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1. Does the impact of COVID-19 on patients with systemic sclerosis change over time?

Author

MS Reumatologie/Immunologie/Infectie, Infection & Immunity, Deibel, Elisabeth, Carreira, Patricia E, Vonk, Madelon, Del Papa, Nicoletta, Bečvář, Radim, Guillén-Del-Castillo, Alfredo, Campochiaro, Corrado, Poormoghim, Hadi, Liem, Sophie, Lazzaroni, Maria-Grazia, Giollo, Alessandro, Mekinian, Arsène, de Vries-Bouwstra, Jeska, De Santis, Maria, Balbir-Gurman, Alexandra, Mihai, Carina, De Luca, Giacomo, Moiseev, Sergey, Zanatta, Elisabetta, Foti, Rosario, Rednic, Simona, Denton, Christopher, Cutolo, Maurizio, Belloli, Laura, Airo, Paolo, Garzanova, Liudmila, Moroncini, Gianluca, İnanç, Murat, Panopoulos, Stylianos, Tandaipan, Jose-Luis, Chatelus, Emmanuel, Rosato, Edoardo, Kuwana, Masataka, Yavuz, Sule, Alegre-Sancho, Juan J, Smith, Vanessa, Szűcs, Gabriella, Henes, Joerg, Rodríguez-Pintó, Ignasi, Atzeni, Fabiola, Spierings, Julia, Truchetet, Marie-Elise, Milchert, Marcin, Brito de Araujo, Daniel, Riemekasten, Gabriela, Bernardino, Vera, Martin, Thierry, Del Galdo, Francesco, Vacca, Alessandra, Mendoza, Fabian, Midtvedt, Øyvind, Murdaca, Giuseppe, Santiago, Tânia, Codullo, Veronica, Cacciapaglia, Fabio, Walker, Ulrich, Brunborg, Cathrine, Tirelli, Francesca, Allanore, Yannick, Furst, Daniel E, Matucci, Marco, Gabrielli, Armando, Distler, Oliver, Hoffmann-Vold, Anna-Maria, MS Reumatologie/Immunologie/Infectie, Infection & Immunity, Deibel, Elisabeth, Carreira, Patricia E, Vonk, Madelon, Del Papa, Nicoletta, Bečvář, Radim, Guillén-Del-Castillo, Alfredo, Campochiaro, Corrado, Poormoghim, Hadi, Liem, Sophie, Lazzaroni, Maria-Grazia, Giollo, Alessandro, Mekinian, Arsène, de Vries-Bouwstra, Jeska, De Santis, Maria, Balbir-Gurman, Alexandra, Mihai, Carina, De Luca, Giacomo, Moiseev, Sergey, Zanatta, Elisabetta, Foti, Rosario, Rednic, Simona, Denton, Christopher, Cutolo, Maurizio, Belloli, Laura, Airo, Paolo, Garzanova, Liudmila, Moroncini, Gianluca, İnanç, Murat, Panopoulos, Stylianos, Tandaipan, Jose-Luis, Chatelus, Emmanuel, Rosato, Edoardo, Kuwana, Masataka, Yavuz, Sule, Alegre-Sancho, Juan J, Smith, Vanessa, Szűcs, Gabriella, Henes, Joerg, Rodríguez-Pintó, Ignasi, Atzeni, Fabiola, Spierings, Julia, Truchetet, Marie-Elise, Milchert, Marcin, Brito de Araujo, Daniel, Riemekasten, Gabriela, Bernardino, Vera, Martin, Thierry, Del Galdo, Francesco, Vacca, Alessandra, Mendoza, Fabian, Midtvedt, Øyvind, Murdaca, Giuseppe, Santiago, Tânia, Codullo, Veronica, Cacciapaglia, Fabio, Walker, Ulrich, Brunborg, Cathrine, Tirelli, Francesca, Allanore, Yannick, Furst, Daniel E, Matucci, Marco, Gabrielli, Armando, Distler, Oliver, and Hoffmann-Vold, Anna-Maria

Published
2024

2. Adherence to the Mediterranean Diet in Italian Patients With Systemic Sclerosis: An Epidemiologic Survey

Author

Natalello, Gerlando, Bosello, Silvia Laura, Campochiaro, Corrado, Abignano, Giuseppina, De Santis, Maria, Ferlito, Arianna, Karadağ, Duygu Temiz, Padula, Angela Anna, Cavalli, Giulio, D'Agostino, Maria Antonietta, Selmi, Carlo, Matucci-Cerinic, Marco, Dagna, Lorenzo, De Luca, Giacomo, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), D'Agostino, Maria Antonietta (ORCID:0000-0002-5347-0060), Natalello, Gerlando, Bosello, Silvia Laura, Campochiaro, Corrado, Abignano, Giuseppina, De Santis, Maria, Ferlito, Arianna, Karadağ, Duygu Temiz, Padula, Angela Anna, Cavalli, Giulio, D'Agostino, Maria Antonietta, Selmi, Carlo, Matucci-Cerinic, Marco, Dagna, Lorenzo, De Luca, Giacomo, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), and D'Agostino, Maria Antonietta (ORCID:0000-0002-5347-0060)

Abstract

ObjectiveSystemic sclerosis (SSc) is an orphan disease that can lead to severe involvement of the gastrointestinal tract with a significant impact on patients' quality of life (QoL). The Mediterranean diet (MD) was consistently demonstrated to have beneficial effects on chronic diseases based on biological bases. We aimed to evaluate the adherence to the MD of Italian patients with SSc to preliminarily assess its association with gastrointestinal symptoms and other disease features, mood, and QoL.MethodsIn this cross-sectional study, adherence to the MD was measured in 387 patients from four SSc Italian referral centers through the 14-item Mediterranean Diet Adherence Screener (14-MEDAS) questionnaire. We also registered patients' reported outcomes related to the QoL and mood.ResultsOverall, an optimal adherence to MD was observed in 14.7% of patients with SSc, a moderate adherence in 71.3%, and a low adherence in 14.0%. In univariate analysis, poor adherence to the MD was associated with a more prominent depressive mood, time missed at work, and perception of more severe Raynaud's phenomenon and digital ulcers, whereas the 14-MEDAS score inversely correlated with depression score and reflux.ConclusionIn our cohort of patients with SSc, overall adherence to MD was moderate. Patients with lower adherence to MD also reported worse outcomes related to QoL and mood. Administration of the 14-MEDAS could be a reasonable choice to assess adherence to the MD in patients with SSc. Future initiatives to study the role of MD in the management of patients with SSc are warranted.

Published
2024

3. Longitudinal Analysis of PUL 2.0 Domains in Ambulant and Non-Ambulant Duchenne Muscular Dystrophy Patients: How do they Change in Relation to Functional Ability?

Author

Pane, Marika, Coratti, Giorgia, Brogna, Claudia, Bovis, Francesca, D'Amico, Adele, Pegoraro, Elena, Bello, Luca, Sansone, Valeria, Albamonte, Emilio, Ferraroli, Elisabetta, Mazzone, Elena Stacy, Fanelli, Lavinia, Messina, Sonia, Catteruccia, Michela, Cicala, Gianpaolo, Ricci, Martina, Frosini, Silvia, De Luca, Giacomo, Rolle, Enrica, De Sanctis, Roberto, Forcina, Nicola, Norcia, Giulia, Passamano, Luigia, Gardani, Alice, Pini, Antonella, Monaco, Giulia, D'Angelo, Maria Grazia, Capasso, Anna, Leone, Daniela, Zanin, Riccardo, Vita, Gian Luca, Panicucci, Chiara, Bruno, Claudio, Mongini, Tiziana, Ricci, Federica, Berardinelli, Angela, Battini, Roberta, Masson, Riccardo, Baranello, Giovanni, Dosi, Claudia, Bertini, Enrico Silvio, Politano, Luisa, Mercuri, Eugenio Maria, Pane, Marika (ORCID:0000-0002-4851-6124), Coratti, Giorgia (ORCID:0000-0001-6666-5628), Bertini, Enrico, Mercuri, Eugenio (ORCID:0000-0002-9851-5365), Pane, Marika, Coratti, Giorgia, Brogna, Claudia, Bovis, Francesca, D'Amico, Adele, Pegoraro, Elena, Bello, Luca, Sansone, Valeria, Albamonte, Emilio, Ferraroli, Elisabetta, Mazzone, Elena Stacy, Fanelli, Lavinia, Messina, Sonia, Catteruccia, Michela, Cicala, Gianpaolo, Ricci, Martina, Frosini, Silvia, De Luca, Giacomo, Rolle, Enrica, De Sanctis, Roberto, Forcina, Nicola, Norcia, Giulia, Passamano, Luigia, Gardani, Alice, Pini, Antonella, Monaco, Giulia, D'Angelo, Maria Grazia, Capasso, Anna, Leone, Daniela, Zanin, Riccardo, Vita, Gian Luca, Panicucci, Chiara, Bruno, Claudio, Mongini, Tiziana, Ricci, Federica, Berardinelli, Angela, Battini, Roberta, Masson, Riccardo, Baranello, Giovanni, Dosi, Claudia, Bertini, Enrico Silvio, Politano, Luisa, Mercuri, Eugenio Maria, Pane, Marika (ORCID:0000-0002-4851-6124), Coratti, Giorgia (ORCID:0000-0001-6666-5628), Bertini, Enrico, and Mercuri, Eugenio (ORCID:0000-0002-9851-5365)

Abstract

Background: The performance of upper limb 2.0 (PUL) is widely used to assess upper limb function in DMD patients. The aim of the study was to assess 24 month PUL changes in a large cohort of DMD patients and to establish whether domains changes occur more frequently in specific functional subgroups. Methods: The PUL was performed in 311 patients who had at least one pair of assessments at 24 months, for a total of 808 paired assessments. Ambulant patients were subdivided according to the ability to walk: >350, 250-350, ≤250 meters. Non ambulant patients were subdivided according to the time since they lost ambulation: <1, 1-2, 2-5 or >5 years. Results: At 12 months, the mean PUL 2.0 change on all the paired assessments was -1.30 (-1.51--1.05) for the total score, -0.5 (-0.66--0.39) for the shoulder domain, -0.6 (-0.74--0.5) for the elbow domain and -0.1 (-0.20--0.06) for the distal domain.At 24 months, the mean PUL 2.0 change on all the paired assessments was -2.9 (-3.29--2.60) for the total score, -1.30 (-1.47--1.09) for the shoulder domain, -1.30 (-1.45--1.11) for the elbow domain and -0.4 (-1.48--1.29) for the distal domain.Changes at 12 and 24 months were statistically significant between subgroups with different functional abilities for the total score and each domain (p < 0.001). Conclusion: There were different patterns of changes among the functional subgroups in the individual domains. The time of transition, including the year before and after loss of ambulation, show the peak of negative changes in PUL total scores that reflect not only loss of shoulder but also of elbow activities. These results suggest that patterns of changes should be considered at the time of designing clinical trials.

