المؤلفون: Sobanski, Vincent, Giovannelli, Jonathan, Allanore, Yannick, Riemekasten, Gabriela, Airo, Paolo, Vettori, Serena, Cozzi, Franco, Distler, Oliver, Matucci-Cerinic, Marco, Denton, Christopher P., Launay, David, Hachulla, Eric

المساهمون: CHU Lille, Inserm, Université de Lille, Lille Inflammation Research International Center (LIRIC) - U995, Lille Inflammation Research International Center - U 995 LIRIC, Hôpital Cochin AP-HP, Universität zu Lübeck = University of Lübeck Lübeck, Civic Hospital of Brescia, Second University of Naples-Caserta, Università degli Studi di Padova = University of Padua Unipd, University hospital of Zurich Zurich, Università degli Studi di Firenze = University of Florence UniFI, University College of London London UCL

الوصف: OBJECTIVE: Systemic sclerosis (SSc) is a heterogeneous connective tissue disease that is typically subdivided into limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) depending on the extent of skin involvement. This subclassification may not capture the entire variability of clinical phenotypes. The European Scleroderma Trials and Research (EUSTAR) database includes data on a prospective cohort of SSc patients from 122 European referral centers. This study was undertaken to perform a cluster analysis of EUSTAR data to distinguish and characterize homogeneous phenotypes without any a priori assumptions, and to examine survival among the clusters obtained. METHODS: A total of 11,318 patients were registered in the EUSTAR database, and 6,927 were included in the study. Twenty-four clinical and serologic variables were used for clustering. RESULTS: Clustering analyses provided a first delineation of 2 clusters showing moderate stability. In an exploratory attempt, we further characterized 6 homogeneous groups that differed with regard to their clinical features, autoantibody profile, and mortality. Some groups resembled usual dcSSc or lcSSc prototypes, but others exhibited unique features, such as a majority of lcSSc patients with a high rate of visceral damage and antitopoisomerase antibodies. Prognosis varied among groups and the presence of organ damage markedly impacted survival regardless of cutaneous involvement. CONCLUSIONS: Our findings suggest that restricting subsets of SSc patients to only those based on cutaneous involvement may not capture the complete heterogeneity of the disease. Organ damage and antibody profile should be taken into consideration when individuating homogeneous groups of patients with a distinct prognosis. ; 71;9

وصف الملف: application/octet-stream; application/rdf+xml; charset=utf-8; application/pdf

العلاقة: Arthritis & rheumatology; "null"; http://hdl.handle.net/20.500.12210/13737Test

الإتاحة: https://doi.org/20.500.12210/13737Test
https://hdl.handle.net/20.500.12210/13737Test

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

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

مصطلحات موضوعية: COVID-19, COVID-19 vaccine, Interstitial lung disease, SARS-CoV-2, Scleroderma, Systemic sclerosis

الوصف: Introduction: The impact of COVID-19 pandemic represents a serious challenge for 'frail' patients' populations with inflammatory autoimmune systemic diseases such as systemic sclerosis (SSc). We investigated the prevalence and severity of COVID-19, as well the effects of COVID-19 vaccination campaign in a large series of SSc patients followed for the entire period (first 38 months) of pandemic. Patients and method: This prospective survey study included 1755 unselected SSc patients (186M, 1,569F; mean age 58.7±13.4SD years, mean disease duration 8.8±7.3SD years) recruited in part by telephone survey at 37 referral centers from February 2020 to April 2023. The following parameters were carefully evaluated: i. demographic, clinical, serological, and therapeutical features; ii. prevalence and severity of COVID-19; and iii. safety, immunogenicity, and efficacy of COVID-19 vaccines. Results: The prevalence of COVID-19 recorded during the whole pandemic was significantly higher compared to Italian general population (47.3% vs 43.3%, p<0.000), as well the COVID-19-related mortality (1.91% vs 0.72 %, p<0.001). As regards the putative prognostic factors of worse outcome, COVID-19 positive patients with SSc-related interstitial lung involvement showed significantly higher percentage of COVID-19-related hospitalization compared to those without (5.85% vs 1.73%; p<0.0001), as well as of mortality rate (2.01% vs 0.4%; p=0.002). Over half of patients (56.3%) received the first two plus one booster dose of vaccine; while a fourth dose was administered to 35.6%, and only few of them (1.99%) had five or more doses of vaccine. Of note, an impaired seroconversion was recorded in 25.6% of individuals after the first 2 doses of vaccine, and in 8.4% of patients also after the booster dose. Furthermore, the absence of T-cell immunoreactivity was observed in 3/7 patients tested by QuantiFERON® SARSCoV-2 Starter Set (Qiagen). The efficacy of vaccines, evaluated by comparing the COVID-19-related death rate recorded during pre- ...

