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1. Decline in forced vital capacity in subjects with systemic sclerosis-associated interstitial lung disease in the SENSCIS trial compared with healthy reference subjects.

Author

Maher, Toby, Bourdin, Arnaud, Volkmann, Elizabeth, Vettori, Serena, Distler, Jörg, Alves, Margarida, Stock, Christian, and Distler, Oliver

Subjects
Connective tissue diseases, Pulmonary fibrosis, Scleroderma, systemic, Vital capacity, Humans, Lung, Lung Diseases, Interstitial, Scleroderma, Systemic, Vital Capacity
Abstract

BACKGROUND: The forced vital capacity (FVC) of healthy individuals depends on their age, sex, ethnicity and height. Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is characterised by loss of FVC. We compared FVC values in the subjects with SSc-ILD in the SENSCIS trial of nintedanib versus placebo with values from hypothetical matched healthy references. METHODS: The SENSCIS trial enrolled subjects with SSc with first non-Raynaud symptom in the prior ≤ 7 years, extent of fibrotic ILD on HRCT ≥ 10%, and FVC ≥ 40% predicted. FVC at baseline and decline in FVC over 52 weeks were compared with FVC values in hypothetical healthy reference subjects matched 1:1 to the subjects in the trial for age, sex, ethnicity and height, determined using equations published by the European Respiratory Society Global Lung Function Initiative. RESULTS: At baseline, mean (SD) FVC was 2460 (737) mL in the nintedanib group (n = 287) compared with 3403 (787) mL in the hypothetical matched healthy references. Mean (SD) FVC was 2544 (817) mL in the placebo group (n = 286) compared with 3516 (887) mL in the hypothetical matched healthy references. Mean (SE) changes in FVC at week 52, i.e., age-related loss of lung function, in the hypothetical healthy references matched to the nintedanib and placebo groups, respectively, were - 26.3 (0.5) mL and - 25.8 (0.5) mL. The difference in the change in FVC at week 52 between the nintedanib group and the hypothetical healthy references was 26.6 mL (95% CI: 1.2, 52.0; p = 0.04). The difference in the change in FVC at week 52 between the placebo group and the hypothetical healthy references was 77.5 mL (95% CI: 51.4, 103.7; p

Published
2022

3. A phase 2 randomized, double-blinded, placebo-controlled, multicenter trial evaluating the efficacy and safety of raloxifene for patients with mild to moderate COVID-19

Author

Nicastri, Emanuele, Marinangeli, Franco, Pivetta, Emanuele, Torri, Elena, Reggiani, Francesco, Fiorentino, Giuseppe, Scorzolini, Laura, Vettori, Serena, Marsiglia, Carolina, Gavioli, Elizabeth Marie, Beccari, Andrea R., Terpolilli, Giuseppe, De Pizzol, Maria, Goisis, Giovanni, Mantelli, Flavio, Vaia, Francesco, and Allegretti, Marcello

Published
2022
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10. Effect of Nintedanib on Progression of Systemic Sclerosis-Associated Interstitial Lung Disease Over 100 Weeks: Data From a Randomized Controlled Trial

Author

Assassi, Shervin, Distler, Oliver, Allanore, Yannick, Ogura, Takashi, Varga, John, Vettori, Serena, Crestani, Bruno, Voss, Florian, Alves, Margarida, Stowasser, Susanne, Maher, Toby M, SENSCIS trial investigators, University of Zurich, and Assassi, Shervin

Subjects
2745 Rheumatology, 10051 Rheumatology Clinic and Institute of Physical Medicine, 610 Medicine & health
Published
2022
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11. Effectiveness and safety of tocilizumab in patients with systemic sclerosis: a propensity score matched controlled observational study of the EUSTAR cohort

Author

Kuster, Simon, primary, Jordan, Suzana, additional, Elhai, Muriel, additional, Held, Ulrike, additional, Steigmiller, Klaus, additional, Bruni, Cosimo, additional, Cacciapaglia, Fabio, additional, Vettori, Serena, additional, Siegert, Elise, additional, Rednic, Simona, additional, Codullo, Veronica, additional, Airo, Paolo, additional, Braun-Moscovici, Yolanda, additional, Hunzelmann, Nicolas, additional, Joao Salvador, Maria, additional, Riccieri, Valeria, additional, Gheorghiu, Ana-Maria, additional, Alegre Sancho, Juan José, additional, Romanowska-Prochnicka, Katarzyna, additional, Castellví, Ivan, additional, Kötter, Ina, additional, Truchetet, Marie-Elise, additional, López-Longo, FJ, additional, Novikov, Pavel I, additional, Giollo, Alessandro, additional, Shirai, Yuichiro, additional, Belloli, Laura, additional, Zanatta, Elisabetta, additional, Hachulla, Eric, additional, Smith, Vanessa, additional, Denton, Chris, additional, Ionescu, Ruxandra M, additional, Schmeiser, Tim, additional, Distler, Joerg H W, additional, Gabrielli, Armando, additional, Hoffmann-Vold, Anna-Maria, additional, Kuwana, Masataka, additional, Allanore, Yannick, additional, and Distler, Oliver, additional

Published
2022
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12. Decline in forced vital capacity in subjects with systemic sclerosis-associated interstitial lung disease in the SENSCIS trial compared with healthy reference subjects

Author

Maher, Toby M, Bourdin, Arnaud, Volkmann, Elizabeth R, Vettori, Serena, Distler, Jörg H W, Alves, Margarida, Stock, Christian, Distler, Oliver, British Lung Foundation, University of Zurich, and Maher, Toby M

Subjects
Connective tissue diseases, Vital capacity, Scleroderma, Systemic, Respiratory System, 10051 Rheumatology Clinic and Institute of Physical Medicine, 610 Medicine & health, 1103 Clinical Sciences, Pulmonary fibrosis, 2740 Pulmonary and Respiratory Medicine, Humans, ddc:610, Lung Diseases, Interstitial, Lung, 1102 Cardiorespiratory Medicine and Haematology
Abstract

Background The forced vital capacity (FVC) of healthy individuals depends on their age, sex, ethnicity and height. Systemic sclerosis-associated interstitial lung disease (SSc-ILD) is characterised by loss of FVC. We compared FVC values in the subjects with SSc-ILD in the SENSCIS trial of nintedanib versus placebo with values from hypothetical matched healthy references. Methods The SENSCIS trial enrolled subjects with SSc with first non-Raynaud symptom in the prior ≤ 7 years, extent of fibrotic ILD on HRCT ≥ 10%, and FVC ≥ 40% predicted. FVC at baseline and decline in FVC over 52 weeks were compared with FVC values in hypothetical healthy reference subjects matched 1:1 to the subjects in the trial for age, sex, ethnicity and height, determined using equations published by the European Respiratory Society Global Lung Function Initiative. Results At baseline, mean (SD) FVC was 2460 (737) mL in the nintedanib group (n = 287) compared with 3403 (787) mL in the hypothetical matched healthy references. Mean (SD) FVC was 2544 (817) mL in the placebo group (n = 286) compared with 3516 (887) mL in the hypothetical matched healthy references. Mean (SE) changes in FVC at week 52, i.e., age-related loss of lung function, in the hypothetical healthy references matched to the nintedanib and placebo groups, respectively, were − 26.3 (0.5) mL and − 25.8 (0.5) mL. The difference in the change in FVC at week 52 between the nintedanib group and the hypothetical healthy references was 26.6 mL (95% CI: 1.2, 52.0; p = 0.04). The difference in the change in FVC at week 52 between the placebo group and the hypothetical healthy references was 77.5 mL (95% CI: 51.4, 103.7; p Conclusions Subjects with SSc-ILD in the SENSCIS trial had impaired lung function at baseline and experienced further deterioration over 52 weeks. The decline in FVC in the placebo group was four-fold greater than in a hypothetical group of matched healthy references, whereas the decline in FVC in patients who received nintedanib was two-fold greater than in hypothetical healthy references. These data highlight the clinical relevance of the slowing of FVC decline provided by nintedanib. Trial registration Registered 5 November 2015, https://clinicaltrials.gov/ct2/show/NCT02597933.

