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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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4. 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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6. Prognostic Value of Lung Ultrasound B-Lines in Systemic Sclerosis

Author

Gargani, Luna, Bruni, Cosimo, Romei, Chiara, Frumento, Paolo, Moreo, Antonella, Agoston, Gergely, Guiducci, Serena, Bellando-Randone, Silvia, Lepri, Gemma, Belloli, Laura, Della Rossa, Alessandra, Delle Sedie, Andrea, Stagnaro, Chiara, De Nes, Michele, Salvadori, Stefano, Mosca, Marta, Falaschi, Fabio, Epis, Oscar, Picano, Eugenio, and Matucci-Cerinic, Marco

Published
2020
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7. 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

9. 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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11. Multiparametric Skin Assessment in a Monocentric Cohort of Systemic Sclerosis Patients: Is There a Role for Ultra-High Frequency Ultrasound?

Author

Di Battista, Marco, primary, Barsotti, Simone, additional, Vitali, Saverio, additional, Palma, Marco, additional, Granieri, Giammarco, additional, Oranges, Teresa, additional, Aringhieri, Giacomo, additional, Dini, Valentina, additional, Della Rossa, Alessandra, additional, Neri, Emanuele, additional, Romanelli, Marco, additional, and Mosca, Marta, additional

Published
2023
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12. Systemic sclerosis: one year in review 2023

Author

Di Battista, Marco, primary, Lepri, Gemma, additional, Codullo, Veronica, additional, Da Rio, Mattia, additional, Fiorentini, Elisa, additional, Della Rossa, Alessandra, additional, and Guiducci, Serena, additional

Published
2023
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15. Is oropharyngoesophageal scintigraphy the method of choice for assessing dysphagia in systemic sclerosis? A single center experience

Author

Di Battista, Marco, primary, Grosso, Mariano, additional, Da Rio, Mattia, additional, De Mattia, Giammarco, additional, Marciano, Andrea, additional, Valevich, Anastasiya, additional, Grosso, Antonio, additional, Morganti, Riccardo, additional, Volterrani, Duccio, additional, Della Rossa, Alessandra, additional, and Mosca, Marta, additional

Published
2023
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16. 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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17. Long-Term Data on Efficacy and Safety of Selexipag for Digital Systemic Sclerosis Vasculopathy

Author

Di Battista, Marco, Della Rossa, Alessandra, and Mosca, Marta

Abstract

ObjectiveRaynaud phenomenon (RP) and digital ulcers (DUs) are the main signs of digital vasculopathy in systemic sclerosis (SSc). Selexipag is an oral prostacyclin agonist approved for SSc-related pulmonary arterial hypertension. Following our previous preliminary short-course report, we herein present long-term data on selexipag safety and efficacy in the treatment of SSc digital vasculopathy.MethodsSelexipag was administered to patients with SSc with severe digital vasculopathy refractory or with contraindication to all other vasoactive therapies. Each subject was assessed at baseline and after 3, 6, and 12 months. Clinical outcomes related to RP and DUs were evaluated along with modified Rodnan skin score of the fingers. Digital perfusion was assessed by laser speckle contrast analysis (LASCA). Nailfold videocapillaroscopy (NVC) was also performed.ResultsEight patients with SSc (63% female, mean age 50.1 years) received selexipag. After 12 months of treatment, RP was reported to significantly decrease in the number of daily episodes and mean duration (P< 0.001 and P= 0.01, respectively). All patients achieved a complete healing of their DUs (P= 0.03) within 6 months. A progressive reduction of fingers skin score was observed (P= 0.03). No structural changes of capillaries were noted on NVC. Conversely, LASCA revealed an important increase in total digital perfusion (P= 0.004) despite seasonal variability. The safety profile was consistent with that reported in the literature.ConclusionWe observed a sustained efficacy of selexipag on SSc digital vasculopathy during 1 year of administration. Our promising results encourage the design of a new randomized controlled trial to evaluate the effect of selexipag on SSc digital vasculopathy.

Published
2024
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18. Sex-related Differences in Systemic Sclerosis: A Multicenter Cross-sectional Study From the National Registry of the Italian Society for Rheumatology

Author

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 Laura, Cavazzana, Ilaria, Ingegnoli, Francesca, Santis, Maria De, 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, Dall'Ara, Francesca, Lazzaroni, Maria Grazia, Generali, Elena, Mennillo, Gianna, Barsotti, Simone, Mariano, Giuseppa Pagano, Calabrese, Francesca, Furini, Federica, Vultaggio, Licia, Parisi, Simone, Peroni, Clara Lisa, Risa, Anna Maria, 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, Matucci-Cerinic, Marco, Ferri, Clodoveo, Bosello, Silvia (ORCID:0000-0002-4837-447X), 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 Laura, Cavazzana, Ilaria, Ingegnoli, Francesca, Santis, Maria De, 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, Dall'Ara, Francesca, Lazzaroni, Maria Grazia, Generali, Elena, Mennillo, Gianna, Barsotti, Simone, Mariano, Giuseppa Pagano, Calabrese, Francesca, Furini, Federica, Vultaggio, Licia, Parisi, Simone, Peroni, Clara Lisa, Risa, Anna Maria, 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, Matucci-Cerinic, Marco, Ferri, Clodoveo, and Bosello, Silvia (ORCID:0000-0002-4837-447X)

Abstract

Objective. There is still a great deal to learn about the influence of sex in systemic sclerosis (SSc). In this respect, national registries provide large and homogeneous patient cohorts for analytical studies. We therefore investigated a wide-ranging and well-characterized SSc series with the aim of identifying sex differences in disease expression, with a special focus on demographic, clinical, and serological characteristics.Methods. A multicenter SSc cohort of 2281 patients, including 247 men, was recruited in the Italian Systemic sclerosis PRogression INvestiGation (SPRING) registry. Demographic data, disease manifestations, serological profile, and internal organ involvement were compared.Results. The overall female/male ratio was 8.2:1. Female/male ratios for limited cutaneous SSc, diffuse cutaneous SSc, and SSc sine scleroderma subsets were 8.7:1, 4.9:1, and 10.7:1, respectively. A shorter time from onset of Raynaud phenomenon to SSc diagnosis, an increased prevalence of the diffuse cutaneous subset, renal crisis, and digital ulcers were found in males, whereas a significantly higher percentage of sicca syndrome, serum antinuclear antibodies, antiextractable nuclear antigens, anti-La/SSB, and anticentromere protein B was detected in the female group. Males exhibited lower left ventricular ejection fraction, as well as higher prevalence of conduction blocks, arrhythmias, ground glass, and honeycombing. Moreover, forced vital capacity and total lung capacity were medially lower in men than in women. Finally, males were more frequently treated with immunosuppressive drugs.Conclusion. Our study further supports the presence of several sex-related differences in patients with SSc. These differences were pronounced in the severity of cutaneous, peripheral vascular, and cardiopulmonary involvement for male patients, whereas an increased prevalence of sicca syndrome and a specific autoantibody profile characterized the female sex.

