin's lymphoma (NHL), usually of the mucosa-associated lymphoid tissue (MALT) type and very often located in the major salivary glands. Currently, there is a lack of evidence-based intervention therapy which may influence SS-related chronic inflammation and lymphoproliferation. B cells are involved in the pathogenesis of SS, and B cell downregulation may lead to a decrease of disease activity. Rituximab (RTX), a chimeric monoclonal antibody targeting the CD20 antigen on the B cell surface, has been successfully investigated in other autoimmune diseases, such as rheumatoid arthritis, systemic lupus erythematosus, ANCA-associated vasculitis, and mixed cryoglobulinemic syndrome. Preliminary experiences of RTX therapy in SS patients with or without a lymphoproliferative disorder suggest that SS patients with more residual exocrine gland function might better benefit from RTX. Efficacy of RTX in SS-associated B-cell lymphoma, mainly in low-grade salivary gland lymphomas, remains an open issue.

Published
2009
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28. Biomarkers of lymphoma in Sjögren’s syndrome and evaluation of the lymphoma risk in prelymphomatous conditions: Results of a multicenter study

Author

Quartuccio, Luca, primary, Isola, Miriam, additional, Baldini, Chiara, additional, Priori, Roberta, additional, Bartoloni Bocci, Elena, additional, Carubbi, Francesco, additional, Maset, Marta, additional, Gregoraci, Giorgia, additional, Della Mea, Vincenzo, additional, Salvin, Sara, additional, De Marchi, Ginevra, additional, Luciano, Nicoletta, additional, Colafrancesco, Serena, additional, Alunno, Alessia, additional, Giacomelli, Roberto, additional, Gerli, Roberto, additional, Valesini, Guido, additional, Bombardieri, Stefano, additional, and De Vita, Salvatore, additional

Published
2014
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29. Preliminary classification criteria for the cryoglobulinaemic vasculitis

Author

De Vita, Salvatore, Soldano, Franca, Isola, Miriam, Monti, Giuseppe, Gabrielli, Armando, Tzioufas, Athanasios, Ferri, Clodoveo, Ferraccioli, Gianfranco Franco, Quartuccio, Luca, Corazza, Laura, De Marchi, Ginevra, Casals, Manuel Ramos, Voulgarelis, Michael, Lenzi, Marco, Saccardo, Francesco, Fraticelli, Paolo, Mascia, Maria Teresa, Sansonno, Domenico, Cacoub, Patrice, Tomsic, Matjia, Tavoni, Antonio Gaetano A., Pietrogrande, Maurizio, Zignego, Anna Linda, Scarpato, Salvatore, Mazzaro, Cesare, Pioltelli, Pietro, Steinfeld, Serge, Lamprecht, Peter, Bombardieri, Stefano, Galli, Massimo, De Vita, Salvatore, Soldano, Franca, Isola, Miriam, Monti, Giuseppe, Gabrielli, Armando, Tzioufas, Athanasios, Ferri, Clodoveo, Ferraccioli, Gianfranco Franco, Quartuccio, Luca, Corazza, Laura, De Marchi, Ginevra, Casals, Manuel Ramos, Voulgarelis, Michael, Lenzi, Marco, Saccardo, Francesco, Fraticelli, Paolo, Mascia, Maria Teresa, Sansonno, Domenico, Cacoub, Patrice, Tomsic, Matjia, Tavoni, Antonio Gaetano A., Pietrogrande, Maurizio, Zignego, Anna Linda, Scarpato, Salvatore, Mazzaro, Cesare, Pioltelli, Pietro, Steinfeld, Serge, Lamprecht, Peter, Bombardieri, Stefano, and Galli, Massimo