Published
2023

4. Myelomonocytic cells in giant cell arteritis activate trained immunity programs sustaining inflammation and cytokine production

Author

Cantoni, E, Merelli, I, Stefanoni, D, Tomelleri, A, Campochiaro, C, Giordano, V, Panigada, M, Baldissera, E, Merlo Pich, L, Natoli, V, Ziogas, A, Domínguez-Andrés, J, De Luca, G, Mazza, D, Zambrano, S, Gnani, D, Ferrarini, M, Ferrero, E, Agresti, A, Vergani, B, Leone, B, Cenci, S, Ravelli, A, Matucci-Cerinic, M, D'Alessandro, A, Joosten, L, Dagna, L, Netea, M, Molteni, R, Cavalli, G, Cantoni, Eleonora, Merelli, Ivan, Stefanoni, Davide, Tomelleri, Alessandro, Campochiaro, Corrado, Giordano, Vito, Panigada, Maddalena, Baldissera, Elena M, Merlo Pich, Laura, Natoli, Valentina, Ziogas, Athanasios, Domínguez-Andrés, Jorge, De Luca, Giacomo, Mazza, Davide, Zambrano, Samuel, Gnani, Daniela, Ferrarini, Marina, Ferrero, Elisabetta, Agresti, Alessandra, Vergani, Barbara, Leone, Biagio Eugenio, Cenci, Simone, Ravelli, Angelo, Matucci-Cerinic, Marco, D'Alessandro, Angelo, Joosten, Leo A B, Dagna, Lorenzo, Netea, Mihai G, Molteni, Raffaella, Cavalli, Giulio, Cantoni, E, Merelli, I, Stefanoni, D, Tomelleri, A, Campochiaro, C, Giordano, V, Panigada, M, Baldissera, E, Merlo Pich, L, Natoli, V, Ziogas, A, Domínguez-Andrés, J, De Luca, G, Mazza, D, Zambrano, S, Gnani, D, Ferrarini, M, Ferrero, E, Agresti, A, Vergani, B, Leone, B, Cenci, S, Ravelli, A, Matucci-Cerinic, M, D'Alessandro, A, Joosten, L, Dagna, L, Netea, M, Molteni, R, Cavalli, G, Cantoni, Eleonora, Merelli, Ivan, Stefanoni, Davide, Tomelleri, Alessandro, Campochiaro, Corrado, Giordano, Vito, Panigada, Maddalena, Baldissera, Elena M, Merlo Pich, Laura, Natoli, Valentina, Ziogas, Athanasios, Domínguez-Andrés, Jorge, De Luca, Giacomo, Mazza, Davide, Zambrano, Samuel, Gnani, Daniela, Ferrarini, Marina, Ferrero, Elisabetta, Agresti, Alessandra, Vergani, Barbara, Leone, Biagio Eugenio, Cenci, Simone, Ravelli, Angelo, Matucci-Cerinic, Marco, D'Alessandro, Angelo, Joosten, Leo A B, Dagna, Lorenzo, Netea, Mihai G, Molteni, Raffaella, and Cavalli, Giulio

Abstract

Objective: Trained immunity (TI) is a de facto memory program of innate immune cells, characterized by immunometabolic and epigenetic changes sustaining enhanced production of cytokines. TI evolved as a protective mechanism against infections; however, inappropriate activation can cause detrimental inflammation and might be implicated in the pathogenesis of chronic inflammatory diseases. In this study, we investigated the role of TI in the pathogenesis of giant cell arteritis (GCA), a large-vessel vasculitis characterized by aberrant macrophage activation and excess cytokine production. Methods: Monocytes from GCA patients and from age- and sex-matched healthy donors were subjected to polyfunctional studies, including cytokine production assays at baseline and following stimulation, intracellular metabolomics, chromatin immunoprecipitation-qPCR, and combined ATAC/RNA sequencing. Immunometabolic activation (i.e. glycolysis) was assessed in inflamed vessels of GCA patients with FDG-PET and immunohistochemistry (IHC), and the role of this pathway in sustaining cytokine production was confirmed with selective pharmacologic inhibition in GCA monocytes. Results: GCA monocytes exhibited hallmark molecular features of TI. Specifically, these included enhanced IL-6 production upon stimulation, typical immunometabolic changes (e.g. increased glycolysis and glutaminolysis) and epigenetic changes promoting enhanced transcription of genes governing pro-inflammatory activation. Immunometabolic changes of TI (i.e. glycolysis) were a feature of myelomonocytic cells in GCA lesions and were required for enhanced cytokine production. Conclusions: Myelomonocytic cells in GCA activate TI programs sustaining enhanced inflammatory activation with excess cytokine production.

Published
2023

5. Longitudinal Analysis of PUL 2.0 Domains in Ambulant and Non-Ambulant Duchenne Muscular Dystrophy Patients: How do they Change in Relation to Functional Ability?

Author

Pane, Marika, Coratti, Giorgia, Brogna, Claudia, Bovis, Francesca, D'Amico, Adele, Pegoraro, Elena, Bello, Luca, Sansone, Valeria, Albamonte, Emilio, Ferraroli, Elisabetta, Mazzone, Elena Stacy, Fanelli, Lavinia, Messina, Sonia, Catteruccia, Michela, Cicala, Gianpaolo, Ricci, Martina, Frosini, Silvia, De Luca, Giacomo, Rolle, Enrica, De Sanctis, Roberto, Forcina, Nicola, Norcia, Giulia, Passamano, Luigia, Gardani, Alice, Pini, Antonella, Monaco, Giulia, D'Angelo, Maria Grazia, Capasso, Anna, Leone, Daniela, Zanin, Riccardo, Vita, Gian Luca, Panicucci, Chiara, Bissoli, Claudio Bruno, Mongini, Tiziana, Ricci, Federica, Berardinelli, Angela, Battini, Roberta, Masson, Riccardo, Baranello, Giovanni, Dosi, Claudia, Bertini, Enrico Silvio, Politano, Luisa, Mercuri, Eugenio Maria, Pane, Marika (ORCID:0000-0002-4851-6124), Coratti, Giorgia (ORCID:0000-0001-6666-5628), Bruno, Claudio, Bertini, Enrico, Mercuri, Eugenio (ORCID:0000-0002-9851-5365), Pane, Marika, Coratti, Giorgia, Brogna, Claudia, Bovis, Francesca, D'Amico, Adele, Pegoraro, Elena, Bello, Luca, Sansone, Valeria, Albamonte, Emilio, Ferraroli, Elisabetta, Mazzone, Elena Stacy, Fanelli, Lavinia, Messina, Sonia, Catteruccia, Michela, Cicala, Gianpaolo, Ricci, Martina, Frosini, Silvia, De Luca, Giacomo, Rolle, Enrica, De Sanctis, Roberto, Forcina, Nicola, Norcia, Giulia, Passamano, Luigia, Gardani, Alice, Pini, Antonella, Monaco, Giulia, D'Angelo, Maria Grazia, Capasso, Anna, Leone, Daniela, Zanin, Riccardo, Vita, Gian Luca, Panicucci, Chiara, Bissoli, Claudio Bruno, Mongini, Tiziana, Ricci, Federica, Berardinelli, Angela, Battini, Roberta, Masson, Riccardo, Baranello, Giovanni, Dosi, Claudia, Bertini, Enrico Silvio, Politano, Luisa, Mercuri, Eugenio Maria, Pane, Marika (ORCID:0000-0002-4851-6124), Coratti, Giorgia (ORCID:0000-0001-6666-5628), Bruno, Claudio, Bertini, Enrico, and Mercuri, Eugenio (ORCID:0000-0002-9851-5365)

Abstract

Background: The performance of upper limb 2.0 (PUL) is widely used to assess upper limb function in DMD patients. The aim of the study was to assess 24 month PUL changes in a large cohort of DMD patients and to establish whether domains changes occur more frequently in specific functional subgroups. Methods: The PUL was performed in 311 patients who had at least one pair of assessments at 24 months, for a total of 808 paired assessments. Ambulant patients were subdivided according to the ability to walk: >350, 250-350, =250 meters. Non ambulant patients were subdivided according to the time since they lost ambulation: <1, 1-2, 2-5 or >5 years. Results: At 12 months, the mean PUL 2.0 change on all the paired assessments was -1.30 (-1.51-1.05) for the total score, -0.5 (-0.66-0.39) for the shoulder domain, -0.6 (-0.74-0.5) for the elbow domain and -0.1 (-0.20-0.06) for the distal domain. At 24 months, the mean PUL 2.0 change on all the paired assessments was -2.9 (-3.29-2.60) for the total score, -1.30 (-1.47-1.09) for the shoulder domain, -1.30 (-1.45-1.11) for the elbow domain and -0.4 (-1.48--1.29) for the distal domain. Changes at 12 and 24 months were statistically significant between subgroups with different functional abilities for the total score and each domain (p < 0.001). Conclusion: There were different patterns of changes among the functional subgroups in the individual domains. The time of transition, including the year before and after loss of ambulation, show the peak of negative changes in PUL total scores that reflect not only loss of shoulder but also of elbow activities. These results suggest that patterns of changes should be considered at the time of designing clinical trials.