العلاقة: info:eu-repo/semantics/altIdentifier/pmid/37854035; info:eu-repo/semantics/altIdentifier/wos/WOS:001111116000001; volume:7; numberofpages:9; journal:JOURNAL OF TRANSLATIONAL AUTOIMMUNITY; https://hdl.handle.net/20.500.11768/152999Test; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85173744644; https://www.sciencedirect.com/science/article/pii/S2589909023000254?via=ihubTest

الإتاحة: https://doi.org/20.500.11768/152999Test
https://doi.org/10.1016/j.jtauto.2023.100212Test
https://hdl.handle.net/20.500.11768/152999Test
https://www.sciencedirect.com/science/article/pii/S2589909023000254?via=ihubTest

المؤلفون: De Angelis, Rossella, Ferri, Clodoveo, Giuggioli, Dilia, Bajocchi, Gianluigi, Dagna, Lorenzo, Bellando-Randone, Silvia, Zanframundo, Giovanni, Foti, Rosario, Cacciapaglia, Fabio, Cuomo, Giovanna, Ariani, Alarico, Rosato, Edoardo, Lepri, Gemma, 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, Anna Maria, Ciano, Giovanni, Beretta, Lorenzo, Bagnato, Gianluca, Lubrano, Ennio, De Andres, Ilenia, Giollo, Alessandro, Saracco, Marta, Agnes, Cecilia, Cipolletta, Edoardo, Lumetti, Federica, Spinella, Amelia, Magnani, Luca, Campochiaro, Corrado, De Luca, Giacomo, Codullo, Veronica, Visalli, Elisa, Di Vico, Claudio, Gigante, Antonietta, Pellagrino, Greta, Pigatto, Erika, Lazzaroni, Maria-Grazia, Franceschini, Franco, Generali, Elena, Mennillo, Gianna, Barsotti, Simone, Mariano, Giuseppa Pagano, Furini, Federica, Vultaggio, Licia, Parisi, Simone, Peroni, Clara Lisa, Rozza, Davide, Zanetti, Anna, Carrara, Greta, Landolfi, Gianpiero, Scirè, Carlo Alberto, Bianchi, Gerolamo, Fusaro, Enrico, Sebastiani, Gian Domenico, Govoni, Marcello, D'Angelo, Salvatore, Cozzi, Franco, Guiducci, Serena, Doria, Andrea, Salvarani, Carlo, Iannone, Florenzo, Matucci-Cerinic, Marco, MATUCCI CERINIC, MARCO