Published
2022

19. Systemic sclerosis: one year in review 2022

Author

Lepri, Gemma, primary, Orlandi, Martina, additional, Di Battista, Marco, additional, De Mattia, Gianmarco, additional, Da Rio, Mattia, additional, Codullo, Veronica, additional, Guiducci, Serena, additional, and Della Rossa, Alessandra, additional

Published
2022
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22. Contributors

Author

Abdellatif, Engy, primary, Abeles, Aryeh M., additional, Abelson, Abby G., additional, Abhishek, Abhishek, additional, Abramson, Steven B., additional, Adachi, Jonathan D., additional, Adams, Michael A., additional, Aigner, Thomas, additional, Akira, Shizuo, additional, Aletaha, Daniel, additional, Aliprantis, Antonios O., additional, Almeida de Jesus, Adriana, additional, Altman, Roy D., additional, Amigo, Mary-Carmen, additional, Aringer, Martin, additional, Ascherman, Dana P., additional, Assassi, Shervin, additional, Atamas, Sergei P., additional, Azevedo, Pedro Ming, additional, Baer, Alan N., additional, Baeten, Dominique, additional, Baines, Colin, additional, Baker, Nancy A., additional, Balogh, Emese, additional, Balsa, Alejandro, additional, Baraliakos, Xenofon, additional, Bardin, Thomas, additional, Barnsley, Les, additional, Bathon, Joan M., additional, Bauch, Angela, additional, Belch, Jill J.F., additional, Bellamy, Nicholas, additional, Bellido, Teresita, additional, Benjamin, Michael, additional, Beresford, Michael W., additional, Berman, Brian, additional, Bermas, Bonnie Lee, additional, Bertsias, George, additional, Bilezikian, John P., additional, Bilginer, Yelda, additional, Birnbaum, Julius, additional, Bishop, Felicity L., additional, Bleasel, Jane F., additional, Böhm, Markus, additional, Bolster, Marcy B., additional, Bombardieri, Stefano, additional, Bonelli, Michael, additional, Bonnick, Sydney L., additional, Boumpas, Dimitrios T., additional, Bozec, Aline, additional, Brasington, Richard D., additional, Braun, Juergen, additional, Brown, Matthew A., additional, Bruce, Ian N., additional, Bugbee, William D., additional, Bukhari, Marwan A.S., additional, Burgos-Vargas, Rubén, additional, Burmester, Gerd-Rüdiger, additional, Burns, Jane C., additional, Burr, David B., additional, Buttgereit, Frank, additional, Bykerk, Vivian P., additional, Calabrese, Leonard H., additional, Callen, Jeffrey P., additional, Cavallo, Sabrina, additional, Cawston, Tim E., additional, Chandran, Vinod, additional, Chard, Michael Denis, additional, Chaudhary, Prateek, additional, Chen, Lan X., additional, Choi, Hyon K., additional, Choy, Ernest H., additional, Christopher-Stine, Lisa, additional, Chu, Alvina D., additional, Clauw, Daniel J., additional, Clements, Philip J., additional, Clowse, Megan E.B., additional, Coghlan, J. Gerry, additional, Conaghan, Philip G., additional, Cooper, Cyrus, additional, Costenbader, Karen H., additional, Creamer, Paul, additional, Crispín, José C., additional, Cronstein, Bruce N., additional, Cross, Raymond, additional, Cusano, Natalie E., additional, Cutolo, Maurizio, additional, D'Adamo, Chris, additional, D'Agati, Vivette, additional, D'Cruz, David P., additional, Dagfinrud, Hanne, additional, Daikh, David I., additional, Dalbeth, Nicola, additional, Dalton, Seamus E., additional, Dass, Shouvik, additional, Davis, Aileen M., additional, De Ceulaer, Karel, additional, Deal, Chad L., additional, Deane, Kevin D., additional, Della Rossa, Alessandra, additional, Dellaripa, Paul F., additional, Dennison, Elaine, additional, Denton, Christopher P., additional, Dieppe, Paul, additional, Doherty, Michael, additional, Dolan, Patricia, additional, Donn, Rachelle, additional, Dvorkina, Olga, additional, Dyer, George S.M., additional, Eastell, Richard, additional, Edwards, N. Lawrence, additional, Emery, Paul, additional, Erturan, Gurhan, additional, Espinoza, Luis R., additional, Eyre, Steve, additional, Fanouriakis, Antonios C., additional, Farber, Joshua, additional, Farrokhi, Shawn, additional, Fasth, Anders, additional, Feist, Eugen, additional, Feldman, Debbie, additional, Felson, David T., additional, Fisk, John D., additional, Fitzgerald, G. Kelley, additional, Flores, Raymond H., additional, Fox, David A., additional, Francomano, Clair A., additional, Frangos, Jennifer, additional, Freemont, Anthony J., additional, Fricka, Kevin B., additional, Furst, Daniel E., additional, Gabay, Cem, additional, Gadina, Massimo, additional, Gaston, J.S. Hill, additional, Gay, Steffen, additional, Gensler, Lianne S., additional, Geraldino-Pardilla, Laura, additional, Gerlag, Danielle M., additional, Ginzler, Ellen M., additional, Gizinski, Alison M., additional, Gladman, Dafna D., additional, Gold, Garry E., additional, Goldbach-Mansky, Raphaela, additional, Goldingay, Sarah, additional, Gordon, Sharon M., additional, Gorodkin, Rachel, additional, Goronzy, Jörg J., additional, Görtz, Simon, additional, Grahame, Rodney, additional, Grainger, Andrew J., additional, Gravallese, Ellen M., additional, Greenberg, Jeffrey D., additional, Gujar, Bansari, additional, Hagan, Matilda, additional, Hagley, Karlene, additional, Hakim, Alan J., additional, Hall, John C., additional, Hamuryudan, Vedat, additional, Hanly, John G., additional, Hanson, Eric P., additional, Haraoui, Boulos, additional, Harley, John B., additional, Hashkes, Philip J., additional, Hawker, Gillian A., additional, Hawkins, Philip N., additional, Heiberg, Turid, additional, Heinegård, Dick, additional, Helfgott, Simon M., additional, Ho, Pauline Y.P., additional, Hochberg, Marc C., additional, Hochman, Jacqueline, additional, Hodgkiss-Harlow, Chelsea J., additional, Hoffman, Robert W., additional, Hoffmann, Markus, additional, Holers, V. Michael, additional, Holick, Michael F., additional, Holroyd, Christopher, additional, Hübscher, Osvaldo, additional, Hunter, David J., additional, Husni, M. Elaine, additional, Inman, Robert D., additional, Isaac, Zacharia, additional, Iversen, Maura D., additional, Jabs, Douglas A., additional, Jackson, William, additional, Jaimungal, Sarada, additional, James, Judith