Abstract

Background: To develop preliminary classification criteria for the cryoglobulinaemic syndrome or cryoglobulinaemic vasculitis (CV). Methods: Study part I developed a questionnaire for CV to be included in the formal, second part (study part II). Positivity of serum cryoglobulins was defined by experts as an essential condition for CV classification. In study part II, a core set of classification items (questionnaire, clinical and laboratory items, as agreed) was tested in three groups of patients and controls - that is, group A (new patients with the CV), group B (controls with serum cryoglobulins but lacking CV) and group C (controls without serum cryoglobulins but with features which can be observed in CV). Results: In study part I (188 cases, 284 controls), a positive response to at least two of three selected questions showed a sensitivity of 81.9% and a specificity of 83.5% for CV. This questionnaire was employed and validated in study part II, which included 272 patients in group A and 228 controls in group B. The final classification criteria for CV, by pooling data from group A and group B, required the positivity of questionnaire plus clinical, questionnaire plus laboratory, or clinical plus laboratory items, or all the three, providing a sensitivity of 88.5% and a specificity of 93.6% for CV. By comparing data in group A versus group C (425 controls), the same classification criteria showed a sensitivity 88.5% and a specificity 97.0% for CV. Conclusion: Classification criteria for CV were developed, and now need validation., SCOPUS: ar.j, info:eu-repo/semantics/published

Published
2011

30. Characterization of B cell lymphoma in patients with Sjögren's syndrome and hepatitis C virus infection

Author

Ramos‐Casals, Manuel, Civita, Luca la, de Vita, Salvatore, Solans, Roser, Luppi, Mario, Medina, Francisco, Caramaschi, Paola, Fadda, Patrizia, de Marchi, Ginevra, Lopez‐Guillermo, Armando, and Font, Josep

Abstract

To characterize the clinical and immunologic patterns of expression, response to therapy, and outcome of patients with Sjögren's syndrome (SS) and associated hepatitis C virus (HCV) infection who developed B cell lymphoma.Various international reference centers constituted a multicenter study group with the purpose of creating a registry of patients with SS‐HCV who developed B cell lymphoma. A protocol form was used to record the main characteristics of SS, chronic HCV infection, and B cell lymphoma.Twenty‐five patients with SS‐HCV with B cell lymphoma were included in the registry. There were 22 (88%) women and 3 (12%) men (mean age 55, 58, and 61 years at SS, HCV infection, and lymphoma diagnosis, respectively). The main extraglandular SS manifestations were cutaneous vasculitis in 15 (60%) patients and peripheral neuropathy in 12 (48%); the main immunologic features were positive rheumatoid factor (RF) in 24 (96%) and type II cryoglobulins in 20 (80%). The main histologic subtypes were mucosa‐associated lymphoid tissue (MALT) lymphoma in 11 (44%) patients, diffuse large B cell lymphoma in 6 (24%), and follicular center cell lymphoma in 6 (24%). Fifteen (60%) patients had an extranodal primary location, most frequently in the parotid gland (5 patients), liver (4 patients), and stomach (4 patients). Twelve (52%) of 23 patients died after a median followup from the time of lymphoma diagnosis of 4 years, with lymphoma progression being the most frequent cause of death. Survival differed significantly between the main types of B cell lymphoma.Patients with SS‐HCV and B cell lymphoma are clinically characterized by a high frequency of parotid enlargement and vasculitis, an immunologic pattern overwhelmingly dominated by the presence of RF and mixed type II cryoglobulins, a predominance of MALT lymphomas, and an elevated frequency of primary extranodal involvement in organs in which HCV replicates (exocrine glands, liver, and stomach).

Published
2007
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31. Early and Late Response and Glucocorticoid-Sparing Effect of Belimumab in Patients with Systemic Lupus Erythematosus with Joint and Skin Manifestations: Results from the Belimumab in Real Life Setting Study—Joint and Skin (BeRLiSS-JS)