Published
2023

6. Primary systemic sclerosis heart involvement: A systematic literature review and preliminary data-driven, consensus-based WSF/HFA definition

Author

Bruni, Cosimo, Buch, Maya H., Furst, Daniel E., De Luca, Giacomo, Djokovic, Aleksandra, Dumitru, Raluca B., Giollo, Alessandro, Polovina, Marija, Steelandt, Alexia, Bratis, Kostantinos, Suliman, Yossra Atef, Milinkovic, Ivan, Baritussio, Anna, Hasan, Ghadeer, Xintarakou, Anastasia, Isomura, Yohei, Markousis-Mavrogenis, George, Tofani, Lorenzo, Mavrogeni, Sophie, Gargani, Luna, Caforio, Alida L. P., Tschoepe, Carsten, Ristic, Arsen, Klingel, Karin, Plein, Sven, Behr, Elijah R., Allanore, Yannick, Kuwana, Masataka, Denton, Christopher P., Khanna, Dinesh, Krieg, Thomas, Marcolongo, Renzo, Galetti, Ilaria, Zanatta, Elisabetta, Tona, Francesco, Seferovic, Petar, Matucci-Cerinic, Marco, Bruni, Cosimo, Buch, Maya H., Furst, Daniel E., De Luca, Giacomo, Djokovic, Aleksandra, Dumitru, Raluca B., Giollo, Alessandro, Polovina, Marija, Steelandt, Alexia, Bratis, Kostantinos, Suliman, Yossra Atef, Milinkovic, Ivan, Baritussio, Anna, Hasan, Ghadeer, Xintarakou, Anastasia, Isomura, Yohei, Markousis-Mavrogenis, George, Tofani, Lorenzo, Mavrogeni, Sophie, Gargani, Luna, Caforio, Alida L. P., Tschoepe, Carsten, Ristic, Arsen, Klingel, Karin, Plein, Sven, Behr, Elijah R., Allanore, Yannick, Kuwana, Masataka, Denton, Christopher P., Khanna, Dinesh, Krieg, Thomas, Marcolongo, Renzo, Galetti, Ilaria, Zanatta, Elisabetta, Tona, Francesco, Seferovic, Petar, and Matucci-Cerinic, Marco

Abstract

Introduction: Primary heart involvement in systemic sclerosis may cause morpho-functional and electrical cardiac abnormalities and is a common cause of death. The absence of a clear definition of primary heart involvement in systemic sclerosis limits our understanding and ability to focus on clinical research. We aimed to create an expert consensus definition for primary heart involvement in systemic sclerosis. Methods: A systematic literature review of cardiac involvement and manifestations in systemic sclerosis was conducted to inform an international and multi-disciplinary task force. In addition, the nominal group technique was used to derive a definition that was then subject to voting. A total of 16 clinical cases were evaluated to test face validity, feasibility, reliability and criterion validity of the newly created definition. Results: In total, 171 publications met eligibility criteria. Using the nominal group technique, experts added their opinion, provided statements to consider and ranked them to create the consensus definition, which received 100% agreement on face validity. A median 60(5-300) seconds was taken for the feasibility on a single case. Inter-rater agreement was moderate (mKappa (95% CI) = 0.56 (0.46-1.00) for the first round and 0.55 (0.44-1.00) for the second round) and intra-rater agreement was good (mKappa (95% CI) = 0.77 (0.47-1.00)). Criterion validity showed a 78 (73-84)% correctness versus gold standard. Conclusion: A preliminary primary heart involvement in systemic sclerosis consensus-based definition was created and partially validated, for use in future clinical research.

Published
2022

7. 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 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, 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, Matucci-Cerinic, Marco, Bosello, Silvia (ORCID:0000-0002-4837-447X), 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 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, 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, Matucci-Cerinic, Marco, and Bosello, Silvia (ORCID:0000-0002-4837-447X)

Abstract

Introduction: Systemic sclerosis (SSc) is characterized by a complex etiopathogenesis encompassing both host genetic and environmental -infectious/toxic- factors responsible for altered fibrogenesis and diffuse microangiopathy. A wide spectrum of clinical phenotypes may be observed in patients' populations from different geographical areas. We investigated the prevalence of specific clinical and serological phenotypes in patients with definite SSc enrolled at tertiary referral centres in different Italian geographical macro-areas. The observed findings were compared with those reported in the world literature.Materials and methods: The clinical features of 1538 patients (161 M, 10.5%; mean age 59.8 +/- 26.9 yrs.; mean disease duration 8.9 +/- 7.7 yrs) with definite SSc recruited in 38 tertiary referral centres of the SPRING (Systemic sclerosis Progression INvestiGation Group) registry promoted by Italian Society of Rheumatology (SIR) were obtained and clustered according to Italian geographical macroareas.Results: Patients living in Southern Italy were characterized by more severe clinical and/or serological SSc phenotypes compared to those in Northern and Central Italy; namely, they show increased percentages of diffuse cutaneous SSc, digital ulcers, sicca syndrome, muscle involvement, arthritis, cardiopulmonary symptoms, interstitial lung involvement at HRCT, as well increased prevalence of serum anti-Scl70 autoantibodies. In the same SSc population immunusppressive drugs were frequently employed. The review of the literature underlined the geographical heterogeneity of SSc phenotypes, even if the observed findings are scarcely comparable due to the variability of methodological approaches.Conclusion: The phenotypical differences among SSc patients' subgroups from Italian macro-areas might be correlated to genetic/environmental co-factors, and possibly to a not equally distributed national network of information and healthcare facilities.

Published
2022

8. Prevalence and Death Rate of COVID-19 in Autoimmune Systemic Diseases in the First Three Pandemic Waves. Relationship with Disease Subgroups and Ongoing Therapies

Author

Ferri, Clodoveo, Raimondo, Vincenzo, Gragnani, Laura, Giuggioli, Dilia, Dagna, Lorenzo, Tavoni, Antonio, Ursini, Francesco, L'Andolina, Massimo, Caso, Francesco, Ruscitti, Piero, Caminiti, Maurizio, Foti, Rosario, Riccieri, Valeria, Guiducci, Serena, Pellegrini, Roberta, Zanatta, Elisabetta, Varcasia, Giuseppe, Olivo, Domenico, Gigliotti, Pietro, Cuomo, Giovanna, Murdaca, Giuseppe, Cecchetti, Riccardo, De Angelis, Rossella, Romeo, Nicoletta, Ingegnoli, Francesca, Cozzi, Franco, Codullo, Veronica, Cavazzana, Ilaria, Colaci, Michele, Abignano, Giuseppina, De Santis, Maria, Lubrano, Ennio, Fusaro, Enrico, Spinella, Amelia, Lumetti, Federica, De Luca, Giacomo, Bellando-Randone, Silvia, Visalli, Elisa, Bosco, Ylenia Dal, Amato, Giorgio, Giannini, Daiana, Bilia, Silvia, Masini, Francesco, Pellegrino, Greta, Pigatto, Erika, Generali, Elena, Mariano, Giuseppa Pagano, Pettiti, Giorgio, Zanframundo, Giovanni, Brittelli, Raffaele, Aiello, Vincenzo, Caminiti, Rodolfo, Scorpiniti, Daniela, Ferrari, Tommaso, Campochiaro, Corrado, Brusi, Veronica, Fredi, Micaela, Moschetti, Liala, Cacciapaglia, Fabio, Paparo, Sabrina Rosaria, Ragusa, Francesca, Mazzi, Valeria, Elia, Giusy, Ferrari, Silvia Martina, Di Cola, Ilenia, Vadacca, Marta, Lorusso, Sebastiano, Monti, Monica, Lorini, Serena, Aprile, Maria Letizia, Tasso, Marco, Miccoli, Mario, Bosello, Silvia Laura, D'Angelo, Salvatore, Doria, Andrea, Franceschini, Franco, Meliconi, Riccardo, Matucci-Cerinic, Marco, Iannone, Florenzo, Giacomelli, Roberto, Salvarani, Carlo, Zignego, Anna Linda, Fallahi, Poupak, Antonelli, Alessandro, Bosello, Silvia (ORCID:0000-0002-4837-447X), Ferri, Clodoveo, Raimondo, Vincenzo, Gragnani, Laura, Giuggioli, Dilia, Dagna, Lorenzo, Tavoni, Antonio, Ursini, Francesco, L'Andolina, Massimo, Caso, Francesco, Ruscitti, Piero, Caminiti, Maurizio, Foti, Rosario, Riccieri, Valeria, Guiducci, Serena, Pellegrini, Roberta, Zanatta, Elisabetta, Varcasia, Giuseppe, Olivo, Domenico, Gigliotti, Pietro, Cuomo, Giovanna, Murdaca, Giuseppe, Cecchetti, Riccardo, De Angelis, Rossella, Romeo, Nicoletta, Ingegnoli, Francesca, Cozzi, Franco, Codullo, Veronica, Cavazzana, Ilaria, Colaci, Michele, Abignano, Giuseppina, De Santis, Maria, Lubrano, Ennio, Fusaro, Enrico, Spinella, Amelia, Lumetti, Federica, De Luca, Giacomo, Bellando-Randone, Silvia, Visalli, Elisa, Bosco, Ylenia Dal, Amato, Giorgio, Giannini, Daiana, Bilia, Silvia, Masini, Francesco, Pellegrino, Greta, Pigatto, Erika, Generali, Elena, Mariano, Giuseppa Pagano, Pettiti, Giorgio, Zanframundo, Giovanni, Brittelli, Raffaele, Aiello, Vincenzo, Caminiti, Rodolfo, Scorpiniti, Daniela, Ferrari, Tommaso, Campochiaro, Corrado, Brusi, Veronica, Fredi, Micaela, Moschetti, Liala, Cacciapaglia, Fabio, Paparo, Sabrina Rosaria, Ragusa, Francesca, Mazzi, Valeria, Elia, Giusy, Ferrari, Silvia Martina, Di Cola, Ilenia, Vadacca, Marta, Lorusso, Sebastiano, Monti, Monica, Lorini, Serena, Aprile, Maria Letizia, Tasso, Marco, Miccoli, Mario, Bosello, Silvia Laura, D'Angelo, Salvatore, Doria, Andrea, Franceschini, Franco, Meliconi, Riccardo, Matucci-Cerinic, Marco, Iannone, Florenzo, Giacomelli, Roberto, Salvarani, Carlo, Zignego, Anna Linda, Fallahi, Poupak, Antonelli, Alessandro, and Bosello, Silvia (ORCID:0000-0002-4837-447X)