A., additional, Javier, Rose-Marie, additional, Johnsen, Alyssa K., additional, Jordan, Joanne M., additional, Kaisho, Tsuneyasu, additional, Kallenberg, Cees G.M., additional, Kane, David, additional, Kapoor, Mohit, additional, Karlson, Elizabeth W., additional, Kassimos, Dimitrios G., additional, Kastner, Daniel L., additional, Katz, Jeffrey N., additional, Kay, Jonathan, additional, Kelly, Jennifer A., additional, Keystone, Edward, additional, Khamashta, Munther A., additional, Khanna, Dinesh, additional, Kjeken, Ingvild, additional, Koch, Alisa E., additional, Koff, Matthew F., additional, Kottyan, Leah, additional, Koutsogeorgopoulou, Loukia A., additional, Kraus, Virginia Byers, additional, Kumar, Pradeep, additional, Kvien, Tore K., additional, Lafyatis, Robert, additional, Landewé, Robert B.M., additional, Langford, Carol A., additional, Lau, Arthur N., additional, Laxer, Ronald M., additional, Learch, Thomas J., additional, Lewith, George, additional, Li, Yi, additional, Liao, Katherine P., additional, Littlejohn, Geoffrey, additional, Lorenzo, Pilar, additional, Luger, Thomas A., additional, Lundberg, Ingrid E., additional, Lundberg, Karin, additional, Machold, Klaus P., additional, MacKenzie, C. Ronald, additional, Mahr, Alfred D., additional, Manheimer, Eric, additional, Marini, Joan C., additional, Marquez, Javier, additional, Marsden, Debbie, additional, Martel-Pelletier, Johanne, additional, Martín-Mola, Emilio, additional, Martínez-Lavín, Manuel, additional, Massarotti, Elena M., additional, Matteson, Eric L., additional, Matzat, Stephen J., additional, Mayahi, Reza, additional, Mayes, Maureen Davidica, additional, McAlindon, Timothy, additional, McBain, Hayley, additional, McCarberg, Bill, additional, McCarthy, Edward F., additional, McCarthy, Geraldine, additional, McDermott, Michael F., additional, McGonagle, Dennis, additional, McLean, Lachy, additional, Merkel, Peter A., additional, Mikdashi, Jamal A., additional, Miller, Frederick W., additional, Miller, Paul D., additional, Minden, Kirsten, additional, Monach, Paul A., additional, Mulligan, Kathleen, additional, Namur, Gauthier, additional, Naredo, Esperanza, additional, Naylor, Kim E., additional, Nelson, Amanda E., additional, Newman, Stanton P., additional, Nordal, Ellen, additional, Nöth, Ulrich, additional, Ntatsaki, Eleana, additional, O'Shea, John J., additional, Oddis, Chester V., additional, Olivé, Alejandro, additional, Omair, Mohammed A., additional, Ombrello, Michael J., additional, Omisade, Antonina, additional, Ong, Voon H., additional, Önnerfjord, Patrik, additional, Orcel, Philippe, additional, Ospelt, Caroline, additional, Ozen, Seza, additional, Paget, Stephen A., additional, Patel, Dipak R., additional, Patrono, Carlo, additional, Pelletier, Jean-Pierre, additional, Pereira, Rosa Maria Rodrigues, additional, Pilkington, Clarissa A., additional, Pillinger, Michael H., additional, Pineda, Carlos, additional, Plenge, Robert M., additional, Price, Andrew, additional, Pricop, Luminita, additional, Rackwitz, Lars, additional, Ravelli, Angelo, additional, Redmond, Anthony C., additional, Reeves, Westley H., additional, Remmers, Elaine F., additional, Requena, Luis, additional, Ribbens, Clio, additional, Richardson, Bruce C., additional, Riera Alonso, Elena, additional, Riley, Graham, additional, Ritchlin, Christopher, additional, Ritter, Susan Y., additional, Rosas, Ivan O., additional, Rowan, Drew D., additional, Rudwaleit, Martin, additional, Rull, Marina, additional, Rygg, Marite, additional, Saag, Kenneth G., additional, Salmon, Jane E., additional, Salter, Donald M., additional, Salzberg, Daniel J., additional, Sambrook, Philip N., additional, Saxne, Tore, additional, Schaible, Hans-Georg, additional, Scher, Jose U., additional, Schett, Georg, additional, Schmitz, Nicole, additional, Schreiber, Benjamin, additional, Schumacher, H. Ralph, additional, Schwartz, Daniella Muallem, additional, Scott, David G.I., additional, Seton, Margaret, additional, Shapiro, Lauren M., additional, Sharby, Nancy, additional, Siegel, Jeffrey, additional, Siegel, Richard M., additional, Sieper, Joachim, additional, Silver, Richard M., additional, Silverberg, Shonni J., additional, Simard, Julia F., additional, Simmons, Barry P., additional, Simms, Robert W., additional, Singer, Nora G., additional, Smith, Malcolm D., additional, Smith, Stacy E., additional, Smolen, Josef S., additional, Spector, Tim D., additional, Steen, Virginia D., additional, Steere, Allen C., additional, Steiner, Günter, additional, Steinert, Andre F., additional, Stojan, George, additional, Stone, John H., additional, Strand, Vibeke, additional, Straub, Rainer H., additional, Streeten, Elizabeth A., additional, Superti-Furga, Giulio, additional, Symmons, Deborah P.M., additional, Szekanecz, Zoltan, additional, Tak, Paul P., additional, Tavoni, Antonio, additional, Taylor, Peter C., additional, Terkeltaub, Robert, additional, Thomas, Sarah S., additional, Thorne, Jennifer E., additional, Tobias, Jonathan H., additional, Tremoulet, Adriana H., additional, Tsokos, George C., additional, Tuan, Rocky S., additional, Turesson, Carl, additional, Unizony, Sebastian H., additional, Valdes, Ana M., additional, van den Berg, Wim B., additional, van der Heijde, Désirée, additional, van Vollenhoven, Ronald Frits, additional, Varga, John, additional, Vassilopoulos, Dimitrios, additional, Vital, Edward M., additional, Walker-Bone, Karen, additional, Wallace, Daniel J., additional, Warburton, Gary, additional, Ward, Robert J., additional, Watts, Richard, additional, Wechalekar, Mihir D., additional, Wedderburn, Lucy R., additional, Weinblatt, Michael E., additional, Weir, Matthew R., additional, Wenham, Claire Y.J., additional, West, Sterling G., additional, Weyand, Cornelia M., additional, White, Kenneth E., additional, Winthrop, Kevin L., additional, Wong, John B., additional, Woolf, Anthony D., additional, Worthington, Jane, additional, Xu, Huji, additional, Yazici, Hasan, additional, Young, D.A., additional, Yurdakul, Sebahattin, additional, Zhang, Yuqing, additional, and Zhuang, Haoyang, additional