المساهمون: De Angelis, Rossella, Ferri, Clodoveo, Giuggioli, Dilia, Bajocchi, Gianluigi, Dagna, Lorenzo, Bellando-Randone, Silvia, Zanframundo, Giovanni, Foti, Rosario, Cacciapaglia, Fabio, Cuomo, Giovanna, Ariani, Alarico, Rosato, Edoardo, Lepri, Gemma, 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, Anna Maria, Ciano, Giovanni, Beretta, Lorenzo, Bagnato, Gianluca, Lubrano, Ennio, De Andres, Ilenia, Giollo, Alessandro, Saracco, Marta, Agnes, Cecilia, Cipolletta, Edoardo, Lumetti, Federica, Spinella, Amelia, Magnani, Luca, Campochiaro, Corrado, De Luca, Giacomo, Codullo, Veronica, Visalli, Elisa, Di Vico, Claudio, Gigante, Antonietta, Pellagrino, Greta, Pigatto, Erika, Lazzaroni, Maria-Grazia, Franceschini, Franco, Generali, Elena, Mennillo, Gianna, Barsotti, Simone, Mariano, Giuseppa Pagano, Furini, Federica, Vultaggio, Licia, Parisi, Simone, Peroni, Clara Lisa, Rozza, Davide, Zanetti, Anna, Carrara, Greta, Landolfi, Gianpiero, Scirè, Carlo Alberto, Bianchi, Gerolamo, Fusaro, Enrico, Sebastiani, Gian Domenico, Govoni, Marcello, D'Angelo, Salvatore, Cozzi, Franco, Guiducci, Serena, Doria, Andrea, Salvarani, Carlo, Iannone, Florenzo, Matucci-Cerinic, Marco, MATUCCI CERINIC, Marco

مصطلحات موضوعية: Autoimmune Diseases, Autoimmunity, Epidemiology, Scleroderma, Systemic, systemic sclerosis

الوصف: ObjectiveTo describe demographic, clinical and laboratory features of systemic sclerosis sine scleroderma (ssSSc) in a large multicentre systemic sclerosis (SSc) cohort.MethodsData 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.ResultsAmong 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<0.001). Clinical ssSSc phenotype was comparable to lcSSc, except for digital pitting scars (DPS) (19.7% vs 42%, p=0.01), but significantly milder than dcSSc, particularly for digital ulcers (DU) (6.6% vs 35.7%, p<0.001), oesophagus (46.2% vs 63.5%, p=0.009), lung (mean diffusion capacity for carbon monoxide 72.2 +/- 19.6 vs 62.4 +/- 22.8, p=0.009; mean forced vital capacity 105.6 +/- 21.7 vs 89.2 +/- 20.9, p<0.001) and major videocapillaroscopic alterations (late pattern 8.6% vs 47.6%, p<0.001). Moreover, in ssSSc the percentages of anticentromere and antitopoisomerase were comparable to lcSSc (40% and 18.3% vs 36.7% and 26.6%), but divergent respect to dcSSc (8.6% and 67.4%, p<0.001).ConclusionThe ssSSc is a quite rare disease variant characterised by clinico-serological features comparable to lcSSc, but significantly different from dcSSc. Overall, longer RP duration, low percentages of DPS and peripheral microvascular abnormalities, and increased anti-centromere seropositivity distinguish ssSSc. Further investigations based on national registries might provide useful insights on the actual relevance of the ssSSc within the scleroderma spectrum.

العلاقة: info:eu-repo/semantics/altIdentifier/pmid/36868782; info:eu-repo/semantics/altIdentifier/wos/WOS:000950031300008; volume:9; issue:1; numberofpages:11; journal:RMD OPEN; https://hdl.handle.net/20.500.11768/153005Test; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85149568456; https://rmdopen.bmj.com/content/9/1/e002890Test

الإتاحة: https://doi.org/20.500.11768/153005Test
https://doi.org/10.1136/rmdopen-2022-002890Test
https://hdl.handle.net/20.500.11768/153005Test
https://rmdopen.bmj.com/content/9/1/e002890Test

المؤلفون: 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, Zanframundo Giovanni

المصدر: RMD Open, Vol 9, Iss 1 (2023)

مصطلحات موضوعية: Medicine

الوصف: 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

وصف الملف: electronic resource

العلاقة: https://rmdopen.bmj.com/content/9/1/e002890.fullTest; https://doaj.org/toc/2056-5933Test

الوصول الحر: https://doaj.org/article/a19416bfc3614b1385db8eb4929e233cTest

المؤلفون: 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

المساهمون: 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

مصطلحات موضوعية: Systemic sclerosis, intravenous iloprost, therapy

الوصف: 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 in the different centers suggests the need of a ...