Author

Margherita Zen, Mariele Gatto, Roberto Depascale, Francesca Regola, Micaela Fredi, Laura Andreoli, Franco Franceschini, Maria Letizia Urban, Giacomo Emmi, Fulvia Ceccarelli, Fabrizio Conti, Alessandra Bortoluzzi, Marcello Govoni, Chiara Tani, Marta Mosca, Tania Ubiali, Maria Gerosa, Enrica P. Bozzolo, Valentina Canti, Paolo Cardinaletti, Armando Gabrielli, Giacomo Tanti, Elisa Gremese, Ginevra De Marchi, Salvatore De Vita, Serena Fasano, Francesco Ciccia, Giulia Pazzola, Carlo Salvarani, Simone Negrini, Andrea Di Matteo, Rossella De Angelis, Giovanni Orsolini, Maurizio Rossini, Paola Faggioli, Antonella Laria, Matteo Piga, Alberto Cauli, Salvatore Scarpato, Francesca Wanda Rossi, Amato De Paulis, Enrico Brunetta, Angela Ceribelli, Carlo Selmi, Marcella Prete, Vito Racanelli, Angelo Vacca, Elena Bartoloni, Roberto Gerli, Elisabetta Zanatta, Maddalena Larosa, Francesca Saccon, Andrea Doria, Luca Iaccarino, Zen, Margherita, Gatto, Mariele, Depascale, Roberto, Regola, Francesca, Fredi, Micaela, Andreoli, Laura, Franceschini, Franco, Letizia Urban, Maria, Emmi, Giacomo, Ceccarelli, Fulvia, Conti, Fabrizio, Bortoluzzi, Alessandra, Govoni, Marcello, Tani, Chiara, Mosca, Marta, Ubiali, Tania, Gerosa, Maria, Bozzolo, Enrica P., Canti, Valentina, Cardinaletti, Paolo, Gabrielli, Armando, Tanti, Giacomo, Gremese, Elisa, De Marchi, Ginevra, De Vita, Salvatore, Fasano, Serena, Ciccia, Francesco, Pazzola, Giulia, Salvarani, Carlo, Negrini, Simone, Di Matteo, Andrea, DE ANGELIS, Rossella, Orsolini, Giovanni, Rossini, Maurizio, Faggioli, Paola, Laria, Antonella, Piga, Matteo, Cauli, Alberto, Scarpato, Salvatore, Wanda Rossi, Francesca, De Paulis, Amato, Brunetta, Enrico, Ceribelli, Angela, Selmi, Carlo, Prete, Marcella, Racanelli, Vito, Vacca, Angelo, Bartoloni, Elena, Gerli, Roberto, Zanatta, Elisabetta, Larosa, Maddalena, Saccon, Francesca, Doria, Andrea, and Iaccarino, Luca

Subjects
disease activity score (DAS)-28, low disease activity, remission, systemic lupus erythematosus, belimumab, Cutaneous LE Area and Severity Index (CLASI), Medicine (miscellaneous)
Abstract

Aim. To assess the efficacy of belimumab in joint and skin manifestations in a nationwide cohort of patients with SLE. Methods. All patients with skin and joint involvement enrolled in the BeRLiSS cohort were considered. Belimumab (intravenous, 10 mg/kg) effectiveness in joint and skin manifestations was assessed by DAS28 and CLASI, respectively. Attainment and predictors of DAS28 remission (

Published
2023

32. Safety profile of the interleukin-1 inhibitors anakinra and canakinumab in real life clinical practice: a nationwide multicenter retrospective observational study