Published
2022

16. Prediction of improvement in skin fibrosis in diffuse cutaneous systemic sclerosis: a EUSTAR analysis

Author

Dobrota, Rucsandra, Maurer, Britta, Graf, Nicole, Jordan, Suzana, Mihai, Carina, Kowal-Bielecka, Otylia, Allanore, Yannick, Distler, Oliver, Cerinic, Marco Matucci, Guiducci, Serena, Walker, Ulrich, Lapadula, Giovanni, Iannone, Florenzo, Becvar, Radim, Sierakowsky, Stanislaw, Cutolo, Maurizio, Sulli, Alberto, Valentini, Gabriele, Cuomo, Giovanna, Vettori, Serena, Riemekasten, Gabriela, Siegert, Elise, Rednic, Simona, Nicoara, Ileana, Kahan, André, Vlachoyiannopoulos, P., Montecucco, C., Caporali, Roberto, Carreira, Patricia E., Novak, Srdan, Czirják, László, Varju, Cecilia, Chizzolini, Carlo, Kucharz, Eugene J., Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Wu, Chen, Marjanovic, Zora, Faivre, Helene, Hij, Darin, Dhamadi, Roza, Airò, Paolo, Hesselstrand, Roger, Wollheim, Frank, Wuttge, Dirk M, Andréasson, Kristofer, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Trotta, F., Lo Monaco, Andrea, Hunzelmann, Nicolas, Pellerito, Raffaele, Mauriziano, Ospedale, Bambara, Lisa Maria, Caramaschi, Paola, Black, Carol, Denton, Christopher, Damjanov, Nemanja, Henes, Jörg, Ortiz Santamaria, Vera, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthias, Burkhardt, Harald, Himsel, Andrea, Salvador, Maria J., Silva, José Antonio Pereira Da, Stamenkovic, Bojana, Stankovic, Aleksandra, Banja, Niska, Tikly, Mohammed, Ananieva, Lidia P., Denisov, Lev N., Müller-Ladner, Ulf, Frerix, Marc, Tarner, Ingo, Scorza, Raffaella, Clinica, U.O. Immunologia, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Mendoza, Antonio Zea, de la Puente, Carlos, Giraldo, Walter A. Sifuentes, Midtvedt, Øyvind, Reiseter, Silje, Hachulla, Eric, Launay, David, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Soare, Alina, Gorga, Marilena, Bojinca, Mihai, Schett, Georg, Distler, Jörg HW, Beyer, Christian, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco P., Corrado, Ada, Pozzi, Maria R., Eyerich, Kilian, Hein, Rüdiger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Krummel-Lorenz, Brigitte, Aringer, Martin, Günther, Claudia, Westhovens, Rene, de Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Radominski, Sebastião C., de Souza Müller, Carolina, Azevedo, Valderílio F., Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Popa, Sergei, Zenone, Thierry, Highton, John, Stamp, Lisa, Chapman, Peter, O’Donnell, John, Solanki, Kamal, Veale, Douglas, OʼRourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Amoroso, Antonio, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Chirieac, Rodica, Ancuta, Codrina, Villiger, Peter, Adler, Sabine, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Hélène, Litinsky, Ira, Venalis, Algirdas, Butrimiene, Irena, Venalis, Paulius, Rugiene, Rita, Karpec, Diana, Saketkoo, Lesley Ann, Lasky, Joseph A., Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Spertini, François, Ribi, Camillo, Buss, Guillaume, Pasquali, Jean Louis, Martin, Thierry, and Gorse, Audrey

Published
2016
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17. Effectiveness and safety of tocilizumab in patients with systemic sclerosis: a propensity score matched controlled observational study of the EUSTAR cohort

Author

Kuster, Simon, Jordan, Suzana, Elhai, Muriel, Held, Ulrike, Steigmiller, Klaus, Bruni, Cosimo, Cacciapaglia, Fabio, Vettori, Serena, Siegert, Elise, Rednic, Simona, Codullo, Veronica, Airo, Paolo, Braun-Moscovici, Yolanda, Hunzelmann, Nicolas, Salvador, Maria Joao, Riccieri, Valeria, Gheorghiu, Ana-Maria, Sancho, Juan Jose Alegre, Romanowska-Prochnicka, Katarzyna, Castellvi, Ivan, Koetter, Ina, Truchetet, Marie-Elise, Lopez-Longo, Fj, Novikov, Pavel, I, Giollo, Alessandro, Shirai, Yuichiro, Belloli, Laura, Zanatta, Elisabetta, Hachulla, Eric, Smith, Vanessa, Denton, Chris, Ionescu, Ruxandra M., Schmeiser, Tim, Distler, Joerg H. W., Gabrielli, Armando, Hoffmann-Vold, Anna-Maria, Kuwana, Masataka, Allanore, Yannick, Distler, Oliver, Kuster, Simon, Jordan, Suzana, Elhai, Muriel, Held, Ulrike, Steigmiller, Klaus, Bruni, Cosimo, Cacciapaglia, Fabio, Vettori, Serena, Siegert, Elise, Rednic, Simona, Codullo, Veronica, Airo, Paolo, Braun-Moscovici, Yolanda, Hunzelmann, Nicolas, Salvador, Maria Joao, Riccieri, Valeria, Gheorghiu, Ana-Maria, Sancho, Juan Jose Alegre, Romanowska-Prochnicka, Katarzyna, Castellvi, Ivan, Koetter, Ina, Truchetet, Marie-Elise, Lopez-Longo, Fj, Novikov, Pavel, I, Giollo, Alessandro, Shirai, Yuichiro, Belloli, Laura, Zanatta, Elisabetta, Hachulla, Eric, Smith, Vanessa, Denton, Chris, Ionescu, Ruxandra M., Schmeiser, Tim, Distler, Joerg H. W., Gabrielli, Armando, Hoffmann-Vold, Anna-Maria, Kuwana, Masataka, Allanore, Yannick, and Distler, Oliver

Abstract

Objectives Tocilizumab showed trends for improving skin fibrosis and prevented progression of lung fibrosis in systemic sclerosis ( SSc) in randomised controlled clinical trials. We aimed to assess safety and effectiveness of tocilizumab in a real-life setting using the European Scleroderma Trial and Research (EUSTAR) database. Methods Patients with SSc fulfilling the American College of Rheumatology (ACR)/EULAR 2013 classification criteria, with baseline and follow-up visits at 12 +/- 3 months, receiving tocilizumab or standard of care as the control group, were selected. Propensity score matching was applied. Primary endpoints were the modified Rodnan skin score (mRSS) and FVC at 12 +/- 3 months compared between the groups. Secondary endpoints were the percentage of progressive/regressive patients for skin and lung at 12 +/- 3 months. Results Ninety-three patients with SSc treated with tocilizumab and 3180 patients with SSc with standard of care fulfilled the inclusion criteria. Comparison between groups did not show significant differences, but favoured tocilizumab across all predefined primary and secondary endpoints: mRSS was lower in the tocilizumab group (difference -1.0, 95% CI -3.7 to 1.8, p=0.48). Similarly, FVC % predicted was higher in the tocilizumab group (difference 1.5 (-6.1 to 9.1), p=0.70). The percentage of progressive/regressive patients favoured tocilizumab over controls. These results were robust regarding the sensitivity analyses. Safety analysis confirmed previously reported adverse event profiles. Conclusion Although this large, observational, controlled, real-life EUSTAR study did not show significant effectiveness of tocilizumab on skin and lung fibrosis, the consistency of direction of all predefined endpoints generates hypothesis for potential effectiveness in a broader SSc population.

Published
2022

18. Effect of Nintedanib on Progression of Systemic Sclerosis-Associated Interstitial Lung Disease Over 100 Weeks: Data From a Randomized Controlled Trial

Author

Assassi, Shervin; https://orcid.org/0000-0002-8059-9978, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Allanore, Yannick; https://orcid.org/0000-0002-6149-0002, Ogura, Takashi, Varga, John, Vettori, Serena, Crestani, Bruno, Voss, Florian, Alves, Margarida, Stowasser, Susanne, Maher, Toby M, SENSCIS trial investigators, Assassi, Shervin; https://orcid.org/0000-0002-8059-9978, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Allanore, Yannick; https://orcid.org/0000-0002-6149-0002, Ogura, Takashi, Varga, John, Vettori, Serena, Crestani, Bruno, Voss, Florian, Alves, Margarida, Stowasser, Susanne, Maher, Toby M, and SENSCIS trial investigators

Abstract

OBJECTIVE: In the SENSCIS trial, participants with systemic sclerosis-associated interstitial lung disease (SSc-ILD) were randomized to receive nintedanib or placebo until the last participant reached week 52 but for 100 weeks or less. Nintedanib reduced the rate of decline in forced vital capacity (FVC) (ml/year) over 52 weeks by 44% (41 ml [95% confidence interval (95% CI): 2.9-79.0]) versus placebo. We investigated the effect of nintedanib over the whole SENSCIS trial. METHODS: The annual rate of decline in FVC (ml/year) over the whole trial was assessed descriptively using 1) on-treatment data plus off-treatment data from participants who prematurely discontinued treatment (intent-to-treat analysis) and 2) only on-treatment data to assess the effect of nintedanib in participants who remained on treatment. RESULTS: In the intent-to-treat analysis, the adjusted mean (SE) annual rate of decline in FVC over 100 weeks was -54.9 (11.1) and -88.8 (10.9) ml/year in the nintedanib (n = 287) and placebo (n = 288) groups, respectively (difference 34.0 ml/year [95% CI: 3.4-64.5]). In the on-treatment analysis, the adjusted mean (SE) annual rate of decline in FVC over 100 weeks was -55.1 (12.3) and -94.0 (11.7) ml/year in the nintedanib (n = 286) and placebo (n = 288) groups, respectively (difference 38.9 ml/year [95% CI: 5.6-72.1]). The adverse event profile of nintedanib over 100 weeks was consistent with that observed over 52 weeks. CONCLUSION: Nintedanib provides a sustained benefit on slowing the progression of SSc-ILD over 100 weeks, with adverse events that are manageable for most patients.