وصف الملف: STAMPA

العلاقة: info:eu-repo/semantics/altIdentifier/pmid/38333531; info:eu-repo/semantics/altIdentifier/wos/WOS:001142463700001; volume:9; issue:1; firstpage:38; lastpage:49; journal:JOURNAL OF SCLERODERMA AND RELATED DISORDERS; https://hdl.handle.net/11392/2544871Test; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85184672931

الإتاحة: https://doi.org/10.1177/23971983231209809Test
https://hdl.handle.net/11392/2544871Test

المؤلفون: 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, Matucci-Cerinic, Marco

المساهمون: 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, Matucci-Cerinic, Marco

مصطلحات موضوعية: Environmental, Geographical area, Macro-area, Referral, Scleroderma, Systemic sclerosis

الوصف: 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.

وصف الملف: STAMPA

العلاقة: info:eu-repo/semantics/altIdentifier/pmid/35908642; info:eu-repo/semantics/altIdentifier/wos/WOS:000856598500002; volume:21; issue:10; firstpage:103159-1; lastpage:103159-9; numberofpages:9; journal:AUTOIMMUNITY REVIEWS; https://hdl.handle.net/11392/2547591Test; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85135806196

الإتاحة: https://doi.org/10.1016/j.autrev.2022.103159Test
https://hdl.handle.net/11392/2547591Test

المؤلفون: Jaeger, Veronika K., Tikly, Mohammed, Xu, Dong, Siegert, Elise, Hachulla, Eric, Airo, Paolo, Valentini, Gabriele, Matucci-Cerinic, Marco, Distler, Oliver, Cozzi, Franco, Carreira, Patricia E., Allanore, Yannick, Muller-Ladner, Ulf, Ananieva, Lidia P., Balbir-Gurman, Alexandra, Distler, Jorg H. W., Czirjak, Laszlo, Li, Mengtao, Henes, Jorg, Jimenez, Sergio A., Smith, Vanessa, Damjanov, Nemanja, Denton, Christopher P., Del Galdo, Francesco, Saketkoo, Lesley Ann, Walker, Ulrich A.

المساهمون: CHU Lille, Inserm, Université de Lille, Lille Inflammation Research International Center - U 995 LIRIC, Centre National de Référence des Maladies Auto-Immunes Systémiques Rares du Nord et Nord-Ouest de France CeRAINO, Université Paris Descartes - Paris 5 UPD5

مصطلحات موضوعية: races, organ manifestations, systemic sclerosis

الوصف: OBJECTIVE: Racial factors play a significant role in SSc. We evaluated differences in SSc presentations between white patients (WP), Asian patients (AP) and black patients (BP) and analysed the effects of geographical locations. METHODS: SSc characteristics of patients from the EUSTAR cohort were cross-sectionally compared across racial groups using survival and multiple logistic regression analyses. RESULTS: The study included 9162 WP, 341 AP and 181 BP. AP developed the first non-RP feature faster than WP but slower than BP. AP were less frequently anti-centromere (ACA; odds ratio (OR) = 0.4, P < 0.001) and more frequently anti-topoisomerase-I autoantibodies (ATA) positive (OR = 1.2, P = 0.068), while BP were less likely to be ACA and ATA positive than were WP [OR(ACA) = 0.3, P < 0.001; OR(ATA) = 0.5, P = 0.020]. AP had less often (OR = 0.7, P = 0.06) and BP more often (OR = 2.7, P < 0.001) diffuse skin involvement than had WP. AP and BP were more likely to have pulmonary hypertension [OR(AP) = 2.6, P < 0.001; OR(BP) = 2.7, P = 0.03 vs WP] and a reduced forced vital capacity [OR(AP) = 2.5, P < 0.001; OR(BP) = 2.4, P < 0.004] than were WP. AP more often had an impaired diffusing capacity of the lung than had BP and WP [OR(AP vs BP) = 1.9, P = 0.038; OR(AP vs WP) = 2.4, P < 0.001]. After RP onset, AP and BP had a higher hazard to die than had WP [hazard ratio (HR) (AP) = 1.6, P = 0.011; HR(BP) = 2.1, P < 0.001]. CONCLUSIONS: Compared with WP, and mostly independent of geographical location, AP have a faster and earlier disease onset with high prevalences of ATA, pulmonary hypertension and forced vital capacity impairment and higher mortality. BP had the fastest disease onset, a high prevalence of diffuse skin involvement and nominally the highest mortality. ; 59