Published
2015
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23. COVID-19 and systemic sclerosis: clinicopathological implications from Italian nationwide survey study

Author

Ferri, Clodoveo, Giuggioli, Dilia, Raimondo, Vincenzo, Dagna, Lorenzo, Riccieri, Valeria, Zanatta, Elisabetta, Guiducci, Serena, Tavoni, Antonio, Foti, Rosario, Cuomo, Giovanna, De Angelis, Rossella, Cozzi, Franco, Murdaca, Giuseppe, Cavazzana, Ilaria, Romeo, Nicoletta, Codullo, Veronica, Ingegnoli, Francesca, Pellegrini, Roberta, Varcasia, Giuseppe, Della Rossa, Alessandra, De Santis, Maria, Abignano, Giuseppina, Colaci, Michele, Caminiti, Maurizio, L'Andolina, Massimo, Lubrano, Ennio, Spinella, Amelia, Lumetti, Federica, De Luca, Giacomo, Bellando-Randone, Silvia, Visalli, Elisa, Bilia, Silvia, Giannini, Daiana, Masini, Francesco, Pellegrino, Greta, Pigatto, Erika, Generali, Elena, Dall'Ara, Francesca, Pagano Mariano, Giuseppa, Barsotti, Simone, Pettiti, Giorgio, Zanframundo, Giovanni, Brittelli, Raffaele, Aiello, Vincenzo, Scorpiniti, Daniela, Ferrari, Tommaso, Caminiti, Rodolfo, Campochiaro, Corrado, Cecchetti, Riccardo, Gigliotti, Pietro, Olivo, Domenico, Ursini, Francesco, Brusi, Veronica, Meliconi, Riccardo, Scarpa, Raffaele, Fusaro, Enrico, Zignego, Anna Linda, D'Angelo, Salvatore, Iannone, Florenzo, Matucci-Cerinic, Marco, Doria, Andrea, Miccoli, Mario, Paparo, Sabrina Rosaria, Ragusa, Francesca, Elia, Giusy, Ferrari, Silvia Martina, Fallahi, Poupak, Antonelli, Alessandro, Rossa, Alessandra Della, and Mariano, Giuseppa Pagano

Published
2021
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26. Systemic sclerosis Progression INvestiGation (SPRING) Italian registry: demographic and clinico-serological features of scleroderma spectrum

Author

Ferri, Clodoveo, Giuggioli, Dilia, Guiducci, Serena, Lumetti, Federica, Bajocchi, Gianluigi, Magnani, Luca, Codullo, Veronica, Ariani, Alarico, Girelli, Francesco, Riccieri, Valeria, Pellegrino, Greta, Bosello, Silvia, Foti, Rosario, Visalli, Elisa, Amato, Giorgio, Benenati, Alessia, Cuomo, Giovanna, Iannone, Florenzo, Cacciapaglia, Fabio, Angelis, Rossella, Ingegnoli, Francesca, ROSSELLA TALOTTA, Campochiaro, Corrado, Dagna, Lorenzo, Luca, Giacomo, Bellando-Randone, Silvia, Spinella, Amelia, Murdaca, Giuseppe, Romeo, Nicoletta, Santis, Maria, Generali, Elena, Barsotti, Simone, Della Rossa, Alessandra, Cavazzana, Ilaria, Dall Ara, Francesca, Lazzaroni, Maria G., Cozzi, Franco, Andrea Doria, Pigatto, Erika, ELISABETTA ZANATTA, Ciano, Giovanni, Beretta, Lorenzo, Abignano, Giuseppina, D Angelo, Salvatore, Mennillo, Gianna, Bagnato, Gianluca, Calabrese, Francesca, Caminiti, Maurizio, Pagano Mariano, Giuseppa, Battaglia, Elisabetta, Lubrano, Ennio, Zanframundo, Giovanni, Iuliano, Annamaria, Furini, Federica, Zanetti, Anna, Carrara, Greta, Rumi, Federica, Scirè, Carlo Alberto, Matucci-Cerinic, Marco, Ferri, C, Giuggioli, D, Guiducci, S, Lumetti, F, Bajocchi, G, Magnani, L, Codullo, V, Ariani, A, Girelli, F, Riccieri, V, Pellegrino, G, Bosello, S, Foti, R, Visalli, E, Amato, G, Benenati, A, Cuomo, G, Iannone, F, Cacciapaglia, F, De Angelis, R, Ingegnoli, F, Talotta, R, Campochiaro, C, Dagna, L, De Luca, G, Bellando-Randone, S, Spinella, A, Murdaca, G, Romeo, N, De Santis, M, Generali, E, Barsotti, S, Della Rossa, A, Cavazzana, I, Dall'Ara, F, Lazzaroni, M, Cozzi, F, Doria, A, Pigatto, E, Zanatta, E, Ciano, G, Beretta, L, Abignano, G, D'Angelo, S, Mennillo, G, Bagnato, G, Calabrese, F, Caminiti, M, Pagano Mariano, G, Battaglia, E, Lubrano, E, Zanframundo, G, Iuliano, A, Furini, F, Zanetti, A, Carrara, G, Rumi, F, Scirè, C, Matucci-Cerinic, M, Ferri, Clodoveo, Giuggioli, Dilia, Guiducci, Serena, Lumetti, Federica, Bajocchi, Gianluigi, Magnani, Luca, Codullo, Veronica, Ariani, Alarico, Girelli, Francesco, Riccieri, Valeria, Pellegrino, Greta, Bosello, Silvia, Foti, Rosario, Visalli, Elisa, Amato, Giorgio, Benenati, Alessia, Cuomo, Giovanna, Iannone, Florenzo, Cacciapaglia, Fabio, De Angelis, Rossella, Ingegnoli, Francesca, Talotta, Rossella, Campochiaro, Corrado, Dagna, Lorenzo, De Luca, Giacomo, Bellando-Randone, Silvia, Spinella, Amelia, Murdaca, Giuseppe, Romeo, Nicoletta, De Santis, Maria, Generali, Elena, Barsotti, Simone, Della Rossa, Alessandra, Cavazzana, Ilaria, Dall'Ara, Francesca, Lazzaroni, Maria G, Cozzi, Franco, Doria, Andrea, Pigatto, Erika, Zanatta, Elisabetta, Ciano, Giovanni, Beretta, Lorenzo, Abignano, Giuseppina, D'Angelo, Salvatore, Mennillo, Gianna, Bagnato, Gianluca, Calabrese, Francesca, Caminiti, Maurizio, Pagano Mariano, Giuseppa, Battaglia, Elisabetta, Lubrano, Ennio, Zanframundo, Giovanni, Iuliano, Annamaria, Furini, Federica, Zanetti, Anna, Carrara, Greta, Rumi, Federica, Scirè, Carlo Alberto, and Matucci-Cerinic, Marco