Published
2022

19. Mapping and predicting mortality from systemic sclerosis

Author

Elhai, Muriel, Meune, Christophe, Boubaya, Marouane, Avouac, Jérôme, Hachulla, Eric, Balbir-Gurman, Alexandra, Riemekasten, Gabriela, Airò, Paolo, Joven, Beatriz, Vettori, Serena, Cozzi, Franco, Ullman, Susanne, Czirják, László, Tikly, Mohammed, Müller-Ladner, Ulf, Caramaschi, Paola, Distler, Oliver, Iannone, Florenzo, Ananieva, Lidia P, Hesselstrand, Roger, Becvar, Radim, Gabrielli, Armando, Damjanov, Nemanja, Salvador, Maria J, Riccieri, Valeria, Mihai, Carina, Szücs, Gabriella, Walker, Ulrich A, Hunzelmann, Nicolas, Martinovic, Duska, Smith, Vanessa, Müller, Carolina de Souza, Montecucco, Carlo Maurizio, Opris, Daniela, Ingegnoli, Francesca, Vlachoyiannopoulos, Panayiotis G, Stamenkovic, Bojana, Rosato, Edoardo, Heitmann, Stefan, Distler, Jörg H W, Zenone, Thierry, Seidel, Matthias, Vacca, Alessandra, Langhe, Ellen De, Novak, Srdan, Cutolo, Maurizio, Mouthon, Luc, Henes, Jörg, Chizzolini, Carlo, Mühlen, Carlos Alberto von, Solanki, Kamal, Rednic, Simona, Stamp, Lisa, Anic, Branimir, Santamaria, Vera Ortiz, Santis, Maria De, Yavuz, Sule, Sifuentes-Giraldo, Walter Alberto, Chatelus, Emmanuel, Stork, Jiri, Laar, Jacob van, Loyo, Esthela, García de la Peña Lefebvre, Paloma, Eyerich, Kilian, Cosentino, Vanesa, Alegre-Sancho, Juan Jose, Kowal-Bielecka, Otylia, Rey, Grégoire, Matucci-Cerinic, Marco, and Allanore, Yannick

Published
2017
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20. The European Scleroderma Trials and Research group (EUSTAR) task force for the development of revised activity criteria for systemic sclerosis: derivation and validation of a preliminarily revised EUSTAR activity index

Author

Valentini, Gabriele, Iudici, Michele, Walker, Ulrich A, Jaeger, Veronika K, Baron, Murray, Carreira, Patricia, Czirják, László, Denton, Christopher P, Distler, Oliver, Hachulla, Eric, Herrick, Ariane L, Kowal-Bielecka, Otylia, Pope, Janet, Müller-Ladner, Ulf, Riemekasten, Gabriela, Avouac, Jerome, Frerix, Marc, Jordan, Suzana, Minier, Tünde, Siegert, Elise, Ong, Voon H, Vettori, Serena, and Allanore, Yannick

Published
2017
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21. Additional file 2 of Decline in forced vital capacity in subjects with systemic sclerosis-associated interstitial lung disease in the SENSCIS trial compared with healthy reference subjects

Author

Maher, Toby M., Bourdin, Arnaud, Volkmann, Elizabeth R., Vettori, Serena, Distler, Jörg H. W., Alves, Margarida, Stock, Christian, and Distler, Oliver

Abstract

Additional file 2: Table S1. Baseline FVC (mL) in subjects in the SENSCIS trial versus healthy reference subjects in subgroups by baseline characteristics.

Published
2022
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24. A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: a EUSTAR prospective study

Author

Elhai, Muriel, Avouac, Jérôme, Walker, Ulrich A, Matucci-Cerinic, Marco, Riemekasten, Gabriela, Airò, Paolo, Hachulla, Eric, Valentini, Gabriele, Carreira, Patricia E, Cozzi, Franco, Balbir Gurman, Alexandra, Braun-Moscovici, Yolanda, Damjanov, Nemanja, Ananieva, Lidia P, Scorza, Raffaella, Jimenez, Sergio, Busquets, Joanna, Li, Mengtao, Müller-Ladner, Ulf, Kahan, André, Distler, Oliver, Allanore, Yannick, Guiducci, Serena, Tyndall, Alan, Lapadula, Giovanni, Iannone, Florenzo, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Cutolo, Maurizio, Sulli, Alberto, Cuomo, Giovanna, Vettori, Serena, Rednic, Simona, Nicoara, Ileana, Vlachoyiannopoulos, P., Montecucco, C., Caporali, Roberto, Novak, Srdan, Czirják, László, Varju, Cecilia, Chizzolini, Carlo, Kucharz, Eugene J., Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Hij, Adrian, Hesselstrand, Roger, Scheja, Agneta, Wollheim, Frank, Martinovic, Duska, Govoni, M., Monaco, Andrea Lo, Hunzelmann, Nicolas, Pellerito, Raffaele, Bambara, Lisa Maria, Caramaschi, Paola, Black, Carol, Denton, Christopher, Henes, Jörg, Santamaria, Vera Ortiz, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthias, Oleszowsky, Mara, Burkhardt, Harald, Himsel, Andrea, Salvador, Maria J., Stamenkovic, Bojana, Stankovic, Aleksandra, Tikly, Mohammed, Starovoytova, Maya N., Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szücs, Gabriella, Zea Mendoza, Antonio, de la Puente Buijdos, Carlos, Sifuentes Giraldo, Walter A., Midtvedt, Øyvind, Garen, Torhild, Launay, David, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Wigley, Fredrick M., Mihai, Carmen M., Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Gorga, Marilena, Dobrota, Rucsandra, Bojinca, Mihai, Schett, Georg, Distler, Jörg HW, Meroni, Pierluigi, Zeni, Silvana, Mouthon, Luc, Keyser, Filip De, Smith, Vanessa, Cantatore, Francesco P., Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria R., Eyerich, Kilian, Hein, Rüdiger, Knott, Elisabeth, Szechinski, Jacek, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Günther, Claudia, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Radominski, Sebastião C., Müller, Carolina de Souza, Azevedo, Valderílio F., Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Zenone, Thierry, Stebbings, Simon, Highton, John, Stamp, Lisa, Chapman, Peter, Baron, Murray, OʼDonnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, OʼRourke, Marie, Loyo, Esthela, Rosato, Edoardo, Pisarri, Simonetta, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Chirieac, Rodica, Ancuta, Codrina, Furst, Daniel E., Kafaja, Suzanne, Lefebvre, Paloma García de la Peña, Rubio, Silvia Rodriguez, Exposito, Marta Valero, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Hélène, Litinsky, Ira, Venalis, Algirdas, Butrimiene, Irena, Venalis, Paulius, Rugiene, Rita, Karpec, Diana, Kerzberg, Eduardo, Montoya, Fabiana, and Cosentino, Vanesa

Published
2016
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26. Prediction of worsening of skin fibrosis in patients with diffuse cutaneous systemic sclerosis using the EUSTAR database

Author

Maurer, Britta, Graf, Nicole, Michel, Beat A, Müller-Ladner, Ulf, Czirják, László, Denton, Christopher P, Tyndall, Alan, Metzig, Carola, Lanius, Vivian, Khanna, Dinesh, Distler, Oliver, Tarner, Ingo H, Cerinic, Marco Matucci, Guiducci, Serena, Walker, Ulrich, Lapadula, Giovanni, Iannone, Florenzo, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Cutolo, Maurizio, Sulli, Alberto, Valentini, Gabriele, Cuomo, Giovanna, Vettori, Serena, Riemekasten, Gabriele, Rednic, Simona, Nicoara, Ileana, Kahan, André, Allanore, Yannick, Vlachoyiannopoulos, P, Montecucco, C, Caporali, Roberto, Carreira, Patricia E, Novak, Srdan, Varju, Cecilia, Chizzolini, Carlo, Kucharz, Eugene J, Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Cozzi, Franco, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Hij, Adrian, Airò, Paolo, Hesselstrand, Roger, Scheja, Agneta, Wollheim, Frank, Martinovic, Duska, Gurman, Alexandra Balbir, Braun-Moscovici, Yolanda, Govoni, M, Monaco, Andrea Lo, Hunzelmann, Nicolas, Pellerito, Raffaele, Bambara, Lisa Maria, Caramaschi, Paola, Black, Carol, Damjanov, Nemanja, Santamaria, Vera Ortiz, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthias, Oleszowsky, Mara, Burkhardt, Harald, Himsel, Andrea, Salvador, Maria J, Stamenkovic, Bojana, Stankovic, Aleksandra, Tikly, Mohammed, Starovoytova, Maya N, Ananieva, Lidia P, Scorza, Raffaella, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szücs, Gabriella, Mendoza, Antonio Zea, Buijdos, Carlos de la Puente, Sifuentes Giraldo, Walter A., Midtvedt, Øyvind, Garen, Torhild, Hachulla, Eric, Launay, David, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Wigley, Fredrick M, Mihai, Carmen M, Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Gorga, Marilena, Dobrota, Rucsandra, Bojinca, Mihai, Schett, Georg, Distler, Jörg HW, Meroni, Pierluigi, Zeni, Silvana, Mouthon, Luc, Keyser, Filip De, Cantatore, Francesco P, Corrado, Ada, Ullman, Susanne, Iversen, Line, Pozzi, Maria R, Eyerich, Kilian, Hein, Rüdiger, Knott, Elisabeth, Szechinski, Jacek, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Günther, Claudia, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Radominski, Sebastião C, Müller, Carolina de Souza, Azevedo, Valderílio F, Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Zenone, Thierry, Highton, John, Stamp, Lisa, Chapman, Peter, OʼDonnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O’Rourke, Marie, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Pisarri, Simonetta, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Chirieac, Rodica, Ancuta, Codrina, E Furst, Daniel, Kafaja, Suzanne, Lefebvre, Paloma García de la Peña, Rubio, Silvia Rodriguez, Exposito, Marta Valero, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Hélène, Litinsky, Ira, Venalis, Algirdas, Butrimiene, Irena, Venalis, Paulius, Rugiene, Rita, Karpec, Diana, Kerzberg, Eduardo, Montoya, Fabiana, and Cosentino, Vanesa