Abstract

Objective: Autoimmune systemic diseases (ASD) represent a predisposing condition to COVID-19. Our prospective, observational multicenter telephone survey study aimed to investigate the prevalence, prognostic factors, and outcomes of COVID-19 in Italian ASD patients. Methods: The study included 3,918 ASD pts (815 M, 3103 F; mean age 59 +/- 12SD years) consecutively recruited between March 2020 and May 2021 at the 36 referral centers of COVID-19 and ASD Italian Study Group. The possible development of COVID-19 was recorded by means of a telephone survey using a standardized symptom assessment questionnaire. Results: ASD patients showed a significantly higher prevalence of COVID-19 (8.37% vs. 6.49%; p<0.0001) but a death rate statistically comparable to the Italian general population (3.65% vs. 2.95%). Among the 328 ASD patients developing COVID-19, 17% needed hospitalization, while mild-moderate manifestations were observed in 83% of cases. Moreover, 12/57 hospitalized patients died due to severe interstitial pneumonia and/or cardiovascular events; systemic sclerosis (SSc) patients showed a significantly higher COVID-19-related death rate compared to the general population (6.29% vs. 2.95%; p=0.018). Major adverse prognostic factors to develop COVID-19 were: older age, male gender, SSc, pre-existing ASD-related interstitial lung involvement, and long-term steroid treatment. Of note, patients treated with conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) showed a significantly lower prevalence of COVID-19 compared to those without (3.58% vs. 46.99%; p=0.000), as well as the SSc patients treated with low dose aspirin (with 5.57% vs. without 27.84%; p=0.000). Conclusion: During the first three pandemic waves, ASD patients showed a death rate comparable to the general population despite the significantly higher prevalence of COVID-19. A significantly increased COVID-19-related mortality was recorded in only SSc patients' subgroup, possibly favored

Published
2022

9. 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

10. Primary systemic sclerosis heart involvement: A systematic literature review and preliminary data-driven, consensus-based WSF/HFA definition

Author

Bruni, Cosimo, Buch, Maya H., Furst, Daniel E., De Luca, Giacomo, Djokovic, Aleksandra, Dumitru, Raluca B., Giollo, Alessandro, Polovina, Marija, Steelandt, Alexia, Bratis, Kostantinos, Suliman, Yossra Atef, Milinkovic, Ivan, Baritussio, Anna, Hasan, Ghadeer, Xintarakou, Anastasia, Isomura, Yohei, Markousis-Mavrogenis, George, Tofani, Lorenzo, Mavrogeni, Sophie, Gargani, Luna, Caforio, Alida L. P., Tschoepe, Carsten, Ristic, Arsen, Klingel, Karin, Plein, Sven, Behr, Elijah R., Allanore, Yannick, Kuwana, Masataka, Denton, Christopher P., Khanna, Dinesh, Krieg, Thomas, Marcolongo, Renzo, Galetti, Ilaria, Zanatta, Elisabetta, Tona, Francesco, Seferovic, Petar, Matucci-Cerinic, Marco, Bruni, Cosimo, Buch, Maya H., Furst, Daniel E., De Luca, Giacomo, Djokovic, Aleksandra, Dumitru, Raluca B., Giollo, Alessandro, Polovina, Marija, Steelandt, Alexia, Bratis, Kostantinos, Suliman, Yossra Atef, Milinkovic, Ivan, Baritussio, Anna, Hasan, Ghadeer, Xintarakou, Anastasia, Isomura, Yohei, Markousis-Mavrogenis, George, Tofani, Lorenzo, Mavrogeni, Sophie, Gargani, Luna, Caforio, Alida L. P., Tschoepe, Carsten, Ristic, Arsen, Klingel, Karin, Plein, Sven, Behr, Elijah R., Allanore, Yannick, Kuwana, Masataka, Denton, Christopher P., Khanna, Dinesh, Krieg, Thomas, Marcolongo, Renzo, Galetti, Ilaria, Zanatta, Elisabetta, Tona, Francesco, Seferovic, Petar, and Matucci-Cerinic, Marco

Abstract

Introduction: Primary heart involvement in systemic sclerosis may cause morpho-functional and electrical cardiac abnormalities and is a common cause of death. The absence of a clear definition of primary heart involvement in systemic sclerosis limits our understanding and ability to focus on clinical research. We aimed to create an expert consensus definition for primary heart involvement in systemic sclerosis. Methods: A systematic literature review of cardiac involvement and manifestations in systemic sclerosis was conducted to inform an international and multi-disciplinary task force. In addition, the nominal group technique was used to derive a definition that was then subject to voting. A total of 16 clinical cases were evaluated to test face validity, feasibility, reliability and criterion validity of the newly created definition. Results: In total, 171 publications met eligibility criteria. Using the nominal group technique, experts added their opinion, provided statements to consider and ranked them to create the consensus definition, which received 100% agreement on face validity. A median 60(5-300) seconds was taken for the feasibility on a single case. Inter-rater agreement was moderate (mKappa (95% CI) = 0.56 (0.46-1.00) for the first round and 0.55 (0.44-1.00) for the second round) and intra-rater agreement was good (mKappa (95% CI) = 0.77 (0.47-1.00)). Criterion validity showed a 78 (73-84)% correctness versus gold standard. Conclusion: A preliminary primary heart involvement in systemic sclerosis consensus-based definition was created and partially validated, for use in future clinical research.

Published
2022

11. The role of chest CT in deciphering interstitial lung involvement: systemic sclerosis versus COVID-19

Author

Orlandi, M, Landini, N, Sambataro, G, Nardi, C, Tofani, L, Bruni, C, Bellando-Randone, S, Blagojevic, J, Melchiorre, D, Hughes, M, Denton, C, Luppi, F, Ruaro, B, Della Casa, F, Rossi, F, De Luca, G, Campochiaro, C, Spinicci, M, Zammarchi, L, Tomassetti, S, Caminati, A, Cavigli, E, Albanesi, M, Melchiorre, F, Palmucci, S, Vegni, V, Guiducci, S, Moggi-Pignone, A, Allanore, Y, Bartoloni, A, Confalonieri, M, Dagna, L, De Cobelli, F, De Paulis, A, Harari, S, Khanna, D, Kuwana, M, Taliani, G, Lavorini, F, Miele, V, Morana, G, Pesci, A, Vancheri, C, Colagrande, S, Matucci-Cerinic, M, Orlandi, Martina, Landini, Nicholas, Sambataro, Gianluca, Nardi, Cosimo, Tofani, Lorenzo, Bruni, Cosimo, Bellando-Randone, Silvia, Blagojevic, Jelena, Melchiorre, Daniela, Hughes, Michael, Denton, Christopher P, Luppi, Fabrizio, Ruaro, Barbara, Della Casa, Francesca, Rossi, Francesca W, De Luca, Giacomo, Campochiaro, Corrado, Spinicci, Michele, Zammarchi, Lorenzo, Tomassetti, Sara, Caminati, Antonella, Cavigli, Edoardo, Albanesi, Marco, Melchiorre, Fabio, Palmucci, Stefano, Vegni, Virginia, Guiducci, Serena, Moggi-Pignone, Alberto, Allanore, Yannick, Bartoloni, Alessandro, Confalonieri, Marco, Dagna, Lorenzo, De Cobelli, Francesco, De Paulis, Amato, Harari, Sergio, Khanna, Dinesh, Kuwana, Masataka, Taliani, Gloria, Lavorini, Federico, Miele, Vittorio, Morana, Giovanni, Pesci, Alberto, Vancheri, Carlo, Colagrande, Stefano, Matucci-Cerinic, Marco, Orlandi, M, Landini, N, Sambataro, G, Nardi, C, Tofani, L, Bruni, C, Bellando-Randone, S, Blagojevic, J, Melchiorre, D, Hughes, M, Denton, C, Luppi, F, Ruaro, B, Della Casa, F, Rossi, F, De Luca, G, Campochiaro, C, Spinicci, M, Zammarchi, L, Tomassetti, S, Caminati, A, Cavigli, E, Albanesi, M, Melchiorre, F, Palmucci, S, Vegni, V, Guiducci, S, Moggi-Pignone, A, Allanore, Y, Bartoloni, A, Confalonieri, M, Dagna, L, De Cobelli, F, De Paulis, A, Harari, S, Khanna, D, Kuwana, M, Taliani, G, Lavorini, F, Miele, V, Morana, G, Pesci, A, Vancheri, C, Colagrande, S, Matucci-Cerinic, M, Orlandi, Martina, Landini, Nicholas, Sambataro, Gianluca, Nardi, Cosimo, Tofani, Lorenzo, Bruni, Cosimo, Bellando-Randone, Silvia, Blagojevic, Jelena, Melchiorre, Daniela, Hughes, Michael, Denton, Christopher P, Luppi, Fabrizio, Ruaro, Barbara, Della Casa, Francesca, Rossi, Francesca W, De Luca, Giacomo, Campochiaro, Corrado, Spinicci, Michele, Zammarchi, Lorenzo, Tomassetti, Sara, Caminati, Antonella, Cavigli, Edoardo, Albanesi, Marco, Melchiorre, Fabio, Palmucci, Stefano, Vegni, Virginia, Guiducci, Serena, Moggi-Pignone, Alberto, Allanore, Yannick, Bartoloni, Alessandro, Confalonieri, Marco, Dagna, Lorenzo, De Cobelli, Francesco, De Paulis, Amato, Harari, Sergio, Khanna, Dinesh, Kuwana, Masataka, Taliani, Gloria, Lavorini, Federico, Miele, Vittorio, Morana, Giovanni, Pesci, Alberto, Vancheri, Carlo, Colagrande, Stefano, and Matucci-Cerinic, Marco

Abstract

Objective: The aim of this study was to identify the main CT features that may help in distinguishing a progression of interstitial lung disease (ILD) secondary to SSc from COVID-19 pneumonia. Methods: This multicentric study included 22 international readers grouped into a radiologist group (RADs) and a non-radiologist group (nRADs). A total of 99 patients, 52 with COVID-19 and 47 with SSc-ILD, were included in the study. Results: Fibrosis inside focal ground-glass opacities (GGOs) in the upper lobes; fibrosis in the lower lobe GGOs; reticulations in lower lobes (especially if bilateral and symmetrical or associated with signs of fibrosis) were the CT features most frequently associated with SSc-ILD. The CT features most frequently associated with COVID- 19 pneumonia were: consolidation (CONS) in the lower lobes, CONS with peripheral (both central/peripheral or patchy distributions), anterior and posterior CONS and rounded-shaped GGOs in the lower lobes. After multivariate analysis, the presence of CONs in the lower lobes (P < 0.0001) and signs of fibrosis in GGOs in the lower lobes (P < 0.0001) remained independently associated with COVID-19 pneumonia and SSc-ILD, respectively. A predictive score was created that was positively associated with COVID-19 diagnosis (96.1% sensitivity and 83.3% specificity). Conclusion: CT diagnosis differentiating between COVID-19 pneumonia and SSc-ILD is possible through a combination of the proposed score and radiologic expertise. The presence of consolidation in the lower lobes may suggest COVID-19 pneumonia, while the presence of fibrosis inside GGOs may indicate SSc-ILD.

