Racial differences in systemic sclerosis disease presentation: a European Scleroderma Trials and Research group study

Objectives. 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].Conclusion. 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

Epidémiologie de la sclérodermie systémique (présentation du projet d'étude de la prévalence de la sclérodermie systémique en Alsace (EPSA))

STRASBOURG-Medecine (674822101) / SudocPARIS-BIUM (751062103) / SudocSudocFranceF

A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: A EUSTAR prospective study

Objectives. In agreement with other autoimmune diseases, systemic sclerosis (SSc) is associated with a strong sex bias. However, unlike lupus, the effects of sex on disease phenotype and prognosis are poorly known. Therefore, we aimed to determine sex effects on outcomes. Method. We performed a prospective observational study using the latest 2013 data extract from the EULAR scleroderma trials and research (EUSTAR) cohort. We looked at (i) sex influence on disease characteristics at baseline and (ii) then focused on patients with at least 2 years of follow-up to estimate the effects of sex on disease progression and survival. Results. 9182 patients with SSc were available (1321 men) for the baseline analyses. In multivariate analysis, male sex was independently associated with a higher risk of diffuse cutaneous subtype (OR: 1.68, (1.45 to 1.94); p<0.001), a higher frequency of digital ulcers (OR: 1.28 (1.11 to 1.47); p<0.001) and pulmonary hypertension (OR: 3.01 (1.47 to 6.20); p<0.003). In the longitudinal analysis (n=4499), after a mean follow-up of 4.9 (±2.7) years, male sex was predictive of new onset of pulmonary hypertension (HR: 2.66 (1.32 to 5.36); p=0.006) and heart failure (HR: 2.22 (1.06 to 4.63); p=0.035). 908 deaths were recorded, male sex predicted deaths of all origins (HR: 1.48 (1.19 to 1.84); p<0.001), but did not significantly account for SSc-related deaths. Conclusions. Although more common in women, SSc appears as strikingly more severe in men. Our results obtained through the largest worldwide database demonstrate a higher risk of severe cardiovascular involvement in men. These results raise the point of including sex in the management and the decisionmaking process

Prediction of improvement in skin fibrosis in diffuse cutaneous systemic sclerosis: A EUSTAR analysis

none194Dobrota, 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.; Monaco, Andrea Lo; Hunzelmann, Nicolas; Pellerito, Raffaele; Mauriziano, Ospedale; Bambara, Lisa Maria; Caramaschi, Paola; Black, Carol; Denton, Christopher; Damjanov, Nemanja; Henes, Jörg; Santamaria, Vera Ortiz; Heitmann, Stefan; Krasowska, Dorota; Seidel, Matthias; Burkhardt, Harald; Himsel, Andrea; Salvador, Maria J.; Da Silva, José Antonio Pereira; Stamenkovic, Bojana; Stankovic, Aleksandra; Tikly, Mohammed; Ananieva, Lidia P.; Denisov, Lev N.; Müller-Ladner, Ulf; Frerix, Marc; Tarner, Ingo; Scorza, Raffaella; 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 H.W.; Beyer, Christian; Meroni, Pierluigi; Ingegnoli, Francesca; Mouthon, Luc; Keyser, Filip De; 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; Saar, Petra; 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; Stebbings, Simon; Highton, John; Stamp, Lisa; Chapman, Peter; O'Donnell, John; Solanki, Kamal; Doube, Alan; Veale, Douglas; O'Rourke, Marie; Loyo, Esthela; Li, Mengtao; Rosato, Edoardo; Amoroso, Antonio; Gigante, Antonietta; Tanaseanu, Cristina-Mihaela; Popescu, Monica; Dumitrascu, Alina; Tiglea, Isabela; Foti, Rosario; Chirieac, Rodica; Ancuta, Codrina; Villiger, Peter; Adler, Sabine; de la Peña Lefebvre, Paloma García; 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; 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; Gorse, AudreyDobrota, 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, Carlomaurizio; 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.; Monaco, Andrea Lo; Hunzelmann, Nicolas; Pellerito, Raffaele; Mauriziano, Ospedale; Bambara, Lisa Maria; Caramaschi, Paola; Black, Carol; Denton, Christopher; Damjanov, Nemanja; Henes, Jörg; Santamaria, Vera Ortiz; Heitmann, Stefan; Krasowska, Dorota; Seidel, Matthias; Burkhardt, Harald; Himsel, Andrea; Salvador, Maria J.; Da Silva, José Antonio Pereira; Stamenkovic, Bojana; Stankovic, Aleksandra; Tikly, Mohammed; Ananieva, Lidia P.; Denisov, