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1. Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry ‘SPRING’ of the Italian Society for Rheumatology

Author

Carlo Alberto Scirè, Andrea Doria, Marcello Govoni, Gerolamo Bianchi, Marco Matucci-Cerinic, Florenzo Iannone, Ennio Lubrano, Corrado Campochiaro, Veronica Codullo, Alessandra Della Rossa, Gemma Lepri, Elisabetta Zanatta, Beretta Lorenzo, Doria Andrea, Lepri Gemma, Lorenzo Beretta, Greta Carrara, Alarico Ariani, Simone Parisi, Marta Saracco, Francesco Girelli, Maria De Santis, Federica Lumetti, Dilia Giuggioli, Enrico Fusaro, Simone Barsotti, Ilaria Cavazzana, Federica Furini, Carlo Salvarani, Serena Guiducci, Franco Cozzi, Valeria Riccieri, Francesca Ingegnoli, Edoardo Rosato, Antonietta Gigante, Rosario Foti, Elisa Visalli, Fabio Cacciapaglia, Lorenzo Dagna, Franco Franceschini, Silvia Bellando-Randone, Giovanna Cuomo, Gianluigi Bajocchi, Alessandro Giollo, Giacomo De Luca, Giuseppina Abignano, Carlo Sciré, Anna Zanetti, Giovanni Zanframundo, Edoardo Cipolletta, Silvia Laura Bosello, Clodoveo Ferri, Amelia Spinella, Giuseppa Pagano Mariano, Maurizio Caminiti, Giuseppe Murdaca, Salvatore D'Angelo, Gianpiero Landolfi, Nicoletta Romeo, Gian Domenico Sebastiani, Erika Pigatto, Rossella De Angelis, Davide Rozza, Maria-Grazia Lazzaroni, Anna Maria Iuliano, Giovanni Ciano, Gianluca Bagnato, Ilenia De Andres, Cecilia Agnes, Luca Magnani, Claudio Di Vico, Greta Pellagrino, Elena Generali, Gianna Mennillo, Licia Vultaggio, Clara Lisa Peroni, Abignano Giuseppina, Agnes Cecilia, Amato Giorgio, Ariani Alarico, Bagnato Gianluca, Bajoicchi Gianluigi, Barsotti Simone, Bellando-Randone Silvia, Benenati Alessia, Bianchi Gerolamo, Bosello Silvia, Cacciapaglia Fabio, Calabrese Francesca, Caminiti Maurizio, Campochiaro Corrado, Carignola Renato, Ciano Giovanni, Cipolletta Edoardo, Codullo Veronica, Cozzi Franco, Cuomo Giovanna, D’Angelo Salvatore, Dagna Lorenzo, Dall’Ara Francesca, De Andres Ilenia, De Angelis Rossella, De Cata Angelo, De Luca Giacomo, De Santis Maria, Della Rossa Alessandra, Di Vico Claudio, Doveri Marica, Foti Rosario, Furini Federica, Fusaro Enrico, Generali Elena, Gigante Antonietta, Giollo Alessandro, Girelli Francesco, Giuggioli Dilia, Govoni Marcello, Guiducci Serena, Iannone Florenzo, Ingegnoli Francesca, Iuliano Anna Maria, Lazzaroni Maria Grazia, Lubrano Ennio, Lumetti Federica, Magnani Luca, Mennillo Gianna, Murdaca Giuseppe Ospedale, Pagano Mariano Giuseppa, Parisi Simone, Pellegrino Greta, Peroni Clara Lisa, Pigatto Erika, Riccieri Valeria, Romeo Nicoletta, Rosato Edoardo, Sambataro Gianluca, Saracco Marta, Sebastiani Giandomenico, Spinella Amelia, Talotta Rossella, Visalli Elisa, Vultaggio Licia, Zanatta Elisabetta, and Zanframundo Giovanni

Subjects
Medicine
Abstract

Objective To describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort.Methods Data involving 1808 SSc patients from Italian Systemic sclerosis PRogression INvestiGation registry were collected. The ssSSc was defined by the absence of any cutaneous sclerosis and/or puffy fingers. Clinical and serological features of ssSSc were compared with limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subsets.Results Among patients with SSc, only 61 (3.4%) were classified as having ssSSc (F/M=19/1). Time from Raynaud’s phenomenon (RP) onset to diagnosis was longer in ssSSc (3 years, IQR 1–16.5) than lcSSc (2 years, IQR 0–7), and dcSSc (1 year, IQR 0–3) (p

Published
2023
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3. Doppler ultrasound, a noninvasive tool for the study of mesenteric arterial flow in systemic sclerosis: a cross-sectional study of a patient cohort with review and meta-analysis of the literature

Author

Bandini, Giulia, Monami, Matteo, Ciuti, Gabriele, Mercatelli, Paolo, Lo Cricchio, Anna, De Santis, Maria Cristina, Bonomi, Francesco, Bellando Randone, Silvia, Campochiaro, Corrado, El Aoufy, Khadija, Ruaro, Barbara, Giuggioli, Dilia, Hughes, Michael, McMahan, Zsuzsanna H., Benfaremo, Devis, Moroncini, Gianluca, Maconi, Giovanni, Accogli, Esterita, Dagna, Lorenzo, Matucci Cerinic, Marco, and Moggi Pignone, Alberto

Published
2024
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4. From Localized Scleroderma to Systemic Sclerosis: Coexistence or Possible Evolution

Author

Giuggioli Dilia, Colaci Michele, Cocchiara Emanuele, Spinella Amelia, Lumetti Federica, and Ferri Clodoveo

Subjects
Dermatology, RL1-803
Abstract

Background. Systemic sclerosis (SSc) and localized scleroderma (LoS) are two different diseases that may share some features. We evaluated the relationship between SSc and LoS in our case series of SSc patients. Methods. We analysed the clinical records of 330 SSc patients, in order to find the eventual occurrence of both the two diseases. Results. Eight (2.4%) female patients presented both the two diagnoses in their clinical histories. Six developed LoS prior to SSc; in 4/6 cases, the presence of autoantibodies was observed before SSc diagnosis. Overall, the median time interval between LoS and SSc diagnosis was 18 (range 0–156) months. Conclusions. LoS and SSc are two distinct clinical entities that may coexist. Moreover, as anecdotally reported in pediatric populations, we suggested the possible development of SSc in adult patients with LoS, particularly in presence of Raynaud’s phenomenon or antinuclear antibodies before the SSc onset.

Published
2018
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6. Towards a comprehensive approach to the management and prognosis of systemic sclerosis's patients: The role of comorbidities in the SPRING-SIR registry

Author

Orlandi, Martina, Bellando-Randone, Silvia, De Angelis, Rossella, Ferri, Clodoveo, Giuggioli, Dilia, Cacciapaglia, Fabio, Magnani, Luca, Cuomo, Giovanna, Gigante, Antonietta, Codullo, Veronica, Campochiaro, Corrado, Ariani, Alarico, Foti, Rosario, Guiducci, Serena, Matucci-Cerinic, Marco, and Bruni, Cosimo

Published
2024
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8. Impact of COVID-19 and vaccination campaign on 1,755 systemic sclerosis patients during first three years of pandemic. Possible risks for individuals with impaired immunoreactivity to vaccine, ongoing immunomodulating treatments, and disease-related lung involvement during the next pandemic phase

Author

Ferri, Clodoveo, Raimondo, Vincenzo, Giuggioli, Dilia, Gragnani, Laura, Lorini, Serena, Dagna, Lorenzo, Bosello, Silvia Laura, Foti, Rosario, Riccieri, Valeria, Guiducci, Serena, Cuomo, Giovanna, Tavoni, Antonio, De Angelis, Rossella, Cacciapaglia, Fabio, Zanatta, Elisabetta, Cozzi, Franco, Murdaca, Giuseppe, Cavazzana, Ilaria, Romeo, Nicoletta, Codullo, Veronica, Pellegrini, Roberta, Varcasia, Giuseppe, De Santis, Maria, Selmi, Carlo, Abignano, Giuseppina, Caminiti, Maurizio, L'Andolina, Massimo, Olivo, Domenico, Lubrano, Ennio, Spinella, Amelia, Lumetti, Federica, De Luca, Giacomo, Ruscitti, Piero, Urraro, Teresa, Visentini, Marcella, Bellando-Randone, Silvia, Visalli, Elisa, Testa, Davide, Sciascia, Gabriella, Masini, Francesco, Pellegrino, Greta, Saccon, Francesca, Balestri, Eugenia, Elia, Giusy, Ferrari, Silvia Martina, Tonutti, Antonio, Dall’Ara, Francesca, Pagano Mariano, Giuseppa, Pettiti, Giorgio, Zanframundo, Giovanni, Brittelli, Raffaele, Aiello, Vincenzo, Dal Bosco, Ylenia, Foti, Roberta, Di Cola, Ilenia, Scorpiniti, Daniela, Fusaro, Enrico, Ferrari, Tommaso, Gigliotti, Pietro, Campochiaro, Corrado, Francioso, Francesca, Iandoli, Carlo, Caira, Virginia, Zignego, Anna Linda, D'Angelo, Salvatore, Franceschini, Franco, Matucci-Cerinic, Marco, Giacomelli, Roberto, Doria, Andrea, Santini, Stefano Angelo, Fallahi, Poupak, Iannone, Florenzo, and Antonelli, Alessandro

Published
2023
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9. Non-surgical local treatments of digital ulcers in systemic sclerosis: a systematic literature review

Author

Campochiaro, Corrado, Suliman, Yossra A, Hughes, Michael, Schoones, Jan W, Giuggioli, Dilia, Moinzadeh, Pia, Baron, Murray, Chung, Lorinda, Ross, Laura, Maltez, Nancy, Allanore, Yannick, Denton, Christopher P, Distler, Oliver, Frech, Tracy, Furst, Daniel E, Khanna, Dinesh, Krieg, Thomas, Kuwana, Masataka, Matucci-Cerinic, Marco, Pope, Janet, and Alunno, Alessia

Published
2023
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10. Surgical management of digital ulcers in systemic sclerosis: A systematic literature review

Author

Suliman, Yossra A, Campochiaro, Corrado, Hughes, Michael, Schoones, Jan W., Giuggioli, Dilia, Moinzadeh, Pia, Baron, Murray, Chung, Lorinda, Ross, Laura, Maltez, Nancy, Allanore, Yannick, Denton, Christopher P., Distler, Oliver, Frech, Tracy, Furst, Daniel E., Khanna, Dinesh, Krieg, Thomas, Kuwana, Masataka, Matucci-Cerinic, Marco, Pope, Janet, and Alunno, Alessia

Published
2023
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11. Management of mixed cryoglobulinemia with rituximab: evidence and consensus-based recommendations from the Italian Study Group of Cryoglobulinemia (GISC)

Author

Quartuccio, Luca, Bortoluzzi, Alessandra, Scirè, Carlo Alberto, Marangoni, Antonio, Del Frate, Giulia, Treppo, Elena, Castelnovo, Laura, Saccardo, Francesco, Zani, Roberta, Candela, Marco, Fraticelli, Paolo, Mazzaro, Cesare, Renoldi, Piero, Scaini, Patrizia, Filippini, Davide Antonio, Visentini, Marcella, Scarpato, Salvatore, Giuggioli, Dilia, Mascia, Maria Teresa, Sebastiani, Marco, Zignego, Anna Linda, Lauletta, Gianfranco, Fiorilli, Massimo, Casato, Milvia, Ferri, Clodoveo, Pietrogrande, Maurizio, Pioltelli, Pietro Enrico, De Vita, Salvatore, Monti, Giuseppe, and Galli, Massimo

Published
2023
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12. Geographical heterogeneity of clinical and serological phenotypes of systemic sclerosis observed at tertiary referral centres. The experience of the Italian SIR-SPRING registry and review of the world literature

Author

Ferri, Clodoveo, De Angelis, Rossella, Giuggioli, Dilia, Bajocchi, Gianluigi, Dagna, Lorenzo, Zanframundo, Giovanni, Foti, Rosario, Cacciapaglia, Fabio, Cuomo, Giovanna, Ariani, Alarico, Rosato, Edoardo, Guiducci, Serena, Girelli, Francesco, Riccieri, Valeria, Zanatta, Elisabetta, Bosello, Silvia, Cavazzana, Ilaria, Ingegnoli, Francesca, De Santis, Maria, Murdaca, Giuseppe, Abignano, Giuseppina, Romeo, Nicoletta, Della Rossa, Alessandra, Caminiti, Maurizio, Iuliano, Annamaria, Ciano, Giovanni, Beretta, Lorenzo, Bagnato, Gianluca, Lubrano, Ennio, De Andres, Ilenia, Giollo, Alessandro, Saracco, Marta, Agnes, Cecilia, Lumetti, Federica, Spinella, Amelia, Magnani, Luca, Campochiaro, Corrado, De Luca, Giacomo, Codullo, Veronica, Visalli, Elisa, Masini, Francesco, Gigante, Antonietta, Bellando-Randone, Silvia, Pellegrino, Greta, Pigatto, Erika, Lazzaroni, Maria Grazia, Franceschini, Franco, Generali, Elena, Mennillo, Gianna, Barsotti, Simone, Mariano, Giuseppa Pagano, Calabrese, Francesca, Furini, Federica, Vultaggio, Licia, Parisi, Simone, Peroni, Clara Lisa, Rozza, Davide, Zanetti, Anna, Carrara, Greta, Landolfi, Giampiero, Scirè, Carlo Alberto, Bianchi, Gerolamo, Fusaro, Enrico, Sebastiani, Gian Domenico, Govoni, Marcello, D'Angelo, Salvatore, Cozzi, Franco, Doria, Andrea, Iannone, Florenzo, Salvarani, Carlo, and Matucci-Cerinic, Marco

Published
2022
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13. Absent or suboptimal response to booster dose of COVID-19 vaccine in patients with autoimmune systemic diseases

Author

Ferri, Clodoveo, Gragnani, Laura, Raimondo, Vincenzo, Visentini, Marcella, Giuggioli, Dilia, Lorini, Serena, Foti, Rosario, Cacciapaglia, Fabio, Caminiti, Maurizio, Olivo, Domenico, Cuomo, Giovanna, Pellegrini, Roberta, Pigatto, Erika, Urraro, Teresa, Naclerio, Caterina, Tavoni, Antonio, Puccetti, Lorenzo, Cavazzana, Ilaria, Ruscitti, Piero, Vadacca, Marta, La Gualana, Francesca, Cozzi, Franco, Spinella, Amelia, Visalli, Elisa, Bosco, Ylenia Dal, Amato, Giorgio, Masini, Francesco, Mariano, Giuseppa Pagano, Brittelli, Raffaele, Aiello, Vincenzo, Scorpiniti, Daniela, Rechichi, Giovanni, Varcasia, Giuseppe, Monti, Monica, Elia, Giusy, Franceschini, Franco, Casato, Milvia, Ursini, Francesco, Giacomelli, Roberto, Fallahi, Poupak, Santini, Stefano Angelo, Iannone, Florenzo, Salvarani, Carlo, Zignego, Anna Linda, and Antonelli, Alessandro

Published
2022
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16. Impaired immunogenicity to COVID-19 vaccines in autoimmune systemic diseases. High prevalence of non-response in different patients’ subgroups

Author

Ferri, Clodoveo, Ursini, Francesco, Gragnani, Laura, Raimondo, Vincenzo, Giuggioli, Dilia, Foti, Rosario, Caminiti, Maurizio, Olivo, Domenico, Cuomo, Giovanna, Visentini, Marcella, Cacciapaglia, Fabio, Pellegrini, Roberta, Pigatto, Erika, Urraro, Teresa, Naclerio, Caterina, Tavoni, Antonio, Puccetti, Lorenzo, Varcasia, Giuseppe, Cavazzana, Ilaria, L'Andolina, Massimo, Ruscitti, Piero, Vadacca, Marta, Gigliotti, Pietro, La Gualana, Francesca, Cozzi, Franco, Spinella, Amelia, Visalli, Elisa, Dal Bosco, Ylenia, Amato, Giorgio, Masini, Francesco, Pagano Mariano, Giuseppa, Brittelli, Raffaele, Aiello, Vincenzo, Caminiti, Rodolfo, Scorpiniti, Daniela, Rechichi, Giovanni, Ferrari, Tommaso, Monti, Monica, Elia, Giusy, Franceschini, Franco, Meliconi, Riccardo, Casato, Milvia, Iannone, Florenzo, Giacomelli, Roberto, Fallahi, Poupak, Santini, Stefano Angelo, Zignego, Anna Linda, and Antonelli, Alessandro

Published
2021
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20. Correction to: Management of mixed cryoglobulinemia with rituximab: evidence and consensus-based recommendations from the Italian Study Group of Cryoglobulinemia (GISC)

Author

Quartuccio, Luca, Bortoluzzi, Alessandra, Scirè, Carlo Alberto, Marangoni, Antonio, Del Frate, Giulia, Treppo, Elena, Castelnovo, Laura, Saccardo, Francesco, Zani, Roberta, Candela, Marco, Fraticelli, Paolo, Mazzaro, Cesare, Renoldi, Piero, Scaini, Patrizia, Filippini, Davide Antonio, Visentini, Marcella, Scarpato, Salvatore, Giuggioli, Dilia, Mascia, Maria Teresa, Sebastiani, Marco, Zignego, Anna Linda, Lauletta, Gianfranco, Fiorilli, Massimo, Casato, Milvia, Ferri, Clodoveo, Pietrogrande, Maurizio, Pioltelli, Pietro Enrico, De Vita, Salvatore, Monti, Giuseppe, and Galli, Massimo

Published
2023
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21. BMP-4 and fetuin A in systemic sclerosis patients with or without calcinosis.

Author

Lofaro, Francesco Demetrio, Giuggioli, Dilia, Bonacorsi, Susanna, Orlandi, Martina, Spinella, Amelia, De Pinto, Marco, Secchi, Ottavio, Ferri, Clodoveo, and Boraldi, Federica

Subjects
CALCINOSIS, CONNECTIVE tissue diseases, CARBONATE minerals, PHOSPHATE minerals, SYSTEMIC scleroderma
Abstract

Introduction: Systemic sclerosis (SSc) is a connective tissue disease at the interface between inflammation and autoimmunity progressively leading to diffuse microvascular and fibrotic involvement of the skin and of multiple internal organs. Approximately, 20-40% of SSc patients suffer from cutaneous calcinosis, a debilitating manifestation due to calcium salt deposition in soft connective tissues, causing pain, ulceration, infection, and deformities, responsible of severe functional limitations. Pathomechanisms are poorly understood as well as markers/molecules capable to predict the risk of patients to develop calcinosis. Methods: An observational study was performed in 51 female patients, 25 with and 26 without calcinosis to compare clinical and laboratory parameters and to evaluate pro- and anti-calcifying circulating markers and the in vitro serum calcification potential (T50). Moreover, calcinosis samples were analyzed to characterize their mineral composition. Results and discussion: Data demonstrate statistically significant differences in the prevalence of clinical manifestations and ACA and Scl70 autoantibodies in SSc patient with calcinosis compared to those without calcinosis. In SSc patients with calcinosis, serum levels of BMP-4 are higher, fetuin A might be regarded as a potential circulating prognostic marker and a negative correlation was observed between T50 and the global score of clinical manifestations, suggesting a potential predictive role of pro- and anti-calcifying molecules in SSc patients. Furthermore, calcinosis samples were characterized by the co-existence of phosphate and carbonate minerals with different stability and solubility. Further investigations on circulating markers in larger patient cohorts, especially at the early stages and throughout the natural course of the disease, may clarify their pathogenetic role in the SSc-related cutaneous calcinosis. [ABSTRACT FROM AUTHOR]

Published
2024
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22. The predictive role of the TAPSE/sPAP ratio for cardiovascular events and mortality in systemic sclerosis with pulmonary hypertension.

Author

de Pinto, Marco, Coppi, Francesca, Spinella, Amelia, Pagnoni, Gianluca, Morgante, Vernizia, Macripò, Pierluca, Boschini, Matteo, Guerra, Anna Francesca, Tampieri, Francesca, Secchi, Ottavio, Orlandi, Martina, Amati, Gabriele, Lumetti, Federica, Sandri, Gilda, Rossi, Rosario, Boriani, Giuseppe, Mattioli, Anna Vittoria, Ferri, Clodoveo, and Giuggioli, Dilia

Published
2024
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23. Rituximab retention rate in systemic sclerosis: a long term real-life multicenter study

Author

De Luca, Giacomo, primary, De Lorenzis, Enrico, additional, Campochiaro, Corrado, additional, Cacciapaglia, Fabio, additional, Del Papa, Nicoletta, additional, Zanatta, Elisabetta, additional, Airò, Paolo, additional, Lazzaroni, Maria Grazia, additional, Giuggioli, Dilia, additional, De Santis, Maria, additional, Alonzi, Gabriella, additional, Stano, Stefano, additional, Binda, Marco, additional, Moccaldi, Beatrice, additional, Tonutti, Antonio, additional, Cavalli, Silvia, additional, Batani, Veronica, additional, Natalello, Gerlando, additional, Iannone, Florenzo, additional, D’Agostino, Maria Antonietta, additional, Dagna, Lorenzo, additional, Matucci-Cerinic, Marco, additional, and Bosello, Silvia Laura, additional

Published
2024
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24. Rituximab retention rate in systemic sclerosis: a long term real-life multicentre study

Author

De Luca, Giacomo, De Lorenzis, Enrico, Campochiaro, Corrado, Cacciapaglia, Fabio, Del Papa, Nicoletta, Zanatta, Elisabetta, Airò, Paolo, Lazzaroni, Maria Grazia, Giuggioli, Dilia, De Santis, Maria, Alonzi, Gabriella, Stano, Stefano, Binda, Marco, Moccaldi, Beatrice, Tonutti, Antonio, Cavalli, Silvia, Batani, Veronica, Natalello, Gerlando, Iannone, Florenzo, D'Agostino, Maria Antonietta, Dagna, Lorenzo, Matucci-Cerinic, Marco, Bosello, Silvia Laura, Cavalli, Silvia (ORCID:0000-0002-1754-0084), D'Agostino, Maria Antonietta (ORCID:0000-0002-5347-0060), Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), De Luca, Giacomo, De Lorenzis, Enrico, Campochiaro, Corrado, Cacciapaglia, Fabio, Del Papa, Nicoletta, Zanatta, Elisabetta, Airò, Paolo, Lazzaroni, Maria Grazia, Giuggioli, Dilia, De Santis, Maria, Alonzi, Gabriella, Stano, Stefano, Binda, Marco, Moccaldi, Beatrice, Tonutti, Antonio, Cavalli, Silvia, Batani, Veronica, Natalello, Gerlando, Iannone, Florenzo, D'Agostino, Maria Antonietta, Dagna, Lorenzo, Matucci-Cerinic, Marco, Bosello, Silvia Laura, Cavalli, Silvia (ORCID:0000-0002-1754-0084), D'Agostino, Maria Antonietta (ORCID:0000-0002-5347-0060), and Bosello, Silvia Laura (ORCID:0000-0002-4837-447X)

Abstract

Objectives: to report real-life data on rituximab retention-rate as indicator of safety and efficacy in a multicentric national cohort of systemic sclerosis patients. Methods: SSc patients treated with rituximab and followed for at least 36 months were included, clinically characterized, and longitudinally monitored. A competing risk analysis with sub-Hazard Ratio(sHR) definition was performed to explore the clinical variables linked to specific cause of rituximab discontinuation. Results: One-hundred-fifty-two SSc-patients (mean age 47.3 ± 12.3 years; females 79.6%; diffuse disease 77.6%; anti-topoisomerase-I positivity 63.2%) were evaluated over a median(IQR) time of 3.3(1.7-5.0) years. The primary indication for rituximab were interstitial lung disease (ILD)(38.8%), worsening skin fibrosis(36.8%), and arthritis(13.8%); 138 patients(90.8%) received more than one rituximab course. The 5-years rituximab retention rate was 59.9%(44.6-64.7%). Clinical response was the most common reason for rituximab discontinuation[5.7(3.7-8.4) per 100 patient-year] and was associated with a shorter disease duration[sHR 0.8(0.7-0.9)], anti-topoisomerase-I negativity[sHR 0.4(0.2-0.9)], previous digital ulcers[sHR 2.6(1.1-6.2] and no history of arthritis[sHR 0.3 (0.1-0.8)]. Treatment failure was the second cause of rituximab discontinuation[3.7(2.2-6.0) per 100 patient-year] and was associated with anti-centromere antibody positivity[sHR 2.8(1.1-7.4)] and anti-topoisomerase-I negativity[sHR 0.2(0.1-0.6)]. Adverse events(AEs) were the less common cause of discontinuation[3.1(1.7-5.2) per 100 patient-year], associated with limited cutaneous subset[sHR 3.4(1.2-9.7)] and previous mycophenolate mofetil treatment[sHR 4.5(1.2-16.3)]. Conclusion: rituximab is a safe and effective treatment in SSc: clinical response emerged as the primary reason for rituximab discontinuation, and AEs had a limited impact on treatment persistence. The identification of specific disease features associated with a

Published
2024

25. Long-term retention rate, adverse event temporal patterns and rescue treatment strategies of mycophenolate mofetil in systemic sclerosis: insights from real-life

Author

De Lorenzis, Enrico, Natalello, Gerlando, Pellegrino, Greta, Verardi, Lucrezia, Batani, Veronica, Lepri, Gemma, Stano, Stefano, Armentano, Giuseppe, De Pinto, Marco, Motta, Francesca, Di Donato, Stefano, Kakkar, Vishal, Fiore, Silvia, Bisconti, Ilaria, Campochiaro, Corrado, Cometi, Laura, Tonutti, Antonio, Spinella, Amalia, Truglia, Simona, Cavalli, Silvia, De Santis, Maria, Giuggioli, Dilia, Del Papa, Nicoletta, Guiducci, Serena, Cacciapaglia, Fabio, De Luca, Giacomo, Iannone, Fiorenzo, Ricceri, Valeria, Matucci Cerinic, Marco, D'Agostino, Maria Antonietta, Del Galdo, Francesco, Bosello, Silvia Laura, Cavalli, Silvia (ORCID:0000-0002-1754-0084), D'Agostino, Maria Antonietta (ORCID:0000-0002-5347-0060), Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), De Lorenzis, Enrico, Natalello, Gerlando, Pellegrino, Greta, Verardi, Lucrezia, Batani, Veronica, Lepri, Gemma, Stano, Stefano, Armentano, Giuseppe, De Pinto, Marco, Motta, Francesca, Di Donato, Stefano, Kakkar, Vishal, Fiore, Silvia, Bisconti, Ilaria, Campochiaro, Corrado, Cometi, Laura, Tonutti, Antonio, Spinella, Amalia, Truglia, Simona, Cavalli, Silvia, De Santis, Maria, Giuggioli, Dilia, Del Papa, Nicoletta, Guiducci, Serena, Cacciapaglia, Fabio, De Luca, Giacomo, Iannone, Fiorenzo, Ricceri, Valeria, Matucci Cerinic, Marco, D'Agostino, Maria Antonietta, Del Galdo, Francesco, Bosello, Silvia Laura, Cavalli, Silvia (ORCID:0000-0002-1754-0084), D'Agostino, Maria Antonietta (ORCID:0000-0002-5347-0060), and Bosello, Silvia Laura (ORCID:0000-0002-4837-447X)

Abstract

Objectives MMF is a mainstay for the treatment of SSc. The occurrence and implications of MMF-related adverse events (AEs) on drug retention rates in real life remain poorly defined. We aimed to determine the MMF retention rate and to investigate the causes and patterns of discontinuation, AEs and treatment options used after discontinuation.Methods SSc patients who started MMF treatment underwent a retrospective longitudinal assessment for up to 5 years. We documented the incidence, predictors and impacts of MMF treatment on gastrointestinal intolerance, infections, laboratory abnormalities and cancer. Rescue strategies implemented after MMF discontinuation were recorded.Results The 5-year MMF retention rate of 554 patients stood at 70.7%, and 19.6% of them stopped MMF due to AEs. One out of every four patients experienced a dose reduction or discontinuation of MMF due to AEs, with gastrointestinal intolerance being the predominant cause. The 5-year cumulative incidence rates for gastrointestinal intolerance, cancer, severe infections and laboratory toxicity leading to MMF discontinuation were 6.4%, 4.1%, 3.1% and 2.1%, respectively. Lower respiratory tract was the most affected, with bacteria being the predominant causative agent. Intestinal and pulmonary circulation involvement were tied to elevated AE rates and MMF discontinuation. The most common approaches post-MMF cessation were 'watch and wait' and switch to rituximab.Conclusions : MMF use in SSc appears to be limited by the occurrence of AEs, both in terms of persistence and dosing of the drug. Rescue options after MMF discontinuation are limited and many patients remain without immunosuppressant.

Published
2024

26. Significant nailfold capillary loss and late capillaroscopic pattern are associated with pulmonary arterial hypertension in systemic sclerosis

Author

De Angelis, Rossella, Riccieri, Valeria, Cipolletta, Edoardo, Del Papa, Nicoletta, Ingegnoli, Francesca, Bosello, Silvia Laura, Spinella, Amelia, Pellegrino, Greta, de Pinto, Marco, Papa, Silvia, Armentaro, Giuseppe, Giuggioli, Dilia, Bosello, Silvia (ORCID:0000-0002-4837-447X), De Angelis, Rossella, Riccieri, Valeria, Cipolletta, Edoardo, Del Papa, Nicoletta, Ingegnoli, Francesca, Bosello, Silvia Laura, Spinella, Amelia, Pellegrino, Greta, de Pinto, Marco, Papa, Silvia, Armentaro, Giuseppe, Giuggioli, Dilia, and Bosello, Silvia (ORCID:0000-0002-4837-447X)

Abstract

Objective: To evaluate differences in nailfold videocapillaroscopy (NVC) findings between SSc patients with and without a diagnosis of pulmonary arterial hypertension (PAH).Methods: One hundred and ten SSc patients were enrolled in this cross-sectional, case-control, multicentre study. Patients were divided into cases (SSc-PAH confirmed by right heart catheterization) and controls (SSc-nonPAH with low probability of PAH). NVC patterns (early, active and late) and morphological parameters (microvascular density, non-specific abnormalities, giant capillaries, micro-haemorrhages, avascular areas) were considered using a semiquantitative scoring system.Results: SSc-PAH patients showed higher frequencies of late pattern (P < 0.01), non-specific abnormalities (P < 0.01), lower capillary density (P < 0.01), higher avascular areas (P < 0.01) and a higher mean NVC score (P < 0.01). Contrarily, the early/active pattern (P < 0.01) and a higher rate of micro-haemorrhages (P = 0.04) were more frequent in non-PAH patients. By a multivariate analysis, SSc-PAH patients, compared with non-PAH, had more non-specific abnormalities [27/55, 49.1% vs 10/55, 18.2%; adjusted odd ratio (OR) 16.89; 95% CI: 3.06, 93.16], a lower capillary density (grade 3, 20/55, 36.4% vs 5/55, 9.1%; adjusted OR 38.33; 95% CI: 2.34, 367.80) and avascular areas (18/55, 32.7% vs 10/55, 18.2%; adjusted OR 16.90; 95% CI: 2.64, 44.35). A correlation was found between the mean pulmonary arterial pressure and avascular areas (P < 0.01), capillary density (P < 0.01) and non-specific abnormalities (P < 0.01). A clinical model including the NVC variables may be able to predict a diagnosis of PAH.Conclusion: Our results indicate that the distinctive peripheral microcirculatory injury of SSc, i.e. capillary loss and morphological abnormalities, appear more severe and pronounced in patients with SSc-PAH.

Published
2024

27. Practice pattern for the use of intravenous iloprost for the treatment of peripheral vasculopathy in systemic sclerosis: A case-control study from the Italian national multicenter 'SPRING' (Systemic Sclerosis Progression InvestiGation) Registry

Author

Riccieri, Valeria, Pellegrino, Greta, Cipolletta, Edoardo, Giuggioli, Dilia, Bajocchi, Gianluigi, Bellando-Randone, Silvia, Dagna, Lorenzo, Zanframundo, Giovanni, Foti, Rosario, Cacciapaglia, Fabio, Cuomo, Giovanna, Ariani, Alarico, Rosato, Edoardo, Lepri, Gemma, Girelli, Francesco, Zanatta, Elisabetta, Bosello, Silvia Laura, Cavazzana, Ilaria, Ingegnoli, Francesca, De Santis, Maria, Murdaca, Giuseppe, Abignano, Giuseppina, Romeo, Nicoletta, Della Rossa, Alessandra, Caminiti, Maurizio, Iuliano, Annamaria, Ciano, Giovanni, Beretta, Lorenzo, Bagnato, Gianluca, Lubrano, Ennio, De Andres, Ilenia, Giollo, Alessandro, Saracco, Marta, Agnes, Cecilia, Lumetti, Federica, Spinella, Amelia, Magnani, Luca, Campochiaro, Corrado, De Luca, Giacomo, Codullo, Veronica, Visalli, Elisa, Di Vico, Claudio, Gigante, Antonietta, Saccon, Francesca, Grazia Lazzaroni, Maria, Franceschini, Franco, Generali, Elena, Mennillo, Gianna, Barsotti, Simone, Pagano Mariano, Giuseppa, Calabrese, Francesca, Furini, Federica, Vultaggio, Licia, Parisi, Simone, Peroni, Clara Lisa, Bianchi, Gerolamo, Conti, Fabrizio, Cozzi, Franco, D'Angelo, Salvatore, Doria, Andrea, Fusaro, Enrico, Govoni, Marcello, Guiducci, Serena, Iannone, Florenzo, Salvarani, Carlo, Sebastiani, Gian Domenico, Ferri, Clodoveo, Matucci-Cerinic, Marco, De Angelis, Rossella, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), Riccieri, Valeria, Pellegrino, Greta, Cipolletta, Edoardo, Giuggioli, Dilia, Bajocchi, Gianluigi, Bellando-Randone, Silvia, Dagna, Lorenzo, Zanframundo, Giovanni, Foti, Rosario, Cacciapaglia, Fabio, Cuomo, Giovanna, Ariani, Alarico, Rosato, Edoardo, Lepri, Gemma, Girelli, Francesco, Zanatta, Elisabetta, Bosello, Silvia Laura, Cavazzana, Ilaria, Ingegnoli, Francesca, De Santis, Maria, Murdaca, Giuseppe, Abignano, Giuseppina, Romeo, Nicoletta, Della Rossa, Alessandra, Caminiti, Maurizio, Iuliano, Annamaria, Ciano, Giovanni, Beretta, Lorenzo, Bagnato, Gianluca, Lubrano, Ennio, De Andres, Ilenia, Giollo, Alessandro, Saracco, Marta, Agnes, Cecilia, Lumetti, Federica, Spinella, Amelia, Magnani, Luca, Campochiaro, Corrado, De Luca, Giacomo, Codullo, Veronica, Visalli, Elisa, Di Vico, Claudio, Gigante, Antonietta, Saccon, Francesca, Grazia Lazzaroni, Maria, Franceschini, Franco, Generali, Elena, Mennillo, Gianna, Barsotti, Simone, Pagano Mariano, Giuseppa, Calabrese, Francesca, Furini, Federica, Vultaggio, Licia, Parisi, Simone, Peroni, Clara Lisa, Bianchi, Gerolamo, Conti, Fabrizio, Cozzi, Franco, D'Angelo, Salvatore, Doria, Andrea, Fusaro, Enrico, Govoni, Marcello, Guiducci, Serena, Iannone, Florenzo, Salvarani, Carlo, Sebastiani, Gian Domenico, Ferri, Clodoveo, Matucci-Cerinic, Marco, De Angelis, Rossella, and Bosello, Silvia Laura (ORCID:0000-0002-4837-447X)