Published
2015
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30. Incidence and risk factors for gangrene in patients with systemic sclerosis from the EUSTAR cohort

Author

Mihai, Carina, Distler, Oliver, Gheorghiu, Ana Maria, Constantin, Paul I, Dobrota, Rucsandra, Jordan, Suzana, Smith, Vanessa, Hachulla, Eric, Henes, Jörg, Siegert, Elise, Vettori, Serena, Müller-Ladner, Ulf, Matucci Cerinic, Marco, Allanore, Yannick, Lepri, G, Jaeger, Vk, Walker, Ua, Iannone, F, Cacciapaglia, F, Tomčík, M, Becvar, R, Rednic, S, Petcu, A, Szabo, I, Codullo, V, Caporali, R, Montecucco, C, Carreira, P, Ioven, B, Minier, T, Czirják, L, Chizzolini, C, Allali, D, Zanatta, E, Doria, A, Gabrielli, A, Airò, P, Lazzaroni, Mg, Radić, M, Martinovic, D, Braun-Moscovici, Y, Balbir-Gurman, A, Hunzelmann, N, Caramaschi, P, Morovic-Vergles, J, Denton, C, Santamaria, V, Heitmann, S, Krasowska, D, Michalska-Jakubus, M, Seidel, M, Foeldvari, I, Helmus, N, Salvador, M, Stamenkovic, B, Stankovic, A, Ananieva, L, Herrick, A, Engelhart, M, De La Puente, C, Hoffmann-Vold, Am, Midtvedt, Ø, Launay, D, Sobanski, V, Riccieri, V, Opris-Belinski, D, Groseanu, L, Ionescu, R, Bojinca, M, Sunderkötter, C, Distler, J, Ingegnoli, F, van der Haecke, A, Ullman, S, Pozzi, Mr, Eyerich, K, Vanthuyne, M, Erler, A, Aringer, M, De Langhe, E, Baresic, M, Mayer, M, Anic, B, Yavuz, S, Granel, B, Popa, S, Agachi, S, Zenone, T, Mathieu, A, Vacca, A, Solanki, K, Veale, D, Loyo, E, Tineo, C, Gigante, A, Rosato, E, Oksel, F, Yagurcu, F, Tănăseanu, Cm, Visalli, E, Benenati, A, Foti, R, Ancuta, C, Dan, D, Adler, S, Villiger, P, Fathi, N, de la Peña Lefebvre PG, González Martín, J, Chatelus, E, Sibilia, J, Litinsky, I, Del Galdo, F, Ann Sakettkoo, L, Kerzberg, E, Bianchi, Wa, Bianchi, Bv, Castellví, I, Limonta, M, Rimar, D, Couto, M, Ribi, C, Spertini, F, Kahl, S, Hsu, V, Poindron, V, Meghit, K, Martin, T, Kolstad, K, Chung, L, Thiele, A, Schmeiser, T, Zdrojewski, Z, Riemekasten, G, Levy, Y, Cardoneanu, A, Burlui, A, Rezus, E, Pamuk, On, Talotta, R, Bongiovanni, S, Puttini, Ps., Mihai, Carina, Distler, Oliver, Gheorghiu, Ana Maria, Constantin, Paul I, Dobrota, Rucsandra, Jordan, Suzana, Smith, Vanessa, Hachulla, Eric, Henes, Jörg, Siegert, Elise, Vettori, Serena, Müller-Ladner, Ulf, Matucci Cerinic, Marco, Allanore, Yannick, Giovanna, Cuomo, Chizzolini, Carlo, Allali, Danièle, and University of Zurich

Subjects
Adult, Male, medicine.medical_specialty, Databases, Factual, systemic sclerosis, digital ulcer, 610 Medicine & health, Disease, ddc:616.07, Logistic regression, Systemic scleroderma, Cohort Studies, Rheumatology, Risk Factors, Internal medicine, Cox proportional hazards regression, medicine, Humans, Pharmacology (medical), In patient, digital ulcers, gangrene, vasculopathy, Aged, Gangrene, Scleroderma, Systemic, business.industry, Incidence (epidemiology), Incidence, 10051 Rheumatology Clinic and Institute of Physical Medicine, food and beverages, Middle Aged, medicine.disease, Cross-Sectional Studies, Cohort, Female, business, systemic sclerosi
Abstract

Objective In patients with SSc, peripheral vasculopathy can promote critical ischaemia and gangrene. The aim of this study was to investigate the prevalence, incidence and risk factors for gangrene in the EUSTAR cohort. Methods We included patients from the EUSTAR database fulfilling the ACR 1980 or the ACR/EULAR 2013 classification criteria for SSc, with at least one visit recording data on gangrene. Centres were asked for supplementary data on traditional cardiovascular risk factors. We analysed the cross-sectional relationship between gangrene and its potential risk factors by univariable and multivariable logistic regression. Longitudinal data were analysed by Cox proportional hazards regression. Results 1757 patients were analysed (age 55.9 [14.5] years, disease duration 7.9 [10.3] years, male sex 16.7%, 24.6% diffuse cutaneous subset [dcSSc]). At inclusion, 8.9% of patients had current or previous digital gangrene, 16.1% had current digital ulcers (DUs) and 42.7% had ever had DUs (current or previous). Older age, DUs ever and dcSSc were statistically significant risk factors for gangrene in the cross-sectional multivariable model. During a median follow-up of 13.1 months, 16/771 (0.9%) patients developed gangrene. All 16 patients who developed gangrene had previously had DUs and gangrene. Further risk factors for incident gangrene were the dcSSc subset and longer disease duration. Conclusion In unselected SSc patients, gangrene occurs in about 9% of SSc patients. DUs ever and, to a lesser extent, the dcSSc subset are strongly and independently associated with gangrene, while traditional cardiovascular risk factors could not be identified as risk factors.

Published
2019

31. Phenotypes determined by cluster analysis and their survival in the prospective EUSTAR cohort of patients with systemic sclerosis

Author

Sobanski, Vincent, Giovannelli, Jonathan, Allanore, Yannick, Riemekasten, Gabriela, Airò, Paolo, Vettori, Serena, Cozzi, Franco, Distler, Oliver, Matucci-Cerinic, Marco, Denton, Christopher P, Launay, David, Hachulla, Eric, EUSTAR collaborators, University of Zurich, Launay, David, Sobanski, Vincent, Giovannelli, Jonathan, Allanore, Yannick, Riemekasten, Gabriela, Airò, Paolo, Vettori, Serena, Cozzi, Franco, Distler, Oliver, Matucci-Cerinic, Marco, Denton, Christopher, Hachulla, Eric, and Cuomo, Giovanna

Subjects
Systemic sclerosi, 2403 Immunology, organ involvement, 2745 Rheumatology, cluster analysi, 10051 Rheumatology Clinic and Institute of Physical Medicine, 2723 Immunology and Allergy, 610 Medicine & health, heterogeneity, autoantibody
Abstract

Systemic sclerosis (SSc) is a heterogeneous connective tissue disease. The usual subclassification divides patients into limited (lc) and diffuse cutaneous (dc) subsets relying on the skin extension but may not capture the entire variability of clinical phenotypes. The European Scleroderma Trials and Research (EUSTAR) database is a prospective cohort providing data from 137 European referral centers. We performed a cluster analysis to distinguish and characterize homogeneous phenotypes without any a priori, and examined survival among the obtained clusters.

Published
2019
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32. Use of vasoactive/vasodilating drugs for systemic sclerosis (SSc)-related digital ulcers (DUs) in expert tertiary centres: results from the analysis of the observational real-life DeSScipher study

Author

Blagojevic, Jelena, Abignano, Giuseppina, Avouac, Jérôme, Cometi, L, Frerix, Marc, Bellando-Randone, Silvia, Guiducci, Serena, Bruni, Cosimo, Huscher, Dörte, Jaeger, V K, Lóránd, Veronika, Maurer, Britta, Nihtyanova, Svetlana, Riemekasten, Gabriela, Siegert, Elise, Tarner, I H, Vettori, Serena, Walker, U A, Czirják, László, Denton, C P, Distler, Oliver, Allanore, Yannick, Müller-Ladner, Ulf, Moggi-Pignone, Alberto, Matucci-Cerinic, Marco, Del Galdo, Francesco, EUSTAR co-workers, University of Zurich, and Blagojevic, Jelena

Subjects
2745 Rheumatology, 10051 Rheumatology Clinic and Institute of Physical Medicine, 610 Medicine & health
Published
2020
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33. Translational engagement of LPA$_{1}$ receptor in skin fibrosis: from dermal fibroblasts of patients with scleroderma to Tsk1 mouse

Author

Ledein, Laetitia, Léger, Bertrand, Dees, Clara, Beyer, Christian, Distler, Alfiya, Vettori, Serena, Boukaiba, Rachid, Bidouard, Jean Pierre, Schaefer, Matthias, Pernerstorfer, Josef, Ruetten, Hartmut, Jagerschmidt, Alexandre, Janiak, Philip, Distler, Jörg H W, Distler, Oliver, Illiano, Stéphane, and University of Zurich

Subjects
10051 Rheumatology Clinic and Institute of Physical Medicine, 610 Medicine & health
Published
2020
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34. Prediction of organ involvement and survival in systemic sclerosis patients in the first 5years from diagnosis

Author

van den Hombergh, Wieneke M. T., Knaapen-Hans, Hanneke K. A., van den Hoogen, Frank H. J., Carreira, Patricia, Distler, Oliver, Hesselstrand, Roger, Hunzelmann, Nicolas, Vettori, Serena, Fransen, Jaap, Vonk, Madelon C., van den Hombergh, Wieneke M. T., Knaapen-Hans, Hanneke K. A., van den Hoogen, Frank H. J., Carreira, Patricia, Distler, Oliver, Hesselstrand, Roger, Hunzelmann, Nicolas, Vettori, Serena, Fransen, Jaap, and Vonk, Madelon C.

Abstract

Background: Organ involvement often occurs in early systemic sclerosis and has been related to premature death. Identifying patients at diagnosis at risk of developing early organ involvement would be useful to optimize screening and management strategies. Objective: To develop prediction models for the 5-year development of interstitial lung disease, pulmonary arterial hypertension and death. Methods: A European multicentre inception cohort was created. For modelling, predefined clinical variables with known predictive value at diagnosis were used. Univariate and multivariate regression analysis were done to select baseline predictors and build the prediction models. The models were tested using the area under the receiver operating characteristic curve comparing observed and expected frequencies. Results: Of 735 patients, 23% developed interstitial lung disease, 8% developed pulmonary arterial hypertension 12% died. The interstitial lung disease model included diffuse cutaneous systemic sclerosis (OR = 1.8), systemic sclerosis disease duration<3years (OR = 1.4), puffy fingers (OR = 1.6), and anti-topoisomerase-I-antibodies (OR = 1.8). The pulmonary arterial hypertension model included age>65years (OR = 3.2), forced vital capacity<70% (OR = 2.5) and diffusing capacity of the lung for carbon monoxide<55% (OR = 1.9). Death was predicted best by age>65years (OR = 4.1), male gender (OR = 1.9), no anti-centromere antibodies (OR = 0.5), proteinuria (OR = 1.9), forced vital capacity<70% (OR = 1.8) and pulmonary arterial hypertension at diagnosis (OR = 10.1). The area under the receiver operating characteristic was 0.66 (95% CI 0.64-0.67), 0.66 (95% CI 0.64-0.68) and 0.70 (95% CI 0.69-0.72), respectively. Conclusion: We have shown that it is possible to predict interstitial lung disease, pulmonary arterial hypertension and death using established variables already available at the moment of systemic sclerosis diagnosis. Discriminatory performance of the models was suboptim

Published
2020

35. EUSTAR biobanking: recommendations for the collection, storage and distribution of biospecimens in scleroderma research

Author

Beyer, Christian, Distler, Jörg H W, Allanore, Yannick, Aringer, Martin, Avouac, Jérôme, Czirják, László, Cutolo, Maurizio, Damjanov, Nemanja, Del Galdo, Francesco, Fligelstone, Kim, Guiducci, Serena, Kowal-Bielecka, Otylia, van Laar, Jacob M, Martucci-Cerinic, Marco, Müller-Ladner, Ulf, Riemekasten, Gabriela, Tarner, Ingo H, Tyndall, Alan, Kennedy, Ann Tyrrell, Valentini, Gabriele, Vettori, Serena, Walker, Ulrich A, Denton, Christopher, and Distler, Oliver

Published
2011
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37. Idiopathic pulmonary fibrosis and systemic sclerosis-interstitial lung disease: features at admission to a tertiary pulmonology unit

Author

Vettori, Serena, primary, Lanza, Maurizia, additional, Annunziata, Anna, additional, Cauteruccio, Rosa, additional, Coppola, Antonietta, additional, Di Spirito, Valentina, additional, De Simone, Francesco, additional, Gioia, Sara, additional, Imitazione, Pasquale, additional, Stefanelli, Francesco, additional, and Fiorentino, Giuseppe, additional

Published
2020
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38. Translational engagement of lysophosphatidic acid receptor 1 in skin fibrosis: from dermal fibroblasts of patients with scleroderma to tight skin 1 mouse

Author

Ledein, Laetitia, primary, Léger, Bertrand, additional, Dees, Clara, additional, Beyer, Christian, additional, Distler, Alfiya, additional, Vettori, Serena, additional, Boukaiba, Rachid, additional, Bidouard, Jean Pierre, additional, Schaefer, Matthias, additional, Pernerstorfer, Josef, additional, Ruetten, Hartmut, additional, Jagerschmidt, Alexandre, additional, Janiak, Philip, additional, Distler, Jörg H.W., additional, Distler, Oliver, additional, and Illiano, Stéphane, additional

Published
2020
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40. Outcomes of limited cutaneous systemic sclerosis patients: Results on more than 12,000 patients from the EUSTAR database

Author

Frantz, Camelia, primary, Huscher, Dorte, additional, Avouac, Jérôme, additional, Hachulla, Eric, additional, Balbir-Gurman, Alexandra, additional, Riemekasten, Gabriela, additional, Siegert, Elise, additional, Lazzaroni, Maria-Grazia, additional, Carreira, Patricia E., additional, Vettori, Serena, additional, Zanatta, Elisabetta, additional, Ullman, Susanne, additional, Czirjàk, Laszlo, additional, Kowal-Bielecka, Otylia, additional, Distler, Oliver, additional, Matucci-Cerinic, Marco, additional, and Allanore, Yannick, additional

Published
2020
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42. Outcomes of patients with systemic sclerosis treated with rituximab in contemporary practice: a prospective cohort study

Author

Elhai, Muriel, Boubaya, Marouane, Distler, Oliver, Smith, Vanessa, Matucci-Cerinic, Marco, Alegre Sancho, Juan José, Truchetet, Marie-Elise, Braun-Moscovici, Yolanda, Iannone, Florenzo, Novikov, Pavel I, Lescoat, Alain, Siegert, Elise, Castellví, Ivan, Airó, Paolo, Vettori, Serena, De Langhe, Ellen, Hachulla, Eric, Erler, Anne, Ananieva, Lidia, Krusche, Martin, López-Longo, F J, Distler, Jörg H W, Hunzelmann, Nicolas, Hoffmann-Vold, Anna-Maria, Riccieri, Valeria, Hsu, Vivien M, Pozzi, Maria R, Ancuta, Codrina, Rosato, Edoardo, Mihai, Carina, et al, University of Zurich, and Elhai, Muriel

Subjects
2403 Immunology, 1300 General Biochemistry, Genetics and Molecular Biology, 2745 Rheumatology, 10051 Rheumatology Clinic and Institute of Physical Medicine, 2723 Immunology and Allergy, 610 Medicine & health
Published
2019

43. Differences associated with age at onset in early systemic sclerosis patients: a report from the EULAR Scleroderma Trials and Research Group (EUSTAR) database