Published
2022

12. The Role of the Multidisciplinary Health Care Team in the Management of Patients with Systemic Sclerosis

Author

Farina,Nicola, Benanti,Giovanni, De Luca,Giacomo, Palmisano,Anna, Peretto,Giovanni, Tomassetti,Sara, Giorgione,Veronica, Forma,Ornella, Esposito,Antonio, Danese,Silvio, Dagna,Lorenzo, Matucci-Cerinic,Marco, Campochiaro,Corrado, Farina,Nicola, Benanti,Giovanni, De Luca,Giacomo, Palmisano,Anna, Peretto,Giovanni, Tomassetti,Sara, Giorgione,Veronica, Forma,Ornella, Esposito,Antonio, Danese,Silvio, Dagna,Lorenzo, Matucci-Cerinic,Marco, and Campochiaro,Corrado

Abstract

Nicola Farina,1,2,&ast; Giovanni Benanti,1,2,&ast; Giacomo De Luca,1,2 Anna Palmisano,2,3 Giovanni Peretto,4 Sara Tomassetti,5 Veronica Giorgione,6 Ornella Forma,7 Antonio Esposito,2,3 Silvio Danese,2,8 Lorenzo Dagna,1,2 Marco Matucci-Cerinic,1,9 Corrado Campochiaro1,2 1Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy; 2School of Medicine, Vita-Salute San Raffaele University, Milan, Italy; 3Unit of Clinical and Experimental Radiology, IRCCS San Raffaele Hospital, Milan, Italy; 4Unit of Cardiac Electrophysiology and Arrhythmology, IRCCS San Raffaele Hospital, Milan, Italy; 5Department of Experimental and Clinical Medicine, Careggi University Hospital, Florence, Italy; 6Molecular and Clinical Sciences Research Institute, Fetal Medicine Unit, Department of Obstetrics and Gynaecology, St. George’s University Hospitals NHS Foundation Trust, London, UK; 7Vulnology Nursing Service, IRCCS San Raffaele Scientific Institute, Milan, Italy; 8Unit of Gastroenterology and Endoscopy, IRCCS San Raffaele Hospital, Milan, Italy; 9Department of Experimental and Clinical Medicine, University of Florence and Division of Rheumatology AOUC, Florence, Italy&ast;These authors contributed equally to this workCorrespondence: Corrado Campochiaro, Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Via Olgettina 60, Milan, 20132, Italy, Tel +39 0226437060, Fax +39 0226433729, Email campochiaro.corrado@hsr.itAbstract: Systemic sclerosis (SSc) is a rare connective tissue disease characterised by immune dysfunction, vascular damage and fibrosis affecting the skin and multiple internal organs. The clinical spectrum of SSc is wide and its manifestations may lead to severe morbidity and mortality, in addition to a great impact on patients’ quality of life. Due to the multifaceted clinical manifestations of SSc, its management requires a combined expertise of different medical specialists to gua

Published
2022

13. Covid-19 And Rheumatic Autoimmune Systemic Diseases: Role of Pre-Existing Lung Involvement and Ongoing Treatments

Author

Ferri, Clodoveo, Giuggioli, Dilia, Raimondo, Vincenzo, L'Andolina, Massimo, Dagna, Lorenzo, Tavoni, Antonio, Caso, Francesco, Ursini, Francesco, Ruscitti, Piero, Caminiti, Maurizio, Foti, Rosario, Riccieri, Valeria, Guiducci, Serena, Pellegrini, Roberta, Zanatta, Elisabetta, Varcasia, Giuseppe, Olivo, Domenico, Gigliotti, Pietro, Cuomo, Giovanna, Murdaca, Giuseppe, Cecchetti, Riccardo, De Angelis, Rossella, Romeo, Nicoletta, Ingegnoli, Francesca, Cozzi, Franco, Codullo, Veronica, Cavazzana, Ilaria, Colaci, Michele, Abignano, Giuseppina, De Santis, Maria, Lubrano, Ennio, Fusaro, Enrico, Rossa, Alessandra Della, Spinella, Amelia, Lumetti, Federica, De Luca, Giacomo, Bellando-Randone, Silvia, Visalli, Elisa, Dal Bosco, Ylenia, Amato, Giorgio, Giannini, Daiana, Bilia, Silvia, Masini, Francesco, Pellegrino, Greta, Pigatto, Erika, Generali, Elena, Mariano, Giuseppa Pagano, Pettiti, Giorgio, Zanframundo, Giovanni, Brittelli, Raffaele, Aiello, Vincenzo, Caminiti, Rodolfo, Scorpiniti, Daniela, Ferrari, Tommaso, Campochiaro, Corrado, Brusi, Veronica, Fredi, Micaela, Moschetti, Liala, Cacciapaglia, Fabio, Gragnani, Laura, Monti, Monica, Lorini, Serena, Paparo, Sabrina Rosaria, Ragusa, Francesca, Mazzi, Valeria, Elia, Giusy, Ferrari, Silvia Martina, Di Cola, Ilenia, Vadacca, Marta, Lorusso, Sebastiano, Barsotti, Simone, Aprile, Maria Letizia, Marco, Tasso, Miccoli, Mario, Bosello, Silvia Laura, Matucci-Cerinic, Marco, D'Angelo, Salvatore, Doria, Andrea, Franceschini, Franco, Meliconi, Riccardo, Iannone, Florenzo, Giacomelli, Roberto, Zignego, Anna Linda, Fallahi, Poupak, Antonelli, Alessandro, Bosello, Silvia (ORCID:0000-0002-4837-447X), Ferri, Clodoveo, Giuggioli, Dilia, Raimondo, Vincenzo, L'Andolina, Massimo, Dagna, Lorenzo, Tavoni, Antonio, Caso, Francesco, Ursini, Francesco, Ruscitti, Piero, Caminiti, Maurizio, Foti, Rosario, Riccieri, Valeria, Guiducci, Serena, Pellegrini, Roberta, Zanatta, Elisabetta, Varcasia, Giuseppe, Olivo, Domenico, Gigliotti, Pietro, Cuomo, Giovanna, Murdaca, Giuseppe, Cecchetti, Riccardo, De Angelis, Rossella, Romeo, Nicoletta, Ingegnoli, Francesca, Cozzi, Franco, Codullo, Veronica, Cavazzana, Ilaria, Colaci, Michele, Abignano, Giuseppina, De Santis, Maria, Lubrano, Ennio, Fusaro, Enrico, Rossa, Alessandra Della, Spinella, Amelia, Lumetti, Federica, De Luca, Giacomo, Bellando-Randone, Silvia, Visalli, Elisa, Dal Bosco, Ylenia, Amato, Giorgio, Giannini, Daiana, Bilia, Silvia, Masini, Francesco, Pellegrino, Greta, Pigatto, Erika, Generali, Elena, Mariano, Giuseppa Pagano, Pettiti, Giorgio, Zanframundo, Giovanni, Brittelli, Raffaele, Aiello, Vincenzo, Caminiti, Rodolfo, Scorpiniti, Daniela, Ferrari, Tommaso, Campochiaro, Corrado, Brusi, Veronica, Fredi, Micaela, Moschetti, Liala, Cacciapaglia, Fabio, Gragnani, Laura, Monti, Monica, Lorini, Serena, Paparo, Sabrina Rosaria, Ragusa, Francesca, Mazzi, Valeria, Elia, Giusy, Ferrari, Silvia Martina, Di Cola, Ilenia, Vadacca, Marta, Lorusso, Sebastiano, Barsotti, Simone, Aprile, Maria Letizia, Marco, Tasso, Miccoli, Mario, Bosello, Silvia Laura, Matucci-Cerinic, Marco, D'Angelo, Salvatore, Doria, Andrea, Franceschini, Franco, Meliconi, Riccardo, Iannone, Florenzo, Giacomelli, Roberto, Zignego, Anna Linda, Fallahi, Poupak, Antonelli, Alessandro, and Bosello, Silvia (ORCID:0000-0002-4837-447X)

Abstract

Background: The Covid-19 pandemic may have a deleterious impact on patients with autoimmune systemic diseases (ASD) due to their deep immune-system alterations. Objective: This study aims to investigate the prevalence of symptomatic Covid-19 and its correlations with both organ involvement and ongoing treatments in a large series of Italian ASD patients during the first wave of pandemic. Methods: Our multicenter telephone 6-week survey included 3,029 unselected ASD patients enrolled at 36 tertiary referral centers of northern, central, and southern Italian macro-areas with different diffusion of the pandemic. Symptomatic SARS-CoV-2 infection was classified as definite Covid-19 (presence of symptoms plus positive oral/nasopharyngeal swabs) or highly suspected Covid-19 (highly suggestive symptoms, in the absence of a swab testing). Results: A significantly higher prevalence of definite plus highly suspected Covid-19 compared to the Italian general population was detected in the whole ASD series (p=.000), as well as in patients from the three macro-areas (p=.000 in all). Statistically higher prevalence of Covid-19 was also found in connective tissue diseases compared to chronic arthritis subgroup (p=.000) and in ASD patients with pre-existing interstitial lung involvement (p=.000). Patients treated with either conventional disease-modifying anti-rheumatic drugs (DMARDs) and/or biological DMARDs showed a significantly lower prevalence of Covid-19 (p=.000 in both). Finally, scleroderma patients undergoing low-dose aspirin showed a significantly lower rate of Covid-19 compared to those without (p=0.003). Conclusion: The higher prevalence of Covid-19 in ASD patients, along with the significant correlations with important clinical features and therapeutic regimens, suggests the need to develop targeted prevention/management strategies during the current pandemic wave.