Abstract

Background: Intravenous iloprost has been widely used for the treatment of systemic sclerosis peripheral vasculopathy. No agreement has been found on the regimen and the dosage of intravenous iloprost in different scleroderma subset conditions. This study aimed to evaluate the modalities of intravenous iloprost administration within a large cohort of systemic sclerosis patients from the SPRING Registry and to identify any associated clinical-demographic, instrumental or therapeutic data. Patients and methods: Data of systemic sclerosis patients treated with intravenous iloprost for at least 1 year (case group) were retrospectively analyzed, including different timing and duration of intravenous iloprost session, and compared with those of untreated patients (control group). Results: Out of 1895 analyzed patients, 937 (49%) received intravenous iloprost treatment, while 958 (51%) were assigned to the control group. Among cases, about 70% were treated every 4 weeks, 24% with an interval of more than 4 weeks, and only 6% of less than 4 weeks. Most patients receiving the treatment every 4 weeks, or less, underwent infusion cycle for 1 day only, while if it was scheduled with an interval of more than 4 weeks, a total number of 5 consecutive days of infusions was the preferred regimen. The comparison between the two groups revealed that patients treated with intravenous iloprost had a higher frequency of DUs (p < 0.001), pitting scars (p < 0.001), diffuse cutaneous involvement (p < 0.001), interstitial lung disease (p < 0.002), as well as higher rates of anti-topoisomerase I, "late" scleroderma pattern at nailfold videocapillaroscopy. These findings were confirmed by multivariate analysis. Conclusion: Our data provide a picture on the Italian use of intravenous iloprost among systemic sclerosis patients and showed that it was usually employed in patients with a more aggressive spectrum of the disease. The disparity of intravenous iloprost treatment strategies

Published
2024

33. Iloprost use and medical management of systemic sclerosis-related vasculopathy in Italian tertiary referral centers: results from the PROSIT study

Author

Negrini, Simone, Magnani, Ottavia, Matucci-Cerinic, Marco, Carignola, Renato, Data, Valeria, Montabone, Erika, Santaniello, Alessandro, Adorni, Giuditta, Murdaca, Giuseppe, Puppo, Francesco, Indiveri, Francesco, Della Rossa, Alessandra, D’Ascanio, Anna, Barsotti, Simone, Giuggioli, Dilia, Ferri, Clodoveo, Lumetti, Federica, Bosello, Silvia Laura, Canestrari, Giovanni, Bellando Randone, Silvia, Bruni, Cosimo, Guiducci, Serena, Battaglia, Elisabetta, De Andres, Maria Ilenia, Russo, Alessandra Azzurra, and Beretta, Lorenzo

Published
2019
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36. Significant nailfold capillary loss and late capillaroscopic pattern are associated with pulmonary arterial hypertension in systemic sclerosis.

Author

Angelis, Rossella De, Riccieri, Valeria, Cipolletta, Edoardo, Papa, Nicoletta Del, Ingegnoli, Francesca, Bosello, Silvia, Spinella, Amelia, Pellegrino, Greta, Pinto, Marco de, Papa, Silvia, Armentaro, Giuseppe, and Giuggioli, Dilia

Subjects
PULMONARY hypertension diagnosis, CAPILLARIES, CROSS-sectional method, ANGIOSCOPY, NAILS (Anatomy), DESCRIPTIVE statistics, CHI-squared test, ODDS ratio, SYSTEMIC scleroderma, CASE-control method, RESEARCH, CARDIOVASCULAR system physiology, CONFIDENCE intervals, BIOMARKERS, DISEASE complications
Abstract

Objective To evaluate differences in nailfold videocapillaroscopy (NVC) findings between SSc patients with and without a diagnosis of pulmonary arterial hypertension (PAH). Methods One hundred and ten SSc patients were enrolled in this cross-sectional, case–control, multicentre study. Patients were divided into cases (SSc-PAH confirmed by right heart catheterization) and controls (SSc-nonPAH with low probability of PAH). NVC patterns (early, active and late) and morphological parameters (microvascular density, non-specific abnormalities, giant capillaries, micro-haemorrhages, avascular areas) were considered using a semiquantitative scoring system. Results SSc-PAH patients showed higher frequencies of late pattern (P  < 0.01), non-specific abnormalities (P  < 0.01), lower capillary density (P  < 0.01), higher avascular areas (P  < 0.01) and a higher mean NVC score (P  < 0.01). Contrarily, the early/active pattern (P  < 0.01) and a higher rate of micro-haemorrhages (P  = 0.04) were more frequent in non-PAH patients. By a multivariate analysis, SSc-PAH patients, compared with non-PAH, had more non-specific abnormalities [27/55, 49.1% vs 10/55, 18.2%; adjusted odd ratio (OR) 16.89; 95% CI: 3.06, 93.16], a lower capillary density (grade 3, 20/55, 36.4% vs 5/55, 9.1%; adjusted OR 38.33; 95% CI: 2.34, 367.80) and avascular areas (18/55, 32.7% vs 10/55, 18.2%; adjusted OR 16.90; 95% CI: 2.64, 44.35). A correlation was found between the mean pulmonary arterial pressure and avascular areas (P  < 0.01), capillary density (P  < 0.01) and non-specific abnormalities (P  < 0.01). A clinical model including the NVC variables may be able to predict a diagnosis of PAH. Conclusion Our results indicate that the distinctive peripheral microcirculatory injury of SSc, i.e. capillary loss and morphological abnormalities, appear more severe and pronounced in patients with SSc-PAH. [ABSTRACT FROM AUTHOR]

Published
2024
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39. Long-Term Retention Rate of Tofacitinib in Rheumatoid Arthritis: An Italian Multicenter Retrospective Cohort Study