وصف الملف: application/octet-stream

العلاقة: Rheumatology; Rheumatology (Oxford); http://hdl.handle.net/20.500.12210/105021Test

الإتاحة: https://doi.org/20.500.12210/105021Test
https://hdl.handle.net/20.500.12210/105021Test

المؤلفون: John, Gregor Raphaël, Zanatta, Elisabetta, Polito, Pamela, Piantoni, Silvia, Fredi, Micaela, Coattrenec, Yann, Guemara, Romain, Franceschini, Franco, Truchetet, Marie-Elise, Cozzi, Franco, Airò, Paolo, Chizzolini, Carlo

المصدر: ISSN: 0172-8172 ; Rheumatology international, vol. 42, no. 12 (2022) p. 2141-2150.

مصطلحات موضوعية: info:eu-repo/classification/ddc/616, info:eu-repo/classification/ddc/616.07, Death, Hospital admission, Natural history, Quality of life, Scleroderma, Urinary incontinence

الوصف: Investigate the natural history of urinary incontinence (UI) in systemic sclerosis (SSc) and assess its impact on quality of life (QoL). A longitudinal, international observational study followed 189 patients with SSc for a median duration of 5 years (IQR: 4.8–5.3). Presence, subtype and severity of UI, hospital admission and QoL were assessed using serial self-administered questionnaires. Mortality data came from national death registries. Multilevel mixed-effect logistic regressions explored factors associated with UI. Cox models adjusted the effects of UI on hospitalization and death for age, sex and subtype of SSc. Mean annual rates of new-onset UI and remission were 16.3% (95%CI 8.3%–24.2%) and 20.8% (95%CI 12.6–29.1), respectively. Among UI patients, 57.9% (95%CI 51.8–64.0) changed from one UI subtype to another. Between annual questionnaires, the severity of UI was the same in 51.1% (95%CI 40.8–61.4), milder or resolved in 35.2% (95%CI 25.3–44.9), and worse in 13.8% (95%CI 6.7–20.9). Anti-centromere antibodies, digestive symptoms, sex, age, neurological or urological comorbidities, diuretics and puffy fingers were all associated with UI. The two strongest predictors of UI and UI subtypes were a recent UI episode and the subtype of previous leakage episodes. UI at inclusion was not associated with hospital admission (adjusted HR: 1.86; 95%CI 0.88–3.93), time to death (aHR: 0.84; 95%CI 0.41–1.73) or change in QoL over time. Self-reported UI among SSc patients is highly dynamic: it waxes and wanes, changing from one subtype to another over time.

العلاقة: info:eu-repo/semantics/altIdentifier/pmid/35945297; https://archive-ouverte.unige.ch/unige:167104Test; unige:167104

الإتاحة: https://doi.org/10.1007/s00296-022-05178-1Test
https://archive-ouverte.unige.ch/unige:167104Test

المؤلفون: John, Gregor, Zanatta, Elisabetta, Polito, Pamela, Piantoni, Silvia, Fredi, Micaela, Coattrenec, Yann, Guemara, Romain, Franceschini, Franco, Truchetet, Marie-Elise, Cozzi, Franco, Airò, Paolo, Chizzolini, Carlo