Published
2021

14. Weak States: Causes and Consequences of the Sicilian Mafia

Author

Massachusetts Institute of Technology. Department of Economics, Acemoglu, Daron, De Feo, Giuseppe, De Luca, Giacomo Davide, Massachusetts Institute of Technology. Department of Economics, Acemoglu, Daron, De Feo, Giuseppe, and De Luca, Giacomo Davide

Published
2021

15. Systemic sclerosis myocarditis has unique clinical, histological and prognostic features: a comparative histological analysis

Author

De Luca, Giacomo, Campochiaro, Corrado, De Santis, Maria, Sartorelli, Silvia, Peretto, Giovanni, Sala, Simone, Canestrari, Giovanni, De Lorenzis, Enrico, Basso, Cristina, Rizzo, Stefania, Thiene, Gaetano, Palmisano, Anna, Esposito, Antonio, Selmi, Carlo, Gremese, Elisa, Della Bella, Paolo, Dagna, Lorenzo, Bosello, Silvia Laura, Gremese, Elisa (ORCID:0000-0002-2248-1058), Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), De Luca, Giacomo, Campochiaro, Corrado, De Santis, Maria, Sartorelli, Silvia, Peretto, Giovanni, Sala, Simone, Canestrari, Giovanni, De Lorenzis, Enrico, Basso, Cristina, Rizzo, Stefania, Thiene, Gaetano, Palmisano, Anna, Esposito, Antonio, Selmi, Carlo, Gremese, Elisa, Della Bella, Paolo, Dagna, Lorenzo, Bosello, Silvia Laura, Gremese, Elisa (ORCID:0000-0002-2248-1058), and Bosello, Silvia Laura (ORCID:0000-0002-4837-447X)

Abstract

Objective. To outline the clinical, histological and prognostic features of systemic sclerosis (SSc) endomyocardial biopsy-proven myocarditis with respect to those of diverse endomyocardial biopsy-proven virus-negative myocarditis (VNM).Methods. We retrospectively analysed data from three cohorts of endomyocardial biopsy-proven myocarditis: SSc-related VNM (SSc-VNM); isolated VNM (i-VNM); and VNM related to other systemic autoimmune diseases (a-VNM). The degree of myocardial fibrosis was expressed as relative percentage and fibrotic score (0-3). Clinical data, cardiac enzymes, echocardiogram, 24 h ECG Holter and cardiac magnetic resonance were obtained at baseline and during follow-up. Non-parametric tests were used.Results. We enrolled 12 SSc-VNM [11 females, mean age 49.3 (14.2) years; seven diffuse-SSc, five early-SSc], 12 i-VNM [12 females, mean age 47.7 (10.8) years] and 10 a-VNM [four females, mean age 48.4 (16.3) years] patients. SSc patients had higher degrees of myocardial fibrosis as assessed by both percentage [SSc-VNM: 44.8 (18.8)%; a-VNM: 28.6 (16.5)%; i-VNM: 24.9 (10.3)%; P = 0.019] and score [SSc-VNM: 2.3 (0.8); a-VNM: 1.4 (1.1); i-VNM: 1.2 (0.7); P = 0.002]. Myocardial fibrosis directly correlated with skin score (r = 0.625, P = 0.03) and number of ventricular ectopic beats on 24 h ECG Holter in SSc patients (r = 0.756, P = 0.01). Dyspnoea class was higher at presentation in SSc-VNM patients (P = 0.041) and we found heart failure only in SSc patients (25%) (P = 0.05). At cardiac magnetic resonance, myocardial oedema was nearly undetectable in SSc-VNM patients compared with others (P = 0.02). All patients received immunosuppressive treatment. The number of patients who died during follow-up due to cardiac complications was significantly higher in SSc-VNM patients (50%), as compared with a-VNM (0%) and i-VNM (8.3%) patients (P = 0.006). Patients who died during follow-up had higher degrees of myocardial fibrosis [52.2 (11.6)% vs 27.5 (12.9)%, P = 0.024

Published
2020

16. Measuring the T-cell down-regulation of TCR-zeta, ZAP-70 and CD28 in arthritis patients: An old tool for new biomarkers

Author

Zizzo, Gaetano, De Santis, Maria, Bosello, Silvia Laura, Tolusso, Barbara, Alivernini, Stefano, De Luca, Giacomo, Gremese, Elisa, Ferraccioli, Gianfranco, Bosello, Silvia (ORCID:0000-0002-4837-447X), Tolusso, Barbara (ORCID:0000-0002-9108-6609), Alivernini, Stefano (ORCID:0000-0002-7383-4212), Gremese, Elisa (ORCID:0000-0002-2248-1058), Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), Zizzo, Gaetano, De Santis, Maria, Bosello, Silvia Laura, Tolusso, Barbara, Alivernini, Stefano, De Luca, Giacomo, Gremese, Elisa, Ferraccioli, Gianfranco, Bosello, Silvia (ORCID:0000-0002-4837-447X), Tolusso, Barbara (ORCID:0000-0002-9108-6609), Alivernini, Stefano (ORCID:0000-0002-7383-4212), Gremese, Elisa (ORCID:0000-0002-2248-1058), and Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428)

Abstract

Low T-cell receptor (TCR)/CD28 signaling lymphocytes are expanded in arthritis. We asked whether the down-expression of TCR-related molecules correlates with specific arthritis characteristics and if it has clinical implications. TCR-ZETA, ZAP-70 and CD28 expression was measured by flow cytometry in synovial fluid (SF) and peripheral blood (PB)-derived T cells. In PB, ZETA-downregulation in CD4+ CD28+ and consequent CD4+ CD28lowZETAlow cell expansion correlate with CRP elevation, leukocyte recruitment into SF and, primarily, disease activity (DAS). In some patients, ZETA-downregulation extends to CD8+ CD28null and/or CD8+ CD28+ cells, and this correlates with enhanced leukocyte recruitment, multiple joint involvement, and disability index (HAQ). ZETA-downregulation in CD4+ CD28+ may also lead to CD4+ CD28+ ZETAnull cell expansion, which strongly correlates with HAQ. In SF, ZETA-downregulation in CD8+ CD28null and consequent CD8+ CD28nullZETAlow/null cell expansion correlate with CRP elevation and neutrophilic influx into SF, whereas ZAP-downregulation in CD8+ CD28+ and consequent CD8+ CD28lowZAPlow cell expansion strongly correlate with HAQ and DAS. ZETA-downregulation is preponderant in SF of seronegative arthritides, with seronegative rheumatoid arthritis showing significant down-regulation in CD8+ CD28null, and non-rheumatoid arthritides showing significant down-regulation in CD4+ CD28+ . Altogether, we identified new molecular and cellular biomarkers of arthritis-related T-cell inflammation, useful for assessing arthritis activity, predicting polyarticular progression and functional impairment, characterizing seronegative arthritides, and possibly tailoring immunotherapies.

Published
2019

17. Cardiac troponin T and NT-proBNP as diagnostic and prognostic biomarkers of primary cardiac involvement and disease severity in systemic sclerosis: A prospective study

Author

Bosello, Silvia Laura, De Luca, Giacomo, Berardi, Giorgia, Canestrari, Giovanni, De Waure, Chiara, Gabrielli, Francesca Augusta, Di Mario, Clara, Forni, Franca, Gremese, Elisa, Ferraccioli, Gianfranco, Bosello, Silvia (ORCID:0000-0002-4837-447X), de Waure, Chiara (ORCID:0000-0002-4346-1494), Forni, Franca (ORCID:0000-0002-7208-2047), Gremese, Elisa (ORCID:0000-0002-2248-1058), Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), Bosello, Silvia Laura, De Luca, Giacomo, Berardi, Giorgia, Canestrari, Giovanni, De Waure, Chiara, Gabrielli, Francesca Augusta, Di Mario, Clara, Forni, Franca, Gremese, Elisa, Ferraccioli, Gianfranco, Bosello, Silvia (ORCID:0000-0002-4837-447X), de Waure, Chiara (ORCID:0000-0002-4346-1494), Forni, Franca (ORCID:0000-0002-7208-2047), Gremese, Elisa (ORCID:0000-0002-2248-1058), and Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428)

Abstract

Objectives: The aim of our study was to define the role of high-sensitive cardiac troponin T (hs-cTnT) and NT-proBNP in identifying Systemic Sclerosis (SSc) patients with cardiac involvement and at higher risk of cardiac death. Methods: Plasma hs-cTnT and NT-proBNP concentrations were measured in 245 SSc-patients. Results: hs-cTnT and NT-proBNP levels were higher in SSc-patients than in healthy controls. Hs-cTnT levels were higher than 0.014 ng/ml in 32.3% SSc-patients, while NT-proBNP was above 125 pg/ml in 31.8% of them, irrespective of classical cardiovascular risk factor and of pulmonary arterial hypertension. Elevated hs-cTnT and NT-proBNP were associated with diffuse skin involvement and directly correlated with the skin score. Patients with increased cardiac markers presented a lower left-ventricular ejection fraction (LVEF) and a higher rate of right bundle branch block (RBBB) on electrocardiogram (ECG) compared to patients with normal cardiac enzymes. During the follow-up, 12 SSc-patients experience a disease-related death; 9 of these were directly related to cardiac involvement (sudden cardiac death or heart failure) and the majority of them occurred among patients with increase of at least one cardiac biomarker. Long-term survival was worse in patients with increase of both cardiac biomarkers. Conclusions: Evaluation of hs-cTnT and NT-proBNP levels may provide a tool to screen non-invasively SSc-patients for heart involvement. A higher incidence of impaired systolic function, ECG abnormalities and a poor outcome in SSc-patients with elevated cardiac enzymes suggests that they may be valuable screening biomarkers to detect a cardiac damage at early stages and to improve risk stratification.

Published
2019

18. Violence, Selection and Infant Mortality in Congo

Author

Dagnelie, Olivier, De Luca, Giacomo, Maystadt, Jean-François, Dagnelie, Olivier, De Luca, Giacomo, and Maystadt, Jean-François

Abstract

This paper documents the effects of the recent civil war in the Democratic Republic of Congo on mortality both in utero and during the first year of life. It instruments for conflict intensity using a mineral price index, which exploits the exogenous variation in the potential value of mineral resources generated by changes in world mineral prices to predict the geographic distribution of the conflict. Using estimates of civil war exposure on mortality across male and female newborn to assess their relative health, it provides evidence of culling effect (in utero selection) as a consequence of in utero shocks.

Published
2018

19. Violence, Selection and Infant Mortality in Congo

Author

Dagnelie, Olivier, De Luca, Giacomo, Maystadt, Jean-François, Dagnelie, Olivier, De Luca, Giacomo, and Maystadt, Jean-François

Abstract

This paper documents the effects of the recent civil war in the Democratic Republic of Congo on mortality both in utero and during the first year of life. It instruments for conflict intensity using a mineral price index, which exploits the exogenous variation in the potential value of mineral resources generated by changes in world mineral prices to predict the geographic distribution of the conflict. Using estimates of civil war exposure on mortality across male and female newborn to assess their relative health, it provides evidence of culling effect (in utero selection) as a consequence of in utero shocks.