Author

Paroli, Marino, primary, Becciolini, Andrea, additional, Bravi, Elena, additional, Andracco, Romina, additional, Nucera, Valeria, additional, Parisi, Simone, additional, Ometto, Francesca, additional, Lumetti, Federica, additional, Farina, Antonella, additional, Del Medico, Patrizia, additional, Colina, Matteo, additional, Lo Gullo, Alberto, additional, Ravagnani, Viviana, additional, Scolieri, Palma, additional, Larosa, Maddalena, additional, Priora, Marta, additional, Visalli, Elisa, additional, Addimanda, Olga, additional, Vitetta, Rosetta, additional, Volpe, Alessandro, additional, Bezzi, Alessandra, additional, Girelli, Francesco, additional, Molica Colella, Aldo Biagio, additional, Caccavale, Rosalba, additional, Di Donato, Eleonora, additional, Adorni, Giuditta, additional, Santilli, Daniele, additional, Lucchini, Gianluca, additional, Arrigoni, Eugenio, additional, Platè, Ilaria, additional, Mansueto, Natalia, additional, Ianniello, Aurora, additional, Fusaro, Enrico, additional, Ditto, Maria Chiara, additional, Bruzzese, Vincenzo, additional, Camellino, Dario, additional, Bianchi, Gerolamo, additional, Serale, Francesca, additional, Foti, Rosario, additional, Amato, Giorgio, additional, De Lucia, Francesco, additional, Dal Bosco, Ylenia, additional, Foti, Roberta, additional, Reta, Massimo, additional, Fiorenza, Alessia, additional, Rovera, Guido, additional, Marchetta, Antonio, additional, Focherini, Maria Cristina, additional, Mascella, Fabio, additional, Bernardi, Simone, additional, Sandri, Gilda, additional, Giuggioli, Dilia, additional, Salvarani, Carlo, additional, Franchina, Veronica, additional, Molica Colella, Francesco, additional, Ferrero, Giulio, additional, and Ariani, Alarico, additional

Published
2023
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47. Systemic pharmacological treatment of digital ulcers in systemic sclerosis: a systematic literature review

Author

Ross, Laura, Maltez, Nancy, Hughes, Michael, Schoones, Jan W, Baron, Murray, Chung, Lorinda, Giuggioli, Dilia, Moinzadeh, Pia, Suliman, Yossra A, Campochiaro, Corrado, Allanore, Yannick, Denton, Christopher P, Distler, Oliver, Frech, Tracy, Furst, Daniel E, Khanna, Dinesh, Krieg, Thomas, Kuwana, Masataka, Matucci-Cerinic, Marco, Pope, Janet, Alunno, Alessia, and University of Zurich

Subjects
10051 Rheumatology Clinic and Institute of Physical Medicine, 610 Medicine & health
Published
2023

48. Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry ‘SPRING’ of the Italian Society for Rheumatology

Author

De Angelis, Rossella, primary, Ferri, Clodoveo, additional, Giuggioli, Dilia, additional, Bajocchi, Gianluigi, additional, Dagna, Lorenzo, additional, Bellando-Randone, Silvia, additional, Zanframundo, Giovanni, additional, Foti, Rosario, additional, Cacciapaglia, Fabio, additional, Cuomo, Giovanna, additional, Ariani, Alarico, additional, Rosato, Edoardo, additional, Lepri, Gemma, additional, Girelli, Francesco, additional, Riccieri, Valeria, additional, Zanatta, Elisabetta, additional, Bosello, Silvia Laura, additional, Cavazzana, Ilaria, additional, Ingegnoli, Francesca, additional, De Santis, Maria, additional, Murdaca, Giuseppe, additional, Abignano, Giuseppina, additional, Romeo, Nicoletta, additional, Della Rossa, Alessandra, additional, Caminiti, Maurizio, additional, Iuliano, Anna Maria, additional, Ciano, Giovanni, additional, Beretta, Lorenzo, additional, Bagnato, Gianluca, additional, Lubrano, Ennio, additional, De Andres, Ilenia, additional, Giollo, Alessandro, additional, Saracco, Marta, additional, Agnes, Cecilia, additional, Cipolletta, Edoardo, additional, Lumetti, Federica, additional, Spinella, Amelia, additional, Magnani, Luca, additional, Campochiaro, Corrado, additional, De Luca, Giacomo, additional, Codullo, Veronica, additional, Visalli, Elisa, additional, Di Vico, Claudio, additional, Gigante, Antonietta, additional, Pellagrino, Greta, additional, Pigatto, Erika, additional, Lazzaroni, Maria-Grazia, additional, Franceschini, Franco, additional, Generali, Elena, additional, Mennillo, Gianna, additional, Barsotti, Simone, additional, Mariano, Giuseppa Pagano, additional, Furini, Federica, additional, Vultaggio, Licia, additional, Parisi, Simone, additional, Peroni, Clara Lisa, additional, Rozza, Davide, additional, Zanetti, Anna, additional, Carrara, Greta, additional, Landolfi, Gianpiero, additional, Scirè, Carlo Alberto, additional, Bianchi, Gerolamo, additional, Fusaro, Enrico, additional, Sebastiani, Gian Domenico, additional, Govoni, Marcello, additional, D'Angelo, Salvatore, additional, Cozzi, Franco, additional, Guiducci, Serena, additional, Doria, Andrea, additional, Salvarani, Carlo, additional, Iannone, Florenzo, additional, and Matucci-Cerinic, Marco, additional

Published
2023
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49. Real-life efficacy and safety of nintedanib in systemic sclerosis-interstitial lung disease: data from an Italian multicentre study

Author

Campochiaro, Corrado, primary, De Luca, Giacomo, additional, Lazzaroni, Maria-Grazia, additional, Armentaro, Giuseppe, additional, Spinella, Amelia, additional, Vigone, Barbara, additional, Ruaro, Barbara, additional, Stanziola, Anna, additional, Benfaremo, Devis, additional, De Lorenzis, Enrico, additional, Moccaldi, Beatrice, additional, Bosello, Silvia Laura, additional, Cuomo, Giovanna, additional, Beretta, Lorenzo, additional, Zanatta, Elisabetta, additional, Giuggioli, Dilia, additional, Del Papa, Nicoletta, additional, Airo, Paolo, additional, Confalonieri, Marco, additional, Moroncini, Gianluca, additional, Dagna, Lorenzo, additional, and Matucci-Cerinic, Marco, additional

Published
2023
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