Subjects
Male, Scleroderma, Systemic, Settore MED/16 - REUMATOLOGIA, systemic sclerosis, Raynaud’s phenomenon, VEDOSS, Raynaud Disease, Microscopic Angioscopy, Cohort Studies, Systemic sclerosi, Italy, Humans, Registries, Systemic sclerosis
Abstract

Objectives: Systemic sclerosis (SSc) is a severe multiple-organ disease characterised by unpredictable clinical course, inadequate response to treatment, and poor prognosis. National SSc registries may provide large and representative patients cohorts required for descriptive and prognostic studies. Therefore, the Italian Society for Rheumatology promoted the registry SPRING (Systemic sclerosis Progression INvestiGation). Methods: The SPRING is a multi-centre rheumatological cohort study encompassing the wide scleroderma spectrum, namely the primary Raynaud's phenomenon (pRP), suspected secondary RP, Very Early Diagnosis of Systemic Sclerosis (VEDOSS), and definite SSc. Here we describe the demographic and clinical characteristics of a population of 2,028 Italian patients at the initial phase of enrolment, mainly focusing on the cohort of 1,538 patients with definite SSc. Results: Definite SSc showed a significantly higher prevalence of digital ulcers, capillaroscopic 'late' pattern, oesophageal and cardio-pulmonary involvement compared to VEDOSS, as expected on the basis of the followed classification criteria. The in-depth analysis of definite SSc revealed that male gender, diffuse cutaneous subset, and anti-Scl70 seropositivity were significantly associated with increased prevalence of the most harmful disease manifestations. Similarly, patients with very short RP duration (≤1 year) at SSc diagnosis showed a statistically increased prevalence of unfavourable clinico-serological features. Conclusions: Nationwide registries with suitable subsetting of patients and follow-up studies since the prodromal phase of the disease may give us valuable insights into the SSc natural history and main prognostic factors.

Published
2020

29. One year in review 2021: systemic sclerosis

Author

Di Battista, Marco, primary, Barsotti, Simone, additional, Orlandi, Martina, additional, Lepri, Gemma, additional, Codullo, Veronica, additional, Della Rossa, Alessandra, additional, Guiducci, Serena, additional, and Del Galdo, Francesco, additional

Published
2021
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30. Impact of Joint Involvement on Patients Reported Outcomes in Systemic Lupus Erythematosus

Author

Carli Linda, Talarico Rosaria, Vagnani Sabrina, Mosca Marta, Baldini Chiara, Della Rossa Alessandra, Delle Sedie Andrea, Bombardieri Stefano, Riente Lucrezia, and Tani Chiara

Subjects
Adult, musculoskeletal diseases, 0301 basic medicine, medicine.medical_specialty, Visual analogue scale, Arthritis, ultrasounds, Young Adult, 03 medical and health sciences, 0302 clinical medicine, systemic lupus erythematosus, Rheumatology, Quality of life, medicine, Humans, Lupus Erythematosus, Systemic, In patient, Patient Reported Outcome Measures, Patient reported outcomes, Aged, Vas score, 030203 arthritis & rheumatology, business.industry, Morning stiffness, Middle Aged, medicine.disease, Arthralgia, joint involvement, 030104 developmental biology, Joint involvement, Quality of Life, Physical therapy, Female, General health, business
Abstract

Objective Joint involvement is common among patients with systemic lupus erythematosus (SLE). Aim of this work was to evaluate the correlation between the presence of joint involvement and patient-reported pain, perception of disease activity, general health and quality of life. Methods Fifty consecutive SLE patients were enrolled in the study. All patients underwent a complete clinical evaluation including a 44-joint count; in addition, an ultrasound evaluation of joint involvement of hands and wrists was performed. The following patients reported outcomes (PROs) were completed: Visual Analog Scales 0-100 mm (VAS) evaluating patients reported pain, patient's perception of global disease activity and general health (GH) and a validated Italian version of the Health Assessment Questionnaire (HAQ). Results Fourteen patients (28%) reported a significant morning stiffness lasting for more than 30 minutes; hand or wrist arthritis was clinically detectable in 10 (20%) patients, while the US evaluation exhibited at least one joint or tendon pathology in 18 patients (36%). The mean VAS score for pain and disease activity perception was 27 (±27.7) mm and 25.3 (±25.2) mm, respectively, the mean of GH score was 33.2 (±24.3) mm, and the mean HAQ score was 0.34 (±0.5). A significant correlation was observed between VAS score for pain, patient's perception of disease activity and GH and the presence of arthritis. Conclusion PROs may play an important role in guiding therapeutic decisions and suggest the utility of ultrasound evaluation in patients reporting articular symptoms.