Author

Sota, J, Vitale, A, Insalaco, A, Sfriso, P, Lopalco, G, Emmi, G, Cattalini, M, Manna, R, Cimaz, R, Priori, R, Talarico, R, de Marchi, G, Frassi, M, Gallizzi, R, Soriano, A, Alessio, M, Cammelli, D, Maggio, Mc, Gentileschi, S, Marcolongo, R, La Torre, F, Fabiani, C, Colafrancesco, S, Ricci, F, Galozzi, P, Viapiana, O, Verrecchia, E, Pardeo, M, Cerrito, L, Cavallaro, E, Olivieri, An, Paolazzi, G, Vitiello, G, Maier, A, Silvestri, E, Stagnaro, C, Valesini, G, Mosca, M, de Vita, S, Tincani, A, Lapadula, G, Frediani, B, De Benedetti, F, Iannone, F, Punzi, L, Salvarani, C, Galeazzi, M, Angotti, R, Messina, M, Tosi, Gm, Rigante, D, Cantarini, L, 'Working Group' of Systemic Autoinflammatory Diseases of SIR (Italian Society of, Rheumatology)., Sota, J., Vitale, A., Insalaco, A., Sfriso, P., Lopalco, G., Emmi, G., Cattalini, M., Manna, R., Cimaz, R., Priori, R., Talarico, R., de Marchi, G., Frassi, M., Gallizzi, R., Soriano, A., Alessio, M., Cammelli, D., Maggio, M. C., Gentileschi, S., Marcolongo, R., La Torre, F., Fabiani, C., Colafrancesco, S., Ricci, F., Galozzi, P., Viapiana, O., Verrecchia, E., Pardeo, M., Cerrito, L., Cavallaro, E., Olivieri, A. N., Paolazzi, G., Vitiello, G., Maier, A., Silvestri, E., Stagnaro, C., Valesini, G., Mosca, M., de Vita, S., Tincani, A., Lapadula, G., Frediani, B., De Benedetti, F., Iannone, F., Punzi, L., Salvarani, C., Galeazzi, M., Angotti, R., Messina, M., Tosi, G. M., Rigante, D., Cantarini, L., Sota, Jurgen, Vitale, Antonio, Insalaco, Antonella, Sfriso, Paolo, Lopalco, Giuseppe, Emmi, Giacomo, Cattalini, Marco, Manna, Raffaele, Cimaz, Rolando, Priori, Roberta, Talarico, Rosaria, de Marchi, Ginevra, Frassi, Micol, Gallizzi, Romina, Soriano, Alessandra, Alessio, Maria, Cammelli, Daniele, Maggio, Maria Cristina, Gentileschi, Stefano, Marcolongo, Renzo, La Torre, Francesco, Fabiani, Claudia, Colafrancesco, Serena, Ricci, Francesca, Galozzi, Paola, Viapiana, Ombretta, Verrecchia, Elena, Pardeo, Manuela, Cerrito, Lucia, Cavallaro, Elena, Olivieri, Alma Nunzia, Paolazzi, Giuseppe, Vitiello, Gianfranco, Maier, Armin, Silvestri, Elena, Stagnaro, Chiara, Valesini, Guido, Mosca, Marta, de Vita, Salvatore, Tincani, Angela, Lapadula, Giovanni, Frediani, Bruno, De Benedetti, Fabrizio, Iannone, Florenzo, Punzi, Leonardo, Salvarani, Carlo, Galeazzi, Mauro, Angotti, Rossella, Messina, Mario, Tosi, Gian Marco, Rigante, Donato, and Cantarini, Luca

Subjects
Anakinra, Autoinflammatory disorders, Canakinumab, Interleukin-1, Safety profile, 0301 basic medicine, Male, Settore MED/16 - REUMATOLOGIA, 0302 clinical medicine, Retrospective Studie, Rheumatology, Child, Antibodies, Monoclonal, General Medicine, Middle Aged, Treatment Outcome, Autoinflammation, Female, Cohort study, medicine.drug, Human, Adult, medicine.medical_specialty, Adolescent, Logistic Model, Neutropenia, Antibodies, Monoclonal, Humanized, Autoimmune Disease, Autoimmune Diseases, 03 medical and health sciences, Young Adult, Internal medicine, Injection site reaction, medicine, Humans, Anakinra, Autoinflammatory disorders, Canakinumab, Interleukin-1, Safety profile, Adolescent, Adult, Antibodies, Monoclonal, Antibodies, Monoclonal, Humanized, Autoimmune Diseases, Child, Female, Humans, Interleukin 1 Receptor Antagonist Protein, Logistic Models, Male, Middle Aged, Retrospective Studies, Treatment Outcome, Young Adult, Adverse effect, Retrospective Studies, 030203 arthritis & rheumatology, business.industry, Retrospective cohort study, medicine.disease, Interleukin 1 Receptor Antagonist Protein, Logistic Models, 030104 developmental biology, Autoinflammatory disorder, Observational study, business
Abstract