المساهمون: University of Geneva

المصدر: Rheumatology International ; volume 42, issue 12, page 2141-2150 ; ISSN 1437-160X

مصطلحات موضوعية: Immunology, Immunology and Allergy, Rheumatology

الوصف: Investigate the natural history of urinary incontinence (UI) in systemic sclerosis (SSc) and assess its impact on quality of life (QoL). A longitudinal, international observational study followed 189 patients with SSc for a median duration of 5 years (IQR: 4.8–5.3). Presence, subtype and severity of UI, hospital admission and QoL were assessed using serial self-administered questionnaires. Mortality data came from national death registries. Multilevel mixed-effect logistic regressions explored factors associated with UI. Cox models adjusted the effects of UI on hospitalization and death for age, sex and subtype of SSc. Mean annual rates of new-onset UI and remission were 16.3% (95%CI 8.3%–24.2%) and 20.8% (95%CI 12.6–29.1), respectively. Among UI patients, 57.9% (95%CI 51.8–64.0) changed from one UI subtype to another. Between annual questionnaires, the severity of UI was the same in 51.1% (95%CI 40.8–61.4), milder or resolved in 35.2% (95%CI 25.3–44.9), and worse in 13.8% (95%CI 6.7–20.9). Anti-centromere antibodies, digestive symptoms, sex, age, neurological or urological comorbidities, diuretics and puffy fingers were all associated with UI. The two strongest predictors of UI and UI subtypes were a recent UI episode and the subtype of previous leakage episodes. UI at inclusion was not associated with hospital admission (adjusted HR: 1.86; 95%CI 0.88–3.93), time to death (aHR: 0.84; 95%CI 0.41–1.73) or change in QoL over time. Self-reported UI among SSc patients is highly dynamic: it waxes and wanes, changing from one subtype to another over time.

الإتاحة: https://doi.org/10.1007/s00296-022-05178-1Test

المؤلفون: Ferri, Clodoveo, Giuggioli, Dilia, Raimondo, Vincenzo, L'Andolina, Massimo, Dagna, Lorenzo, Tavoni, Antonio, Caso, Francesco, Ursini, Francesco, Piero, Ruscitti, 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, Matucci-Cerinic, Marco, D'Angelo, Salvatore, Doria, Andrea, Franceschini, Franco, Meliconi, Riccardo, Iannone, Florenzo, Giacomelli, Roberto, Zignego, Anna Linda, Varcasia, Poupak, Antonelli, Alessandro

المساهمون: Ferri, Clodoveo, Giuggioli, Dilia, Raimondo, Vincenzo, L'Andolina, Massimo, Dagna, Lorenzo, Tavoni, Antonio, Caso, Francesco, Ursini, Francesco, Piero, Ruscitti, 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, Matucci-Cerinic, Marco, D'Angelo, Salvatore, Doria, Andrea, Franceschini, Franco, Meliconi, Riccardo, Iannone, Florenzo, Giacomelli, Roberto, Zignego, Anna Linda, Varcasia, Poupak, Antonelli, Alessandro

مصطلحات موضوعية: COVID-19, SARS-CoV-2, arthriti, autoimmune systemic disease, connective tissue disease, interstitial lung disease, rheumatic disease, systemic sclerosis

الوصف: The Covid-19 pandemic may have a deleterious impact on patients with autoimmune systemic diseases (ASD) due to their deep immune-system alterations.

العلاقة: info:eu-repo/semantics/altIdentifier/pmid/34477509; info:eu-repo/semantics/altIdentifier/wos/WOS:000710468800005; volume:27; issue:41; firstpage:4245; lastpage:4252; numberofpages:8; journal:CURRENT PHARMACEUTICAL DESIGN; http://hdl.handle.net/11380/1253235Test; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85100215683

الإتاحة: https://doi.org/10.2174/1381612827666210903103935Test
http://hdl.handle.net/11380/1253235Test