Published
2018
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34. Management of digital ulcers in systemic sclerosis

Author

Barsotti,Simone, Di Battista,Marco, Venturini,Valentina, Della Rossa,Alessandra, and Mosca,Marta

Subjects
Chronic Wound Care Management and Research, integumentary system
Abstract

Simone Barsotti, Marco Di Battista, Valentina Venturini, Alessandra Della Rossa, Marta MoscaRheumatology Unit, Pisa University Hospital, Pisa, ItalyAbstract: Systemic sclerosis (SSc) is a rare disease characterized by autoimmune pathogenesis, alterations to the vascular system, and fibrosis of the skin and internal organs. Digital ulcers (DUs) are common in SSc patients, and represent a major burden for the patients. The management of DUs in systemic sclerosis is difficult, because it needs a multimodal therapeutic approach, as local treatment alone is usually insufficient and also systemic treatment with vasoactive drugs and modification of the lifestyle are usually required for healing of the wounds. In this review, we describe the optimal management of DUs according to recent literature and our clinical practice for systemic and local treatment of chronic digital wounds in SSc patients.Keywords: systemic sclerosis, wound, digital ulcers, dressings &nbsp

Published
2019

37. Systemic sclerosis: state of the art on clinical practice guidelines

Author

Smith, Vanessa, primary, Scirè, Carlo Alberto, additional, Talarico, Rosaria, additional, Airo, Paolo, additional, Alexander, Tobias, additional, Allanore, Yannick, additional, Bruni, Cosimo, additional, Codullo, Veronica, additional, Dalm, Virgil, additional, De Vries-Bouwstra, Jeska, additional, Della Rossa, Alessandra, additional, Distler, Oliver, additional, Galetti, Ilaria, additional, Launay, David, additional, Lepri, Gemma, additional, Mathian, Alexis, additional, Mouthon, Luc, additional, Ruaro, Barbara, additional, Sulli, Alberto, additional, Tincani, Angela, additional, Vandecasteele, Els, additional, Vanhaecke, Amber, additional, Vanthuyne, Marie, additional, Van den Hoogen, Frank, additional, Van Vollenhoven, Ronald, additional, Voskuyl, Alexandre E, additional, Zanatta, Elisabetta, additional, Bombardieri, Stefano, additional, Burmester, Gerd, additional, Fonseca, João Eurico, additional, Frank, Charissa, additional, Hachulla, Eric, additional, Houssiau, Frederic, additional, Mueller-Ladner, Ulf, additional, Schneider, Matthias, additional, van Laar, Jacob M, additional, Vieira, Ana, additional, Cutolo, Maurizio, additional, Mosca, Marta, additional, and Matucci-Cerinic, Marco, additional

Published
2018
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38. Systemic sclerosis chronic ulcers: preliminary results of treatment with allogenic skin grafting in a cohort of Italian patients

Author

Barsotti, Simone, Mattaliano, Vincenzo, d'Ascanio, Anna, Mosti, Giovanni, Della Rossa, Alessandra, Mattaliano, Chiara, Giuggioli, Dilia, Giraldi, Enzo, Ferri, Clodoveo, and Mosca, Marta

Subjects
Adult, Aged, 80 and over, Male, Scleroderma, Systemic, Skin Transplantation, Middle Aged, Cohort Studies, Italy, Humans, Transplantation, Homologous, Aged, Female, Retrospective Studies, Letters to the Editor
Published
2016

40. Systemic vasculitis: an annual critical digest of the most recent literature

Author

Della Rossa, Alessandra, Cioffi, Elisa, Elefante, Elena, Ferro, Francesco, Parma, Alice, Vagelli, Roberta, and Talarico, Rosaria

Subjects
Monitoring, Medication Therapy Management, Giant Cell Arteritis, Systemic Vasculitis, Antineutrophil Cytoplasmic, Disease Management, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Antibodies, Epidemiologic Studies, Cryoglobulinemia, Monoclonal, Diagnosis, Differential, Humans, Immunologic Factors, Antibodies, Antineutrophil Cytoplasmic, Antibodies, Monoclonal, Diagnosis, Differential, Monitoring, Physiologic, Randomized Controlled Trials as Topic, Physiologic
Published
2014

41. Improving organisation to improve care: ERN ReCONNET organisational reference model for systemic sclerosis patients’ care pathway

Author

Talarico, Rosaria, Marinello, Diana, Palla, Ilaria, Cannizzo, Sara, Galetti, Ilaria, Farrington, Sue, Aguilera, Silvia, Andersen, Jeanette, Ceccatelli, Eva, Cornet, Alain, Cutillas, Gema, Esteves, Marco, Frank, Charissa, Leite, Catarina, Niehaus, Gabi, Perez Gomez, Elisabeth, Polfliet, Katleen, Sandulescu, Silvia, Schriemer, Rita, Barsotti, Simone, Bellando-Randone, Silvia, Beretta, Lorenzo, Bernardino, Vera, Boleto, Goncalo, Bombardieri, Stefano, Burmester, Gerd, Cavazzana, Ilaria, Codullo, Veronica, Cutolo, Maurizio, Dalm, Virgil, Damian, Laura, Della Rossa, Alessandra, Doria, Andrea, Farhat, Meryem-Maud, Fonseca, João Eurico, Hachulla, Eric, Houssiau, Frédéric, Grazia Lazzaroni, Maria, Limper, Maarten, Lorenzoni, Valentina, Montecucco, Carlomaurizio, Mosca, Marta, Mouthon, Luc, Müeller-Ladner, Ulf, Pha, Micheline, Ponte, Cristina, Spierings, Julia, Sulli, Alberto, Taulaigo, Anna Viola, Ticciati, Simone, Tincani, Angela, Toplak, Natasha, Trieste, Leopoldo, van Hagen, P.M., van Laar, Jacob, Vanthuyne, Marie, Vigone, Barbara, de Vries-Bouwstra, Jeska K., Zen, Margherita, Turchetti, Giuseppe, Smith, Vanessa, and Matucci Cerinic, Marco

Abstract

Objective: To optimise the organisation of care and encourage the adoption of good clinical practices, the RarERN Path©methodology was designed within ERN ReCONNET. The aim of our work was to report the application of RarERN Path©on systemic sclerosis within the ERN ReCONNET centres, providing a feasible and flexible organisational reference model for optimising the systemic sclerosis care pathway in different countries.Methods: RarERN Path©is a six-phase methodology which enables the creation of a reference organisational model co-designed on the basis of the expertise of different stakeholders. It foresees six phases, ranging from the map of existing patients’ care pathways and patients’ stories, to the consensus on a common organisational patient care pathways, and its key performance indicators definition.Results: The agreed reference model highlights the importance of having an organisational flow for referrals that foresees how patients may access directly the specialised unit from the different referrals. Specific specialised visits were considered as mandatory to be organised and they included cardiologist, pneumologist, gastroenterologist, psychologist, nephrologist, dermatologist, wound care specialist/nurses and other healthcare professionals (such as nurses, social workers and nutritional counselling). Moreover, specific services related to therapy were highlighted as strongly recommended to be organised, mainly represented by infusion therapy and wound care, as well as occupation therapy and physiotherapy.Conclusion: The organisational model emerged from our investigation emphasises that the organisation of specific services for systemic sclerosis treatment should be organised as a solid support for implementing the existing recommendations on systemic sclerosis management in real life.