A few studies have reported the safety profile of interleukin (IL)-1 blockers from real life. The aim of this study is to describe anakinra (ANA) and canakinumab (CAN) safety profile in children and adults, based on data from a real-life setting. Demographic, clinical, and therapeutic data from patients treated with ANA and CAN were retrospectively collected and analyzed. Four hundred and seventy five patients were enrolled; ANA and CAN were prescribed in 421 and 105 treatment courses, respectively. During a mean follow-up of 24.39 ± 27.04 months, 89 adverse events (AE) were recorded; 13 (14.61%) were classified as serious AE (sAE). The overall estimated rate of AE and sAE was 8.4 per 100 patients/year. Safety concerns were more frequent among patients aged ≥ 65 years compared with patients < 16 years (p = 0.002). No differences were detected in the frequency of safety concerns between monotherapy and combination therapy with immunosuppressants (p = 0.055), but a significant difference was observed when injection site reactions were excluded from AE (p = 0.01). No differences were identified in relation to gender (p = 0.462), different lines of biologic therapy (p = 0.775), and different dosages (p = 0.70 ANA; p = 0.39 CAN). The overall drug retention rate was significantly different according to the occurrence of safety concerns (p value < 0.0001); distinguishing between ANA and CAN, significance was maintained only for ANA (p < 0.0001 ANA; p > 0.05 CAN). Treatment duration was the only variable associated with onset of AE (OR = 0.399 [C.I. 0.250–0.638], p = 0.0001). ANA and CAN have shown an excellent safety profile; the risk for AE and sAE tends to decrease over time from the start of IL-1 inhibition.

Published
2018

33. Response to Interleukin-1 Inhibitors in 140 Italian Patients with Adult-Onset Still's Disease: A Multicentre Retrospective Observational Study

Author

Serena Colafrancesco, Roberta Priori, Guido Valesini, Lorenza Argolini, Elena Baldissera, Elena Bartoloni, Daniele Cammelli, Giovanni Canestrari, Luca Cantarini, Elena Cavallaro, Giulio Cavalli, Lucia Cerrito, Paola Cipriani, Lorenzo Dagna, Ginevra De Marchi, Salvatore De Vita, Giacomo Emmi, Gianfranco Ferraccioli, Micol Frassi, Mauro Galeazzi, Roberto Gerli, Roberto Giacomelli, Elisa Gremese, Florenzo Iannone, Giovanni Lapadula, Giuseppe Lopalco, Raffaele Manna, Alessandro Mathieu, Carlomaurizio Montecucco, Marta Mosca, Ilaria Piazza, Matteo Piga, Irene Pontikaki, Micol Romano, Silvia Rossi, Maurizio Rossini, Piero Ruscitti, Elena Silvestri, Chiara Stagnaro, Rosaria Talarico, Angela Tincani, Ombretta Viapiana, Gianfranco Vitiello, Francesca Fabris, Sara Bindoli, Leonardo Punzi, Paola Galozzi, Paolo Sfriso, Colafrancesco, Serena, Priori, Roberta, Valesini, Guido, Argolini, Lorenza, Baldissera, Elena, Bartoloni, Elena, Cammelli, Daniele, Canestrari, Giovanni, Cantarini, Luca, Cavallaro, Elena, Cavalli, Giulio, Cerrito, Lucia, Cipriani, Paola, Dagna, Lorenzo, De Marchi, Ginevra, De Vita, Salvatore, Emmi, Giacomo, Ferraccioli, Gianfranco, Frassi, Micol, Galeazzi, Mauro, Gerli, Roberto, Giacomelli, Roberto, Gremese, Elisa, Iannone, Florenzo, Lapadula, Giovanni, Lopalco, Giuseppe, Manna, Raffaele, Mathieu, Alessandro, Montecucco, Carlomaurizio, Mosca, Marta, Piazza, Ilaria, Piga, Matteo, Pontikaki, Irene, Romano, Micol, Rossi, Silvia, Rossini, Maurizio, Ruscitti, Piero, Silvestri, Elena, Stagnaro, Chiara, Talarico, Rosaria, Tincani, Angela, Viapiana, Ombretta, Vitiello, Gianfranco, Fabris, Francesca, Bindoli, Sara, Punzi, Leonardo, Galozzi, Paola, and Sfriso, Paolo