Author

Carreira, Patricia E, Carmona, Loreto, Joven, Beatriz E, Loza, Estíbaliz, Andreu, Jose Luis, Riemekasten, Gabriela, Vettori, Serena, Balbir-Gurman, Alexandra, Airò, Paolo, Walker, Ulrich A, Damjanov, Nemanja, Matucci-Cerinic, Marco, Ananieva, Lidia P, Rednic, Simona, Czirják, László, Distler, Oliver, Farge, Dominique, Hesselstrand, Roger, Corrado, A, Caramaschi, Paola, Tikly, Mohammed, Allanore, Yannick, EUSTAR co-authors, University of Zurich, and Carreira, Patricia E

Subjects
2403 Immunology, Rheumatology, 2745 Rheumatology, Immunology, 10051 Rheumatology Clinic and Institute of Physical Medicine, 2723 Immunology and Allergy, Immunology and Allergy, 610 Medicine & health, General Medicine
Published
2019
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44. Phenotypes determined by cluster analysis and their survival in the prospective European Scleroderma Trials and Research cohort of patients with systemic sclerosis

Author

Sobanski, Vincent, Giovannelli, Jonathan, Allanore, Yannick, Riemekasten, Gabriela, Airo, Paolo, Vettori, Serena, Cozzi, Franco, Distler, Oliver, Matucci-Cerinic, Marco, Denton, Christopher, Launay, David, Hachulla, Eric, Cerinic, Marco Matucci, Guiducci, Serena, Walker, Ulrich, Kyburz, Diego, Lapadula, Giovanni, Iannone, Florenzo, Maurer, Britta, Jordan, Suzana, Becvar, Radim, Sierakowsky, Stanislaw, Bielecka, Otylia Kowal, Cutolo, Maurizio, Sulli, Alberto, Valentini, Gabriele, Cuomo, Giovanna, Siegert, Elise, Rednic, Simona, Nicoara, Ileana, Kahan, Andre, Vlachoyiannopoulos, Panayiotis, Montecucco, Carlo, Caporali, Roberto, Stork, Jiri, Inanc, Murat, Carreira, Patricia E, Novak, Srdan, Czirjak, Laszlo, Varju, Cecilia, Chizzolini, Carlo, Kucharz, Eugene J, Kotulska, Anna, Kopec-Medrek, Magdalena, Widuchowska, Malgorzata, Rozman, Blaz, Mallia, Carmel, Coleiro, Bernard, Gabrielli, Armando, Farge, Dominique, Wu, Chen, Marjanovic, Zora, Faivre, Helene, Hij, Darin, Dhamadi, Roza, Hesselstrand, Roger, Wollheim, Frank, Wuttge, Dirk M, Andreasson, Kristofer, Martinovic, Duska, Balbir-Gurman, Alexandra, Braun-Moscovici, Yolanda, Trotta, Francesco, Lo Monaco, Andrea, Hunzelmann, Nicolas, Pellerito, Raffaele, Bambara, Lisa Maria, Caramaschi, Paola, Morovic-Vergles, Jadranka, Black, Carol, Damjanov, Nemanja, Henes, Joerg, Ortiz Santamaria, Vera, Heitmann, Stefan, Krasowska, Dorota, Seidel, Matthias, Hasler, Paul, Burkhardt, Harald, Himsel, Andrea, Bajocchi, Gianluigi, Nuova, Arcispedale Santa Maria, Salvador, Maria Joao, Pereira Da Silva, Jose Antonio, Stamenkovic, Bojana, Stankovic, Aleksandra, Selmi, Carlo Francesco, De Santis, Maria, Marasini, Bianca, Tikly, Mohammed, Ananieva, Lidia P, Denisov, Lev N, Mueller-Ladner, Ulf, Frerix, Marc, Tarner, Ingo, Scorza, Raffaella, Puppo, Francesco, Engelhart, Merete, Strauss, Gitte, Nielsen, Henrik, Damgaard, Kirsten, Szucs, Gabriella, Szamosi, Szilvia, Zea Mendoza, Antonio, de la Puente, Carlos, Sifuentes Giraldo, Walter Alberto, Midtvedt, Oyvind, Reiseter, Silje, Garen, Torhild, Valesini, Guido, Riccieri, Valeria, Ionescu, Ruxandra Maria, Opris, Daniela, Groseanu, Laura, Wigley, Fredrick M, Cornateanu, Roxana Sfrent, Ionitescu, Razvan, Gherghe, Ana Maria, Soare, Alina, Gorga, Marilena, Bojinca, Mihai, Mihai, Carina, Milicescu, Mihaela, Sunderkoetter, Cord, Kuhn, Annegret, Sandorfi, Nora, Schett, Georg, Distler, Joerg HW, Beyer, Christian, Meroni, Pierluigi, Ingegnoli, Francesca, Mouthon, Luc, De Keyser, Filip, Smith, Vanessa, Cantatore, Francesco Paolo, Corrado, Ada, Ullman, Susanne, Iversen, Line, von Muehlen, Carlos Alberto, Bohn, Jussara Marilu, Lonzetti, Lilian Scussel, Pozzi, Maria Rosa, Eyerich, Kilian, Hein, Ruediger, Knott, Elisabeth, Wiland, Piotr, Szmyrka-Kaczmarek, Magdalena, Sokolik, Renata, Morgiel, Ewa, Madej, Marta, Houssiau, Frederic A, Jose Alegre-Sancho, Juan, Krummel-Lorenz, Brigitte, Saar, Petra, Aringer, Martin, Guenther, Claudia, Westhovens, Rene, de Langhe, Ellen, Lenaerts, Jan, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Uprus, Maria, Otsa, Kati, Yavuz, Sule, Granel, Brigitte, Radominski, Sebastiao Cezar, Mueller, Carolina de Souza, Azevedo, Valderilio Feijo, Jimenez, Sergio, Busquets, Joanna, Agachi, Svetlana, Groppa, Liliana, Chiaburu, Lealea, Russu, Eugen, Popa, Sergei, Zenone, Thierry, Pileckyte, Margarita, Stebbings, Simon, Highton, John, Mathieu, Alessandro, Vacca, Alessandra, Sampaio-Barros, Percival D, Yoshinari, Natalino H, Marangoni, Roberta G, Martin, Patricia, Fuocco, Luiza, Stamp, Lisa, Chapman, Peter, O'Donnell, John, Solanki, Kamal, Doube, Alan, Veale, Douglas, O'Rourke, Marie, Loyo, Esthela, Li, Mengtao, Mohamed, Walid Ahmed Abdel Atty, Rosato, Edoardo, Amoroso, Antonio, Gigante, Antonietta, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Popescu, Monica, Dumitrascu, Alina, Tiglea, Isabela, Foti, Rosario, Chirieac, Rodica, Ancuta, Codrina, Furst, Daniel E, Villiger, Peter, Adler, Sabine, van Laar, Jacob, Kayser, Cristiane, Eduardo, Andrade Luis C, Fathi, Nihal, Hassanien, Manal, de la Pena Lefebvre, Paloma Garcia, Rodriguez Rubio, Silvia, Valero Exposito, Marta, Sibilia, Jean, Chatelus, Emmanuel, Gottenberg, Jacques Eric, Chifflot, Helene, Litinsky, Ira, Emery, Paul, Buch, Maya, Del Galdo, Francesco, Venalis, Algirdas, Butrimiene, Irena, Venalis, Paulius, Rugiene, Rita, Karpec, Diana, Saketkoo, Lesley Ann, Lasky, Joseph A, Kerzberg, Eduardo, Montoya, Fabiana, Cosentino, Vanesa, Limonta, Massimiliano, Brucato, Antonio Luca, Lupi, Elide, Rosner, Itzhak, Rozenbaum, Michael, Slobodin, Gleb, Boulman, Nina, Rimar, Doron, Couto, Maura, Spertini, Francois, Ribi, Camillo, Buss, Guillaume, Kahl, Sarah, Hsu, Vivien M, Chen, Fei, McCloskey, Deborah, Malveaux, Halina, Pasquali, Jean Louis, Martin, Thierry, Gorse, Audrey, Guffroy, Aurelien, Poindron, Vincent, EUSTAR Collaborators, Guiducci, S., Walker, U., Kyburz, D., Lapadula, G., Iannone, F., Maurer, B., Jordan, S., Becvar, R., Sierakowsky, S., Kowal Bielecka, O., Cutolo, M., Sulli, A., Valentini, G., Cuomo, G., Siegert, E., Rednic, S., Nicoara, I., Kahan, A., Vlachoyiannopoulos, P., Montecucco, C., Caporali, R., Stork, J., Inanc, M., Carreira, P.E., Novak, S., Czirják, L., Varju, C., Chizzolini, C., Kucharz, E.J., Kotulska, A., Kopec-Medrek, M., Widuchowska, M., Rozman, B., Mallia, C., Coleiro, B., Gabrielli, A., Farge, D., Wu, C., Marjanovic, Z., Faivre, H., Hij, D., Dhamadi, R., Airò, P., Hesselstrand, R., Wollheim, F., Wuttge, D.M., Andréasson, K., Martinovic, D., Balbir-Gurman, A., Braun-Moscovici, Y., Trotta, F., Lo Monaco, A., Hunzelmann, N., Pellerito, R., Mauriziano, O., Maria Bambara, L., Caramaschi, P., Morovic-Vergles, J., Black, C., Damjanov, N., Henes, J., Ortiz Santamaria, V., Heitmann, S., Krasowska, D., Seidel, M., Hasler, P., Burkhardt, H., Himsel, A., Bajocchi, G., Maria Nuova, A.S., João Salvador, M., Pereira Da Silva, J.A., Stamenkovic, B., Stankovic, A., Francesco Selmi, C., De Santis, M., Marasini, B., Tikly, M., Ananieva, L.P., Denisov, L.N., Müller-Ladner, U., Frerix, M., Tarner, I., Scorza, R., Puppo, F., Engelhart, M., Strauss, G., Nielsen, H., Damgaard, K., Szücs, G., Szamosi, S., Zea Mendoza, A., de la Puente, C., Sifuentes Giraldo, W.A., Midtvedt, Ø., Reiseter, S., Garen, T., Valesini, G., Riccieri, V., Maria Ionescu, R., Opris, D., Groseanu, L., Wigley, F.M., Sfrent Cornateanu, R., Ionitescu, R., Maria Gherghe, A., Soare, A., Gorga, M., Bojinca, M., Mihai, C., Milicescu, M., Sunderkötter, C., Kuhn, A., Sandorfi, N., Schett, G., Distler, J.H., Beyer, C., Meroni, P., Ingegnoli, F., Mouthon, L., De Keyser, F., Smith, V., Paolo Cantatore, F., Corrado, A., Ullman, S., Iversen, L., Alberto von Mühlen, C., Marilu Bohn, J., Scussel Lonzetti, L., Rosa Pozzi, M., Eyerich, K., Hein, R., Knott, E., Wiland, P., Szmyrka-Kaczmarek, M., Sokolik, R., Morgiel, E., Madej, M., Houssiau, F.A., Jose Alegre-Sancho, J., Krummel-Lorenz, B., Saar, P., Aringer, M., Günther, C., Westhovens, R., de Langhe, E., Lenaerts, J., Anic, B., Baresic, M., Mayer, M., Üprus, M., Otsa, K., Yavuz, S., Granel, B., Cezar Radominski, S., de Souza Müller, C., Azevedo, V.F., Jimenez, S., Busquets, J., Agachi, S., Groppa, L., Chiaburu, L., Russu, E., Popa, S., Zenone, T., Pileckyte, M., Stebbings, S., Highton, J., Mathieu, A., Vacca, A., Sampaio-Barros, P.D., Yoshinari, N.H., Marangoni, R.G., Martin, P., Fuocco, L., Stamp, L., Chapman, P., O'Donnell, J., Solanki, K., Doube, A., Veale, D., O'Rourke, M., Loyo, E., Li, M., Abdel Atty Mohamed, W.A., Rosato, E., Amoroso, A., Gigante, A., Oksel, F., Yargucu, F., Tanaseanu, C.M., Popescu, M., Dumitrascu, A., Tiglea, I., Foti, R., Chirieac, R., Ancuta, C., Furst, D.E., Villiger, P., Adler, S., van Laar, J., Kayser, C., Eduardo C, A.L., Fathi, N., Hassanien, M., de la Peña Lefebvre, P.G., Rodriguez Rubio, S., Valero Exposito, M., Sibilia, J., Chatelus, E., Gottenberg, J.E., Chifflot, H., Litinsky, I., Emery, P., Buch, M., Del Galdo, F., Venalis, A., Butrimiene, I., Venalis, P., Rugiene, R., Karpec, D., Ann Saketkoo, L., Lasky, J.A., Kerzberg, E., Montoya, F., Cosentino, V., Limonta, M., Luca Brucato, A., Lupi, E., Rosner, I., Rozenbaum, M., Slobodin, G., Boulman, N., Rimar, D., Couto, M., Spertini, F., Ribi, C., Buss, G., Kahl, S., Hsu, V.M., Chen, F., McCloskey, D., Malveaux, H., Louis Pasquali, J., Martin, T., Gorse, A., Guffroy, A., Poindron, V., and Chizzolini, Carlo