Published
2018

20. QTc interval prolongation in Systemic Sclerosis: Correlations with clinical variables and arrhythmic risk

Author

De Luca, Giacomo, Bosello, Silvia Laura, Canestrari, Giovanni, Cavalli, Giulio, Dagna, Lorenzo, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), De Luca, Giacomo, Bosello, Silvia Laura, Canestrari, Giovanni, Cavalli, Giulio, Dagna, Lorenzo, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), and Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428)

Abstract

N/A

Published
2017

21. Life-threatening arrhythmias in a scleroderma patient: the role of myocardial inflammation in arrhythmic outburst

Author

De Luca, Giacomo, Bosello, Silvia Laura, Leone, A. M, Gabrielli, Francesca Augusta, Pelargonio, Gemma, Inzani, Frediano, Crea, Filippo, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), Crea, Filippo (ORCID:0000-0001-9404-8846), Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), De Luca, Giacomo, Bosello, Silvia Laura, Leone, A. M, Gabrielli, Francesca Augusta, Pelargonio, Gemma, Inzani, Frediano, Crea, Filippo, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), Crea, Filippo (ORCID:0000-0001-9404-8846), and Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428)

Abstract

not applicable

Published
2016

22. Prognostic Role of Ventricular Ectopic Beats in Systemic Sclerosis: A Prospective Cohort Study Shows ECG Indexes Predicting the Worse Outcome

Author

De Luca, Giacomo, Bosello, Silvia Laura, Gabrielli, Francesca Augusta, Berardi, Giorgia, Parisi, Federico, Rucco, Manuela, Canestrari, Giovanni Battista, Loperfido, Francesco, Galiuto, Leonarda, Crea, Filippo, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), Galiuto, Leonarda (ORCID:0000-0002-6831-479X), Crea, Filippo (ORCID:0000-0001-9404-8846), Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), De Luca, Giacomo, Bosello, Silvia Laura, Gabrielli, Francesca Augusta, Berardi, Giorgia, Parisi, Federico, Rucco, Manuela, Canestrari, Giovanni Battista, Loperfido, Francesco, Galiuto, Leonarda, Crea, Filippo, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), Galiuto, Leonarda (ORCID:0000-0002-6831-479X), Crea, Filippo (ORCID:0000-0001-9404-8846), and Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428)

Abstract

not available

Published
2016

23. Prognostic Role of Ventricular Ectopic Beats in Systemic Sclerosis: A Prospective Cohort Study Shows ECG Indexes Predicting the Worse Outcome

Author

De Luca, Giacomo, Bosello, Silvia Laura, Gabrielli, Francesca Augusta, Berardi, Giorgia, Parisi, Federico, Rucco, Manuela, Canestrari, Giovanni Battista, Loperfido, Francesco, Galiuto, Leonarda, Crea, Filippo, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), Galiuto, Leonarda (ORCID:0000-0002-6831-479X), Crea, Filippo (ORCID:0000-0001-9404-8846), Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), De Luca, Giacomo, Bosello, Silvia Laura, Gabrielli, Francesca Augusta, Berardi, Giorgia, Parisi, Federico, Rucco, Manuela, Canestrari, Giovanni Battista, Loperfido, Francesco, Galiuto, Leonarda, Crea, Filippo, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), Galiuto, Leonarda (ORCID:0000-0002-6831-479X), Crea, Filippo (ORCID:0000-0001-9404-8846), and Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428)

Abstract

Arrhythmias are frequent in Systemic Sclerosis (SSc) and portend a bad prognosis, accounting alone for 6% of total deaths. Many of these patients die suddenly, thus prevention and intensified risk-stratification represent unmet medical needs. The major goal of this study was the definition of ECG indexes of poor prognosis. METHODS: We performed a prospective cohort study to define the role of 24h-ECG-Holter as an additional risk-stratification technique in the identification of SSc-patients at high risk of life-threatening arrhythmias and sudden cardiac death (SCD). One-hundred SSc-patients with symptoms and/or signs suggestive of cardiac involvement underwent 24h-ECG-Holter. The primary end-point was a composite of SCD or need for implantable cardioverter defibrillator (ICD). RESULTS: Fifty-six patients (56%) had 24h-ECG-Holter abnormalities and 24(24%) presented frequent ventricular ectopic beats (VEBs). The number of VEBs correlated with high-sensitive cardiac troponin T (hs-cTnT) levels and inversely correlated with left-ventricular ejection fraction (LV-EF) on echocardiography. During a mean follow-up of 23.1±16.0 months, 5 patients died suddenly and two required ICD-implantation. The 7 patients who met the composite end-point had a higher number of VEBs, higher levels of hs-cTnT and NT-proBNP and lower LV-EF (p = 0.001 for all correlations). All these 7 patients had frequent VEBs, while LV-EF was not reduced in all and its range was wide. At ROC curve, VEBs>1190/24h showed 100% of sensitivity and 83% of specificity to predict the primary end-point (AUROC = 0.92,p<0.0001). Patients with VEBS>1190/24h had lower LV-EF and higher hs-cTnT levels and, at multivariate analysis, the presence of increased hs-cTnT and of right bundle branch block on ECG emerged as independent predictors of VEBs>1190/24h. None of demographic or disease-related characteristics emerged as predictors of poor outcome. CONCLUSIONS: VEBS>1190/24h identify patients at high risk of life-threaten

Published
2016

24. Tumour-associated antigens in systemic sclerosis patients with interstitial lung disease: association with lung involvement and cancer risk

Author

De Luca, Giacomo, Bosello, Silvia Laura, Berardi, Giorgia, Rucco, Manuela, Canestrari, Giovanni Battista, Correra, Miriam, Mirone, Luisa, Forni, Franca, Di Mario, Clara, Danza, Francesco, Pirronti, Tommaso, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), Mirone, Luisa (ORCID:0000-0001-5820-5533), Forni, Franca (ORCID:0000-0002-7208-2047), Danza, Francesco (ORCID:0000-0001-8569-415X), Pirronti, Tommaso (ORCID:0000-0003-3138-4097), Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), De Luca, Giacomo, Bosello, Silvia Laura, Berardi, Giorgia, Rucco, Manuela, Canestrari, Giovanni Battista, Correra, Miriam, Mirone, Luisa, Forni, Franca, Di Mario, Clara, Danza, Francesco, Pirronti, Tommaso, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), Mirone, Luisa (ORCID:0000-0001-5820-5533), Forni, Franca (ORCID:0000-0002-7208-2047), Danza, Francesco (ORCID:0000-0001-8569-415X), Pirronti, Tommaso (ORCID:0000-0003-3138-4097), and Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428)

Abstract

To evaluate the serum levels of tumour-associated antigens (TAAs) in patients with SSc and interstitial lung disease (ILD) and to define whether their levels mirror the severity and the progression of lung damage.

Published
2015

25. Troponin in Stable Ischemic Heart Disease and Diabetes

Author

Bosello, Silvia Laura, De Luca, Giacomo, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), Bosello, Silvia Laura, De Luca, Giacomo, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), and Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428)

Abstract

Troponin in Stable Ischemic Heart Disease and Diabetes

Published
2015

26. Tumour-associated antigens in systemic sclerosis patients with interstitial lung disease: association with lung involvement and cancer risk

Author

De Luca, Giacomo, Bosello, Silvia Laura, Berardi, Giorgia, Rucco, Manuela, Canestrari, Giovanni Battista, Correra, Miriam, Mirone, Luisa, Forni, Franca, Di Mario, Clara, Danza, Francesco M, Pirronti, Tommaso, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), Mirone, Luisa (ORCID:0000-0001-5820-5533), Forni, Franca (ORCID:0000-0002-7208-2047), Pirronti, Tommaso (ORCID:0000-0003-3138-4097), Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), De Luca, Giacomo, Bosello, Silvia Laura, Berardi, Giorgia, Rucco, Manuela, Canestrari, Giovanni Battista, Correra, Miriam, Mirone, Luisa, Forni, Franca, Di Mario, Clara, Danza, Francesco M, Pirronti, Tommaso, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), Mirone, Luisa (ORCID:0000-0001-5820-5533), Forni, Franca (ORCID:0000-0002-7208-2047), Pirronti, Tommaso (ORCID:0000-0003-3138-4097), and Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428)

Abstract

not applicable

Published
2015

27. Troponin in Stable Ischemic Heart Disease and Diabetes

Author

Bosello, Silvia Laura, De Luca, Giacomo, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), Bosello, Silvia Laura, De Luca, Giacomo, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), and Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428)

Abstract

not applicable

Published
2015

28. Mineral resources and conflicts in DRC: a case of ecological fallacy?

Author

UCL - SSH/LIDAM/IRES - Institut de recherches économiques et sociales, Maystadt, Jean-François, De Luca, Giacomo, Sekeris, Petros G., Ulimwengu, John, UCL - SSH/LIDAM/IRES - Institut de recherches économiques et sociales, Maystadt, Jean-François, De Luca, Giacomo, Sekeris, Petros G., and Ulimwengu, John

Abstract

We estimate the impact of geo-located mining concessions on the number of conflict events recorded in the Democratic Republic of the Congo between 1997 and 2007. Instrumenting the variable of interest with historical concessions interacted with changes in international prices of minerals, we unveil an ecological fallacy: whereas concessions have no effect on the number of conflicts at the territory level (lowest administrative unit), they do foster violence at the district level (higher administrative unit). We develop and validate empirically a theoretical model where the incentives of armed groups to exploit and protect mineral resources explain our empirical findings.

Published
2014

29. Mineral resources and conflicts in DRC:a case of ecological fallacy?

Author

Maystadt, Jean-Francois, De Luca, Giacomo, Sekeris, Petros, Ulimwengu, John, Maystadt, Jean-Francois, De Luca, Giacomo, Sekeris, Petros, and Ulimwengu, John

Abstract

We estimate the impact of geo-located mining concessions on the number of conflict events recorded in the Democratic Republic of the Congo between 1997 and 2007. Instrumenting the variable of interest with historical concessions interacted with changes in international prices of minerals, we unveil an ecological fallacy: Whereas concessions have no effect on the number of conflicts at the territory level (lowest administrative unit), they do foster violence at the district level (higher administrative unit). We develop and validate empirically a theoretical model where the incentives of armed groups to exploit and protect mineral resources explain our empirical findings.