Subjects
musculoskeletal diseases, 0301 basic medicine, medicine.medical_specialty, Combination therapy, Canakinumab, Interleukin (IL)-1, Adult-onset Still's disease, canakinumab, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, Pharmacology (medical), Adverse effect, Original Research, Adult-onset Still’s disease, 030203 arthritis & rheumatology, Pharmacology, Anakinra, treatment, business.industry, Treatment, Settore MED/09 - MEDICINA INTERNA, lcsh:RM1-950, Retrospective cohort study, Surgery, Discontinuation, interleukin (IL)-1, anakinra, lcsh:Therapeutics. Pharmacology, 030104 developmental biology, Concomitant, Methotrexate, business, medicine.drug
Abstract

Background: Interleukin (IL)-1 plays a crucial role in the pathogenesis of Adult onset Still’s disease (AOSD).Objectives: To evaluate the efficacy and safety of anakinra (ANA) and canakinumab (CAN) in a large group of AOSD patients.Methods: Data on clinical, serological features, and concomitant treatments were retrospectively collected at baseline and after 3, 6, and 12 months from AOSD patients (Yamaguchi criteria) referred by 18 Italian centers. Pouchot’s score was used to evaluate disease severity.Results: One hundred forty patients were treated with ANA; 4 were subsequently switched to CAN after ANA failure. The systemic pattern of AOSD was identified in 104 (74.2%) of the ANA-treated and in 3 (75%) of the CAN-treated groups; the chronic-articular type of AOSD was identified in 48 (25.8%) of the ANA-treated and in 1 (25%) of the CAN-treated groups. Methotrexate (MTX) was the most frequent disease modifying anti-rheumatic drug (DMARD) used before beginning ANA or CAN [91/140 (75.8%), 2/4 (50%), respectively]. As a second-line biologic DMARD therapy in 29/140 (20.7%) of the patients, ANA was found effective in improving all clinical and serological manifestations (p < 0.0001), and Pouchot’s score was found to be significantly reduced at all time points (p < 0.0001). No differences in treatment response were identified in the ANA-group when the patients were stratified according to age, sex, disease pattern or mono/combination therapy profile. ANA primary and secondary inefficacy at the 12-month time point was 15/140 (10.7%) and 11/140 (7.8%), respectively. Adverse events (AEs) [mainly represented by in situ (28/47, 59.5%) or diffuse (12/47, 25.5%) skin reactions and infections (7/47, 14.8%)] were the main causes for discontinuation. Pouchot’s score and clinical and serological features were significantly ameliorated at all time points (p < 0.0001) in the CAN-group, and no AEs were registered during CAN therapy. Treatment was suspended for loss of efficacy only in one case (1/4, 25%).Conclusion: This is the largest retrospective observational study evaluating the efficacy and safety of IL-1 inhibitors in AOSD patients. A good response was noted at 3 months after therapy onset in both the ANA- and CAN-groups. Skin reaction may nevertheless represent a non-negligible AE during ANA treatment.

Published
2017

34. Therapeutic strategies and outcomes in neuropsychiatric systemic lupus erythematosus: an international multicentre retrospective study.

Author

Bortoluzzi A, Fanouriakis A, Silvagni E, Appenzeller S, Carli L, Carrara G, Cauli A, Conti F, Costallat LTL, De Marchi G, Doria A, Fredi M, Franceschini F, Garaffoni C, Hanly JG, Mosca M, Murphy E, Piga M, Quartuccio L, Scirè CA, Tomietto P, Truglia S, Zanetti A, Zen M, Bertsias G, and Govoni M

Subjects
Humans, Retrospective Studies, Female, Male, Adult, Treatment Outcome, Middle Aged, Adrenal Cortex Hormones therapeutic use, Severity of Illness Index, Lupus Vasculitis, Central Nervous System diagnosis, Lupus Vasculitis, Central Nervous System therapy, Immunosuppressive Agents therapeutic use
Abstract