Published
2024
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43. COLABORADORES

Author

Abdellatif, Engy, Abeles, Aryeh M., Abelson, Abby G., Abhishek, Abhishek, Abramson, Steven B., Adachi, Jonathan D., Adams, Michael A., Aigner, Thomas, Akira, Shizuo, Aletaha, Daniel, Aliprantis, Antonios O., Almeida de Jesus, Adriana, Altman, Roy D., Amigo, Mary-Carmen, Aringer, Martin, Ascherman, Dana P., Assassi, Shervin, Atamas, Sergei P., Azevedo, Pedro Ming, Sr., Baer, Alan N., Baeten, Dominique, Baines, Colin, Baker, Nancy A., Balogh, Emese, Balsa, Alejandro, Baraliakos, Xenofon, Bardin, Thomas, Barnsley, Les, Bathon, Joan M., Bauch, Angela, Belch, Jill J.F., Bellamy, Nicholas, Bellido, Teresita, Benjamin, Michael, Beresford, Michael W., Berman, Brian, Bermas, Bonnie Lee, Bertsias, George, Bilezikian, John P., Bilginer, Yelda, Birnbaum, Julius, Bishop, Felicity L., Bleasel, Jane F., Böhm, Markus, Bolster, Marcy B., Bombardieri, Stefano, Bonelli, Michael, Bonnick, Sydney L., Boumpas, Dimitrios T., Bozec, Aline, Brasington, Richard D., Jr., Braun, Juergen, Brown, Matthew A., Bruce, Ian N., Bugbee, William D., Bukhari, Marwan A.S., Burgos-Vargas, Rubén, Burmester, Gerd-Rüdiger, Burns, Jane C., Burr, David B., Buttgereit, Frank, Bykerk, Vivian P., Calabrese, Leonard H., Callen, Jeffrey P., Cavallo, Sabrina, Cawston, Tim E., Chandran, Vinod, Chard, Michael Denis, Chaudhary, Prateek, Chen, Lan X., Choi, Hyon K., Choy, Ernest H., Christopher-Stine, Lisa, Chu, Alvina D., Clauw, Daniel J., Clements, Philip J., Clowse, Megan E.B., Coghlan, J. Gerry, Conaghan, Philip G., Cooper, Cyrus, Costenbader, Karen H., Creamer, Paul, Crispín, José C., Cronstein, Bruce N., Cross, Raymond, Cusano, Natalie E., Cutolo, Maurizio, D'Adamo, Chris, D'Agati, Vivette, D'Cruz, David P., Dagfinrud, Hanne, Daikh, David I., Dalbeth, Nicola, Dalton, Seamus E., Dass, Shouvik, Davis, Aileen M., De Ceulaer, Karel, Deal, Chad L., Deane, Kevin D., Della Rossa, Alessandra, Dellaripa, Paul F., Dennison, Elaine, Denton, Christopher P., Dieppe, Paul, Doherty, Michael, Dolan, Patricia, Donn, Rachelle, Dvorkina, Olga, Dyer, George S.M., Eastell, Richard, Edwards, N. Lawrence, Emery, Paul, Erturan, Gurhan, Espinoza, Luis R., Eyre, Steve, Fanouriakis, Antonios C., Farber, Joshua, Farrokhi, Shawn, Fasth, Anders, Feist, Eugen, Feldman, Debbie, Felson, David T., Fisk, John D., Fitzgerald, G. Kelley, Flores, Raymond H., Fox, David A., Francomano, Clair A., Frangos, Jennifer, Freemont, Anthony J., Fricka, Kevin B., Furst, Daniel E., Gabay, Cem, Gadina, Massimo, Gaston, J.S. Hill, Gay, Steffen, Gensler, Lianne S., Geraldino-Pardilla, Laura, Gerlag, Danielle M., Ginzler, Ellen M., Gizinski, Alison M., Gladman, Dafna D., Gold, Garry E., Goldbach-Mansky, Raphaela, Goldingay, Sarah, Gordon, Sharon M., Gorodkin, Rachel, Goronzy, Jörg J., Görtz, Simon, Grahame, Rodney, Grainger, Andrew J., Gravallese, Ellen M., Greenberg, Jeffrey D., Gujar, Bansari, Hagan, Matilda, Hagley, Karlene, Hakim, Alan J., Hall, John C., Hamuryudan, Vedat, Hanly, John G., Hanson, Eric P., Haraoui, Boulos, Harley, John B., Hashkes, Philip J., Hawker, Gillian A., Hawkins, Philip N., Heiberg, Turid, Heinegård, †Dick, Helfgott, Simon M., Ho, Pauline Y.P., Hochberg, Marc C., Hochman, Jacqueline, Hodgkiss-Harlow, Chelsea J., Hoffman, Robert W., Hoffmann, Markus, Holers, V. Michael, Holick, Michael F., Holroyd, Christopher, Hübscher, Osvaldo, Hunter, David J., Husni, M. Elaine, Inman, Robert D., Isaac, Zacharia, Iversen, Maura D., Jabs, Douglas A., Jackson, William, Jaimungal, Sarada, James, Judith A., Javier, Rose-Marie, Johnsen, Alyssa K., Jordan, Joanne M., Kaisho, Tsuneyasu, Kallenberg, Cees G.M., Kane, David, Kapoor, Mohit, Karlson, Elizabeth W., Kassimos, Dimitrios G., Kastner, Daniel L., Katz, Jeffrey N., Kay, Jonathan, Kelly, Jennifer A., Keystone, Edward, Khamashta, Munther A., Khanna, Dinesh, Kjeken, Ingvild, Koch, Alisa E., Koff, Matthew F., Kottyan, Leah, Koutsogeorgopoulou, Loukia A., Kraus, Virginia Byers, Kumar, Pradeep, Kvien, Tore K., Lafyatis, Robert, Landewé, Robert B.M., Langford, Carol A., Lau, Arthur N., Laxer, Ronald M., Learch, Thomas J., Lewith, George, Li, Yi, Liao, Katherine P., Littlejohn, Geoffrey, Lorenzo, Pilar, Luger, Thomas A., Lundberg, Ingrid E., Lundberg, Karin, Machold, Klaus P., MacKenzie, C. Ronald, Mahr, Alfred D., Manheimer, Eric, Marini, Joan C., Marquez, Javier, Marsden, Debbie, Martel-Pelletier, Johanne, Martín-Mola, Emilio, Martínez-Lavín, Manuel, Massarotti, Elena M., Matteson, Eric L., Matzat, Stephen J., Mayahi, Reza, Mayes, Maureen Davidica, McAlindon, Timothy, McBain, Hayley, McCarberg, Bill, McCarthy, Edward F., McCarthy, Geraldine, McDermott, Michael F., McGonagle, Dennis, McLean, Lachy, Merkel, Peter A., Mikdashi, Jamal A., Miller, Frederick W., Miller, Paul D., Minden, Kirsten, Monach, Paul A., Mulligan, Kathleen, Namur, Gauthier, Naredo, Esperanza, Naylor, Kim E., Nelson, Amanda E., Newman, Stanton P., Nordal, Ellen, Nöth, Ulrich, Ntatsaki, Eleana, O'Shea, John J., Oddis, Chester V., Olivé, Alejandro, Omair, Mohammed A., Ombrello, Michael J., Omisade, Antonina, Ong, Voon H., Önnerfjord, Patrik, Orcel, Philippe, Ospelt, Caroline, Ozen, Seza, Paget, Stephen A., Patel, Dipak R., Patrono, Carlo, Pelletier, Jean-Pierre, Pereira, Rosa Maria Rodrigues, Pilkington, Clarissa A., Pillinger, Michael H., Pineda, Carlos, Plenge, Robert M., Price, Andrew, Pricop, Luminita, Rackwitz, Lars, Ravelli, Angelo, Redmond, Anthony C., Reeves, Westley H., Remmers, Elaine F., Requena, Luis, Ribbens, Clio, Richardson, Bruce C., Riera Alonso, Elena, Riley, Graham, Ritchlin, Christopher, Ritter, Susan Y., Rosas, Ivan O., Rowan, Drew D., Rudwaleit, Martin, Rull, Marina, Rygg, Marite, Saag, Kenneth G., Salmon, Jane E., Salter, Donald M., Salzberg, Daniel J., Sambrook, †Philip N., Saxne, Tore, Schaible, Hans-Georg, Scher, Jose U., Schett, Georg, Schmitz, Nicole, Schreiber, Benjamin, Schumacher, H. Ralph, Jr., Schwartz, Daniella Muallem, Scott, David G.I., Seton, Margaret, Shapiro, Lauren M., Sharby, Nancy, Siegel, Jeffrey, Siegel, Richard M., Sieper, Joachim, Silver, Richard M., Silverberg, Shonni J., Simard, Julia F., Simmons, Barry P., Simms, Robert W., Singer, Nora G., Smith, Malcolm D., Smith, Stacy E., Smolen, Josef S., Spector, Tim D., Steen, Virginia D., Steere, Allen C., Steiner, Günter, Steinert, Andre F., Stojan, George, Stone, John H., Strand, Vibeke, Straub, Rainer H., Streeten, Elizabeth A., Superti-Furga, Giulio, Symmons, Deborah P.M., Szekanecz, Zoltan, Tak, Paul P., Tavoni, Antonio, Taylor, Peter C., Terkeltaub, Robert, Thomas, Sarah S., Thorne, Jennifer E., Tobias, Jonathan H., Tremoulet, Adriana H., Tsokos, George C., Tuan, Rocky S., Turesson, Carl, Unizony, Sebastian H., Valdes, Ana M., van den Berg, Wim B., van der Heijde, Désirée, van Vollenhoven, Ronald Frits, Varga, John, Vassilopoulos, Dimitrios, Vital, Edward M., Walker-Bone, Karen, Wallace, Daniel J., Warburton, Gary, Ward, Robert J., Watts, Richard, Wechalekar, Mihir D., Wedderburn, Lucy R., Weinblatt, Michael E., Weir, Matthew R., Sr., Wenham, Claire Y.J., West, Sterling G., Weyand, Cornelia M., White, Kenneth E., Winthrop, Kevin L., Wong, John B., Woolf, Anthony D., Worthington, Jane, Xu, Huji, Yazici, Hasan, Young, D.A., Yurdakul, Sebahattin, Zhang, Yuqing, and Zhuang, Haoyang