Subjects
0301 basic medicine, Male, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences, Databases, Factual, systemic sclerosis, SUBSETS, Disease, Severity of Illness Index, Scleroderma, DISEASE, 0302 clinical medicine, Medicine and Health Sciences, Immunology and Allergy, Cluster Analysis, CRITERIA, Prospective Studies, Prospective cohort study, skin and connective tissue diseases, integumentary system, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti, Adult, Aged, Autoantibodies/blood, Europe/epidemiology, Female, Humans, Middle Aged, Phenotype, Prognosis, Scleroderma, Diffuse/blood, Scleroderma, Diffuse/epidemiology, Scleroderma, Diffuse/pathology, Scleroderma, Limited/blood, Scleroderma, Limited/epidemiology, Scleroderma, Limited/pathology, Scleroderma, Systemic/blood, Scleroderma, Systemic/epidemiology, Scleroderma, Systemic/pathology, Connective tissue disease, ddc, Europe, MANIFESTATIONS, Cohort, Life Sciences & Biomedicine, medicine.medical_specialty, Immunology, PROFILE, CLASSIFICATION, 03 medical and health sciences, Rheumatology, Scleroderma, Limited, Internal medicine, Severity of illness, medicine, Autoantibodies, 030203 arthritis & rheumatology, Science & Technology, Scleroderma, Systemic, business.industry, Autoantibody, Systemic sclerosis (SSc), medicine.disease, 030104 developmental biology, Scleroderma, Diffuse, business
Abstract

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. CONCLUSION: 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. ispartof: ARTHRITIS & RHEUMATOLOGY vol:71 issue:9 pages:1553-1570 ispartof: location:United States status: published

Published
2019

45. Classification, categorization and essential items for digital ulcer evaluation in systemic sclerosis: a DeSScipher/European Scleroderma Trials and Research group (EUSTAR) survey

Author

Blagojevic, Jelena, Bellando-Randone, Silvia, Abignano, Giuseppina, Avouac, Jérôme; https://orcid.org/0000-0002-2463-218X, Cometi, L, Czirják, László, Denton, Christopher P; https://orcid.org/0000-0003-3975-8938, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Frerix, Marc, Guiducci, Serena, Huscher, Dörte, Jaeger, Veronika K, Lóránd, Veronika, Maurer, Britta; https://orcid.org/0000-0001-9385-8097, Nihtyanova, Svetlana, Riemekasten, Gabriela, Siegert, Elise; https://orcid.org/0000-0002-9594-0446, Tarner, Ingo H, Vettori, Serena, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, Allanore, Yannick; https://orcid.org/0000-0002-6149-0002, Müller-Ladner, Ulf, Del Galdo, Francesco; https://orcid.org/0000-0002-8528-2283, Matucci-Cerinic, Marco; https://orcid.org/0000-0002-9324-3161, EUSTAR co-workers, Blagojevic, Jelena, Bellando-Randone, Silvia, Abignano, Giuseppina, Avouac, Jérôme; https://orcid.org/0000-0002-2463-218X, Cometi, L, Czirják, László, Denton, Christopher P; https://orcid.org/0000-0003-3975-8938, Distler, Oliver; https://orcid.org/0000-0002-0546-8310, Frerix, Marc, Guiducci, Serena, Huscher, Dörte, Jaeger, Veronika K, Lóránd, Veronika, Maurer, Britta; https://orcid.org/0000-0001-9385-8097, Nihtyanova, Svetlana, Riemekasten, Gabriela, Siegert, Elise; https://orcid.org/0000-0002-9594-0446, Tarner, Ingo H, Vettori, Serena, Walker, Ulrich A; https://orcid.org/0000-0002-9183-550X, Allanore, Yannick; https://orcid.org/0000-0002-6149-0002, Müller-Ladner, Ulf, Del Galdo, Francesco; https://orcid.org/0000-0002-8528-2283, Matucci-Cerinic, Marco; https://orcid.org/0000-0002-9324-3161, and EUSTAR co-workers