Objectives: The management of neuropsychiatric systemic lupus erythematosus (NPSLE) poses considerable challenges due to limited clinical trials. Therapeutic decisions are customized based on suspected pathogenic mechanisms and symptoms severity. This study aimed to investigate therapeutic strategies and disease outcome for patients with NPSLE experiencing their first neuropsychiatric (NP) manifestation., Methods: This retrospective cohort study defined NP events according to the American College of Rheumatology case definition, categorizing them into three clusters: central/diffuse, central/focal and peripheral. Clinical judgment and a validated attribution algorithm were used for NP event attribution. Data included demographic variables, SLE disease activity index, cumulative organ damage, and NP manifestation treatments. The clinical outcome of all NP events was determined by a physician seven-point Likert scale. Predictors of clinical improvement/resolution were investigated in a multivariable logistic regression analysis., Results: The analysis included 350 events. Immunosuppressants and corticosteroids were more frequently initiated/escalated for SLE-attributed central diffuse or focal NP manifestations. At 12 months of follow-up, 64% of patients showed a clinical improvement in NP manifestations. Focal central events and SLE-attributed manifestations correlated with higher rates of clinical improvement. Patients with NP manifestations attributed to SLE according to clinical judgment and treated with immunosuppressants had a significantly higher probability of achieving clinical response (OR 2.55, 95%CI 1.06-6.41, P = 0.04). Age at diagnosis and focal central events emerged as additional response predictors., Conclusion: NP manifestations attributed to SLE by clinical judgment and treated with immunosuppressants demonstrated improved 12-month outcomes. This underscores the importance of accurate attribution and timely diagnosis of NPSLE., (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.)

Published
2024
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36. Efficacy of belimumab for the long-term maintenance therapy of thrombocytopenia in systemic lupus erythematosus.

Author

De Marchi G, Quartuccio L, Bond M, and De Vita S

Subjects
Adult, B-Cell Activating Factor antagonists & inhibitors, Female, Humans, Immunoglobulins, Intravenous therapeutic use, Lupus Erythematosus, Systemic complications, Thrombocytopenia etiology, Antibodies, Monoclonal, Humanized therapeutic use, Immunosuppressive Agents therapeutic use, Lupus Erythematosus, Systemic drug therapy, Thrombocytopenia drug therapy
Published
2017

37. The relevance of cluster analyses to stratify systemic lupus erythematosus: increased mortality with heavier treatment.

Author

De Marchi G, Quartuccio L, Zuliani F, Bond M, and De Vita S

Subjects
Adult, Autoantibodies blood, Biomarkers blood, Cluster Analysis, Disease Progression, Drug Therapy, Combination, Female, Humans, Lupus Erythematosus, Systemic diagnosis, Lupus Erythematosus, Systemic immunology, Male, Middle Aged, Risk Factors, Treatment Outcome, Young Adult, Immunosuppressive Agents therapeutic use, Lupus Erythematosus, Systemic drug therapy, Lupus Erythematosus, Systemic mortality
Published
2017

38. Systemic connective tissue disease complicated by Castleman's disease: report of a case and review of the literature.

Author

De Marchi G, De Vita S, Fabris M, Scott CA, and Ferraccioli G

Subjects
Adult, Autoimmune Diseases etiology, B-Cell Activating Factor, B-Lymphocytes pathology, CD5 Antigens, Clone Cells, Connective Tissue Diseases immunology, Female, Humans, Lymphoproliferative Disorders etiology, Membrane Proteins physiology, Tumor Necrosis Factor-alpha physiology, Castleman Disease etiology, Connective Tissue Diseases complications
Abstract