Published
2016

45. A proposal of simple calculation (ERI calculator) to predict the early response to TNF-α blockers therapy in rheumatoid arthritis

Author

Bazzichi, Laura, primary, Rossi, Paolo, additional, Giacomelli, Camillo, additional, De Feo, Francesca, additional, Bobbio-Pallavicini, Francesca, additional, Rossi, Alessandra, additional, Baldini, Chiara, additional, Consensi, Arianna, additional, Doveri, Marica, additional, Bonino, Claudia, additional, Mazzantini, Maurizio, additional, Della Rossa, Alessandra, additional, Montecucco, Carlomaurizio, additional, and Bombardieri, Stefano, additional

Published
2010
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47. Diagnosis and referral of rheumatoid arthritis by primary care physician: results of a pilot study on the city of Pisa, Italy

Author

Della Rossa, Alessandra, primary, Neri, Rossella, additional, Talarico, Rosaria, additional, Doveri, Marica, additional, Consensi, Arianna, additional, Salvadori, Stefano, additional, Lorenzoni, Valentina, additional, Turchetti, Giuseppe, additional, Bellelli, Stefania, additional, Cazzato, Massimiliano, additional, Bazzichi, Laura, additional, Monicelli, Paolo, additional, Moscardini, Stefano, additional, and Bombardieri, Stefano, additional

Published
2009
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49. TNF-alpha inhibitors in Systemic Lupus Erythematosus. A case report and a systematic literature review.

Author

Mosca, Marta, Tani, Chiara, Filice, Maria Elena, Carli, Linda, Delle Sedie, Andrea, Vagnani, Sabrina, Della Rossa, Alessandra, Baldini, Chiara, and Bombardieri, Stefano

Subjects
SYSTEMIC lupus erythematosus, SYNOVITIS, JOINT diseases, ARTHRITIS, METHOTREXATE
Abstract

Joint involvement is a common manifestation of systemic lupus erythematosus (SLE) and is described as a non-erosive mild synovitis. However some SLE patients may present a more severe joint involvement requiring aggressive therapy. We describe the case of a SLE patient with a severe arthritis unresponsive to methotrexate, successfully treated with anti-TNF-alpha drug as induction therapy and we report the results of a systematic literature review on the use of TNF-alpha inhibitors in SLE. [ABSTRACT FROM AUTHOR]

Published
2015
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