Published
2014

30. Do girls pay the price of civil war?:violence and infant mortality in Congo

Author

Dagnelie, Olivier, De Luca, Giacomo, Maystadt, Jean-Francois, Dagnelie, Olivier, De Luca, Giacomo, and Maystadt, Jean-Francois

Abstract

This paper documents the impact of civil wars in the Democratic Republic of Congo on infant mortality between 1997 and 2004. It adopts an instrumental variable approach to correct for the non-random timing and location of conflict. Strong and robust evidence, including mother fixed effects regressions, shows that conflict significantly increases girl mortality. It also examines the mechanisms explaining this phenomenon, with a focus on disentangling the behavioral from the biological factors. The analysis suggests that gender imbalances in infant mortality are driven by the selection induced by a higher vulnerability of boys in utero rather than by gender discrimination.

Published
2014

31. Do girls pay the price of civil war? : violence and infant mortality in Congo

Author

Dagnelie, Olivier, De Luca, Giacomo, Maystadt, Jean-Francois, Dagnelie, Olivier, De Luca, Giacomo, and Maystadt, Jean-Francois

Abstract

This paper documents the impact of civil wars in the Democratic Republic of Congo on infant mortality between 1997 and 2004. It adopts an instrumental variable approach to correct for the non-random timing and location of conflict. Strong and robust evidence, including mother fixed effects regressions, shows that conflict significantly increases girl mortality. It also examines the mechanisms explaining this phenomenon, with a focus on disentangling the behavioral from the biological factors. The analysis suggests that gender imbalances in infant mortality are driven by the selection induced by a higher vulnerability of boys in utero rather than by gender discrimination.

Published
2014

32. Long-term efficacy of B cell depletion therapy on lung and skin involvement in diffuse systemic sclerosis

Author

Bosello, Silvia Laura, De Luca, Giacomo, Rucco, Manuela, Berardi, Giorgia, Falcione, Matteo, Danza, Fm, Pirronti, Tommaso, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), Pirronti, Tommaso (ORCID:0000-0003-3138-4097), Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), Bosello, Silvia Laura, De Luca, Giacomo, Rucco, Manuela, Berardi, Giorgia, Falcione, Matteo, Danza, Fm, Pirronti, Tommaso, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), Pirronti, Tommaso (ORCID:0000-0003-3138-4097), and Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428)

Abstract

To assess the long-term efficacy and safety of single and multiple courses of rituximab therapy in systemic sclerosis (SSc) patients with and without lung disease.

Published
2014

33. Mineral resources and conflicts in DRC

Author

Maystadt, Jean-François; De Luca, Giacomo; Sekeris, Petros G.; Ulimwengu, John M., http://orcid.org/0000-0002-8905-0201 Ulimwengu, John, Maystadt, Jean-François; De Luca, Giacomo; Sekeris, Petros G.; Ulimwengu, John M., and http://orcid.org/0000-0002-8905-0201 Ulimwengu, John

Abstract

PR, IFPRI3; ISI; F Strengthening institutions and governance, DSGD; WCAO

Published
2014

34. Do girls pay the price of civil war? Violence and infant mortality in Congo

Author

Dagnelie, Olivier; De Luca, Giacomo; Maystadt, Jean-François and Dagnelie, Olivier; De Luca, Giacomo; Maystadt, Jean-François

Abstract

Non-PR, IFPRI1; GRP32; D.1 Agriculture's role in national development strategy, DSGD, This paper documents the impact of the violent civil war affecting the Democratic Republic of Congo in the period 1997–2004 on infant mortality. It adopts an instrumental variable approach to correct for the nonrandom timing and location of conflict events using mineral price index variations by district, taking account of the mineral locations and prices, as instrument. Strong and robust evidence, including mother fixed effects regressions comparing siblings, shows that conflict significantly increases girl mortality. The paper also examines the mechanisms explaining this phenomenon, with a focus on disentangling the behavioral from the biological factors. The analysis suggests that gender imbalances in infant mortality are driven by the selection induced by a higher vulnerability of boys in utero rather than by gender discrimination.

Published
2014

35. Recognizing and treating myocarditis in recent-onset systemic sclerosis heart disease: Potential utility of immunosuppressive therapy in cardiac damage progression

Author

Pieroni, Maurizio, De Santis, Maria, Zizzo, Gaetano, Bosello, Silvia Laura, Smaldone, Costantino, Campioni, Mara, De Luca, Giacomo, Laria, Antonella, Meduri, Agostino, Bellocci, Fulvio, Bonomo, Lorenzo, Crea, Filippo, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), Meduri, Agostino (ORCID:0000-0003-2332-6428), Bonomo, Lorenzo (ORCID:0000-0001-5101-9367), Crea, Filippo (ORCID:0000-0001-9404-8846), Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428), Pieroni, Maurizio, De Santis, Maria, Zizzo, Gaetano, Bosello, Silvia Laura, Smaldone, Costantino, Campioni, Mara, De Luca, Giacomo, Laria, Antonella, Meduri, Agostino, Bellocci, Fulvio, Bonomo, Lorenzo, Crea, Filippo, Ferraccioli, Gianfranco, Bosello, Silvia Laura (ORCID:0000-0002-4837-447X), Meduri, Agostino (ORCID:0000-0003-2332-6428), Bonomo, Lorenzo (ORCID:0000-0001-5101-9367), Crea, Filippo (ORCID:0000-0001-9404-8846), and Ferraccioli, Gianfranco (ORCID:0000-0001-6246-2428)

Abstract

Scleroderma heart disease is a major risk of death in systemic sclerosis (SSc). Mechanisms underlying myocardial damage are still unclear. We performed an extensive study of SSc patients with recent-onset symptoms for heart disease and examined the efficacy of immunosuppressive therapy.

Published
2013

36. Mineral Resources and Conflicts in the Democratic Republic of the Congo: A Case of Ecological Fallacy

Author

De Luca, Giacomo; Maystadt, Jean-François; Sekeris, Petros G.; Ulimwengu, John M.; Folledo, Renato, http://orcid.org/0000-0002-8905-0201 Ulimwengu, John, De Luca, Giacomo; Maystadt, Jean-François; Sekeris, Petros G.; Ulimwengu, John M.; Folledo, Renato, and http://orcid.org/0000-0002-8905-0201 Ulimwengu, John

Subjects
Q34 Natural Resources and Domestic and International Conflicts; O13 Economic Development: Agriculture, Natural Resources, Energy, Environment, Other Primary Product; Q32 Exhaustible Resources and Economic Development; N57 Agriculture, Natural Resources, Environment, and Extractive Industries: Africa, Oceania
Abstract

Non-PR, IFPRI1; Subtheme 6.2, WCAO; DSGD, Civil wars inflict considerable costs on countries which may be trapped in vicious cycles of violence. To avoid these adverse events, scholars have attempted to identify the roots of civil wars. Valuable minerals have been listed among the main drivers of civil conflicts. Yet, despite the large body of literature, the evidence remains mixed. This paper provides a spatially nuanced view of the role of mineral resources in civil wars in the particular case of the Democratic Republic of the Congo. We estimate the impact of geolocated new mining concessions on the number of conflict events between January 1997 and December 2007. Instrumenting the variable of interest with historical concessions interacted with changes in mineral international prices, we unveil an ecological fallacy: Whereas concessions have no effect on the number of conflicts at the territory level (lowest administrative unit), they do foster violence at the district level (higher administrative unit). We develop a theoretical model wherein the incentives of armed groups to exploit and protect mineral resources explain our empirical findings. A spatial analysis of the effect of mining concessions on conflict backs our proposed theoretical explanation.

Published
2012

37. Mineral resources and conflicts in DRC

Author

De Luca Giacomo; Maystadt, Jean-François; Sekeris, Petros G.; Ulimwengu, John M., http://orcid.org/0000-0002-8905-0201 Ulimwengu, John, De Luca Giacomo; Maystadt, Jean-François; Sekeris, Petros G.; Ulimwengu, John M., and http://orcid.org/0000-0002-8905-0201 Ulimwengu, John

Abstract

Non-PR, IFPRI5; F Strengthening institutions and governance, DSGD

Published
2012

38. Essays on Political Economy and Conflict Theory

Author

FUNDP - ECO_CRED (Centre de recherche en économie du développement), FUNDP - Ecole doctorale en sciences économiques et de gestion, Baland, Jean-Marie, Toulemonde, Eric, Platteau, Jean-Philippe, Torvik, Ragnar, Esteban, Joan Maria, De Luca, Giacomo, FUNDP - ECO_CRED (Centre de recherche en économie du développement), FUNDP - Ecole doctorale en sciences économiques et de gestion, Baland, Jean-Marie, Toulemonde, Eric, Platteau, Jean-Philippe, Torvik, Ragnar, Esteban, Joan Maria, and De Luca, Giacomo

Abstract

In the first paper I study the incentives for electoral registration in a system in which registration is costly. I argue that, if some more powerful agents in the society can condition the voting behavior of part of the electorate, then more easily controlled voters are also more likely to be registered. This allows the powerful agents to have a large impact on election results, as the share of actual votes controlled is increased through strategic registration. I show with the help of a theoretical model that reducing the control on votes (for instance with the adoption of a secret ballot) only partially reduces the bias in registration, as scarcely motivated voters will be always easy to control. I test the predictions of the model by examining in detail the effects of the introduction of the secret ballot in Chile in 1958. The second paper, coauthored with my colleague Petros Sekeris, investi- gates the impact of land inequality on conflict intensity. We analyze how land inequality across landlords influences the intensity of the fight against a rebel group constituted by landless individuals. We show that conflict intensity is non-monotonic in land inequality. In particular, the most severe conflicts occur for intermediate land inequality levels. Moreover, under certain condition we show that a Pareto improving transfer of land from the smaller to the larger landlord exists. Finally, the third paper also coauthored with Petros Sekeris, explores the existence of deterrence equilibria in a general equilibrium model of “guns and butter” production. In this class of model two agents choose how to allocate their initial endowment between the production of consumables and weapons in the first period of the game and whether to engage in war in the second period. The standard result in the literature is that war is the only equilibrium of the game. We show that, if fighting entails sufficiently low destruction, indeed war is the unique equilibrium of the game. If, howe, (DOCSESG00 ) -- FUNDP, 2009

Published
2009

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