Abstract

BACKGROUND A consensus on digital ulcer (DU) definition in systemic sclerosis (SSc) has been recently reached (Suliman et al., J Scleroderma Relat Disord 2:115-20, 2017), while for their evaluation, classification and categorisation, it is still missing. The aims of this study were to identify a set of essential items for digital ulcer (DU) evaluation, to assess if the existing DU classification was useful and feasible in clinical practice and to investigate if the new categorisation was preferred to the simple distinction of DU in recurrent and not recurrent, in patients with systemic sclerosis (SSc). METHODS DeSScipher is the largest European multicentre study on SSc. It consists of five observational trials (OTs), and one of them, OT1, is focused on DU management. The DeSScipher OT1 items on DU that reached ≥ 60% of completion rate were administered to EUSTAR (European Scleroderma Trials and Research group) centres via online survey. Questions about feasibility and usefulness of the existing DU classification (DU due to digital pitting scars, to loss of tissue, derived from calcinosis and gangrene) and newly proposed categorisation (episodic, recurrent and chronic) were also asked. RESULTS A total of 84/148 (56.8%) EUSTAR centres completed the questionnaire. DeSScipher items scored by ≥ 70% of the participants as essential and feasible for DU evaluation were the number of DU defined as a loss of tissue (level of agreement 92%), recurrent DU (84%) and number of new DU (74%). For 65% of the centres, the proposed classification of DU was considered useful and feasible in clinical practice. Moreover, 80% of the centres preferred the categorisation of DU in episodic, recurrent and chronic to simple distinction in recurrent/not recurrent DU. CONCLUSIONS For clinical practice, EUSTAR centres identified only three essential items for DU evaluation and considered the proposed classification and categorisation as useful and feasible. The set of items needs to be validated

Published
2019

46. Hemodynamic changes after acute fluid loading in patients with systemic sclerosis without pulmonary hypertension

Author

D’Alto, Michele, Romeo, E., Argiento, P., Mattera Iacono, A., Vettori, Serena, Riccardi, Antonella, Allanore, Yannick, D’andrea, Antonello, Rea, Gaetano, Bossone, E., Valentini, Gabriele, Naeije, Robert, Golino, P., D’Alto, Michele, Romeo, E., Argiento, P., Mattera Iacono, A., Vettori, Serena, Riccardi, Antonella, Allanore, Yannick, D’andrea, Antonello, Rea, Gaetano, Bossone, E., Valentini, Gabriele, Naeije, Robert, and Golino, P.

Abstract

A fluid challenge with a rapid infusion of saline helps to discriminate between pre- and post-capillary pulmonary hypertension (PH) and allows unmasking hidden post-capillary PH. Systemic sclerosis (SSc) patients may present with biventricular systolic and diastolic dysfunction. The aim of this study was to evaluate the hemodynamic changes of the pulmonary circulation in SSc patients without PH after a fluid challenge. Twenty-five SSc patients and 25 controls underwent right heart catheterization in basal conditions and after volume loading with saline infusion of 7 mL/kg over 5–10 min. At baseline, there was no difference in hemodynamics between SSc patients and controls. Rapid volume loading resulted in a significant increase in pressures and flows in both groups. Increases in right atrial pressure (3 ± 1 vs. 2 ± 1 mmHg, P = 0.03), mean pulmonary artery pressure (5 ± 1 vs. 3 ± 1 mmHg, P < 0.001), and pulmonary artery wedge pressure (PAWP; 5 ± 2 vs. 3 ± 1 mmHg, P < 0.001) were larger in SSc patients than in controls. Conversely, cardiac index (0.4 ± 0.2 vs. 0.6 ± 0.3 L/min/m 2 ,P = 0.005) increased less in SSc patients than in controls. Pulmonary vascular resistance did not differ between groups before and after volume loading. Four SSc patients and only one of the controls reached a PAWP > 18 mmHg suggesting latent left heart failure. Even if differences are small and not diagnostic for heart failure, SSc patients without PH have a larger increase in pulmonary vascular pressures and a smaller increase in cardiac output than controls after an acute volume loading, probably due to subclinical left ventricular diastolic dysfunction., SCOPUS: ar.j, info:eu-repo/semantics/published

Published
2019

48. Classification, categorization and essential items for digital ulcer evaluation in systemic sclerosis: a DeSScipher/European Scleroderma Trials and Research group (EUSTAR) survey

Author

Blagojevic, Jelena, Bellando-Randone, Silvia, Abignano, Giuseppina, Avouac, Jérôme, Cometi, L, Czirják, László, Denton, Christopher P, Distler, Oliver, Frerix, Marc, Guiducci, Serena, Huscher, Dörte, Jaeger, Veronika K, Lóránd, Veronika, Maurer, Britta, Nihtyanova, Svetlana, Riemekasten, Gabriela, Siegert, Elise, Tarner, Ingo H, Vettori, Serena, Walker, Ulrich A, Allanore, Yannick, Müller-Ladner, Ulf, Del Galdo, Francesco, Matucci-Cerinic, Marco, EUSTAR co-workers, University of Zurich, Blagojevic, Jelena, Blagojevic, J., Bellando-Randone, S., Abignano, G., Avouac, J., Cometi, L., Czirják, L., Denton, C. P., Distler, O., Frerix, M., Guiducci, S., Huscher, D., Jaeger, V. K., Lóránd, V., Maurer, B., Nihtyanova, S., Riemekasten, G., Siegert, E., Tarner, I. H., Vettori, S., Walker, U. A., Allanore, Y., Müller-Ladner, U., Del Galdo, F., and Matucci-Cerinic, M.

Subjects
0301 basic medicine, Male, lcsh:Diseases of the musculoskeletal system, Observational Trial, 2745 Rheumatology, Digital ulcer, Categorisation, Scleroderma, Systemic sclerosi, 0302 clinical medicine, Surveys and Questionnaires, Immunology and Allergy, Prospective Studies, 10051 Rheumatology Clinic and Institute of Physical Medicine, Digital ulcers, Middle Aged, Calcium Channel Blockers, Classification, 3. Good health, Clinical Practice, Categorization, 2723 Immunology and Allergy, Systemic sclerosis, Drug Therapy, Combination, Female, Research Article, Adult, medicine.medical_specialty, Immunology, 610 Medicine & health, Sildenafil Citrate, Fingers, 03 medical and health sciences, Rheumatology, Skin Ulcer, medicine, Humans, In patient, European Union, Iloprost, 030203 arthritis & rheumatology, 2403 Immunology, Scleroderma, Systemic, business.industry, Bosentan, Essential item, medicine.disease, 030104 developmental biology, Essential items, Physical therapy, Observational study, lcsh:RC925-935, business
Abstract

Background: A consensus on digital ulcer (DU) definition in systemic sclerosis (SSc) has been recently reached (Suliman et al., J Scleroderma Relat Disord 2:115-20, 2017), while for their evaluation, classification and categorisation, it is still missing. The aims of this study were to identify a set of essential items for digital ulcer (DU) evaluation, to assess if the existing DU classification was useful and feasible in clinical practice and to investigate if the new categorisation was preferred to the simple distinction of DU in recurrent and not recurrent, in patients with systemic sclerosis (SSc).Methods: DeSScipher is the largest European multicentre study on SSc. It consists of five observational trials (OTs), and one of them, OT1, is focused on DU management. The DeSScipher OT1 items on DU that reached ≥ 60% of completion rate were administered to EUSTAR (European Scleroderma Trials and Research group) centres via online survey. Questions about feasibility and usefulness of the existing DU classification (DU due to digital pitting scars, to loss of tissue, derived from calcinosis and gangrene) and newly proposed categorisation (episodic, recurrent and chronic) were also asked.Results: A total of 84/148 (56.8%) EUSTAR centres completed the questionnaire. DeSScipher items scored by ≥ 70% of the participants as essential and feasible for DU evaluation were the number of DU defined as a loss of tissue (level of agreement 92%), recurrent DU (84%) and number of new DU (74%). For 65% of the centres, the proposed classification of DU was considered useful and feasible in clinical practice. Moreover, 80% of the centres preferred the categorisation of DU in episodic, recurrent and chronic to simple distinction in recurrent/not recurrent DU.Conclusions: For clinical practice, EUSTAR centres identified only three essential items for DU evaluation and considered the proposed classification and categorisation as useful and feasible. The set of items needs to be validated while further implementation of DU classification and categorisation is warranted.Trial registration: Observational trial on DU (OT1) is one of the five trials of the DeSScipher project (ClinicalTrials.gov; OT1 Identifier: NCT01836263, posted on April 19, 2013).

Published
2018

49. Functional disability and its predictors in systemic sclerosis: a study from the DeSScipher project within the EUSTAR group

Author

Jaeger, Veronika K, Distler, Oliver, Maurer, Britta, Czirják, László, Lóránd, Veronika, Valentini, Gabriele, Vettori, Serena, Del Galdo, Francesco, Abignano, Giuseppina, Denton, Christopher P, Nihtyanova, Svetlana, Allanore, Yannick, Avouac, Jérôme, Riemekasten, Gabriele, Siegert, Elise, Huscher, Dörte, Matucci-Cerinic, Marco, Guiducci, Serena, Frerix, Marc, Tarner, Ingo H, Garay Toth, Beata, Fankhauser, Beat, Umbricht, Jörg, Zakharova, Anastasia, Mihai, Carina, Cozzi, Franco, Yavuz, Sule, Hunzelmann, Nicolas, Rednic, Simona, Vacca, Alessandra, et al, and University of Zurich

Subjects
2745 Rheumatology, 10051 Rheumatology Clinic and Institute of Physical Medicine, 2736 Pharmacology (medical), 610 Medicine & health
Published
2018
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