Castleman's disease is an atypical lymphoproliferative disorder characterized by the prevalence of B CD5-positive cells in the marginal zone. Autoimmune manifestations have often been reported, but the association of Castleman's disease with systemic autoimmune syndromes has been rarely described. However, many authors stress the difficulties in distinguishing between connective tissue disease and Castleman's disease in most cases. To clarify this issue, we describe a patient and review the literature reports of all cases of Castleman's disease associated with a connective tissue disease. A 19-year old woman presented with autoimmune thyroiditis and polymyositis. Seven years after the onset she developed a systemic inflammatory flare and a burst of autoimmunity, followed by generalized lymphoadenopathy. A mediastinal lymph node biopsy led to the diagnosis of Castleman disease of mixed type. Chemotherapy was given, with rapid response of the lymphoproliferative disorder but persistence of the underlying autoimmune disorder. The plasma concentration of B-lymphocyte stimulator (BLyS) was high (13.3 ng/mL) at the diagnosis of Castleman's disease. It fell dramatically after chemotherapy (4.97 ng/mL), even though it remained just above the mean BLyS value found in healthy blood donors (3.37+/-0.78 ng/mL).Castleman's disease can present autoimmune traits. In our patient, Castleman's disease complicated the course of a connective tissue disorder several years after the onset. We hypothesize that chronic stimulation of B-cell clones, particularly CD5+, by BLyS could favor the development of both autoimmune diseases and a broad range of lymphoproliferative disorders (such as Castleman's disease). This is the first report of increased BLyS levels in a patient with Castleman's disease, supporting a possible pathogenetic role of BLyS in the development of an autoimmune disorder and of a B lymphoproliferative disorder years later.

Published
2004

39. True primary Sjögren's syndrome in a subset of patients with hepatitis C infection: a model linking chronic infection to chronic sialadenitis.

Author

De Vita S, Damato R, De Marchi G, Sacco S, and Ferraccioli G

Subjects
Aged, Aged, 80 and over, Antibodies, Antinuclear analysis, Biopsy, Case-Control Studies, Chi-Square Distribution, Enzyme-Linked Immunosorbent Assay, Female, Humans, Immunoblotting, Male, Middle Aged, Rheumatoid Factor analysis, Salivary Glands pathology, Sjogren's Syndrome diagnosis, Sjogren's Syndrome immunology, Statistics, Nonparametric, Hepatitis C, Chronic complications, Sjogren's Syndrome virology
Abstract

Background: Hepatis C virus infection is presently an exclusion criterion to classify Sjögren's syndrome; however, there are distinct clinicopathologic and biologic similarities between HCV-related and SS-related chronic inflammation of mucosa-associated lymphoid tissue and lymphoproliferation that suggest common pathogenetic pathways., Objectives: To determine whether a subset of patients with sicca syndrome and HCV infection may present a true primary SS rather than a distinct clinicobiologic entity., Methods: We extensively characterized 20 consecutive patients with positive anti-HCV antibodies and heavy subjective dry eye and/or dry mouth symptoms, plus positive unstimulated sialometry and/or Shirmer's test. We then compared these features with those in HCV-negative primary SS controls (classified according to the latest American-European Consensus Group Classification Criteria for SS)., Results: Of the 20 HCV-positive patients with sicca manifestations, 12 (60%) had positive anti-SSA/SSB antibodies (3/12 by enzyme-linked immunosorbent assay and 6/12 by immunoblot) and/or positive salivary gland biopsy (at least 1 focus/4 mm2), which met the strict classification criteria for SS, as in the case of HCV-negative SS controls. Comparing the HCV-positive SS subset with HCV-negative SS controls showed similar female to male ratio (11/1 vs. 46/4), major salivary gland swelling (17% vs. 26%), positive antinuclear antibodies (75 vs. 94%) and positive rheumatoid factor (58 vs. 52%). Significant differences (P < 0.05) were seen in mean age (69 vs. 56 years), liver disease (50 vs. 2%), lung disease (25 vs. 0%), anti-SSA/SSB positivity (25 vs. 90%), and low C3 or C4 (83 vs. 36%). HCV-positive SS patients exhibited a trend for more frequent chronic gastritis (50 vs. 22%), fibromyalgia (33 vs. 14%), peripheral neuropathy (33 vs. 18%), purpura (33 vs. 19%) and cryoglobulinemia (33 vs. 6%)., Conclusions: A major subset of HCV-positive patients with definite subjective sicca symptoms and positive objective tests may indeed present a true, though peculiar, subset of SS. There are strict similarities with key clinical, pathologic and immunologic findings of definite HCV-negative SS. Other features appear more characteristic of HCV infection. When also considering that HCV is sialotropic and may be treated, HCV-related chronic sialadenitis represents a unique opportunity to clarify key pathogenetic events occurring in the large majority of HCV-negative SS; and similarities to typical primary SS, rather than differences, should be taken into account.

Published
2002

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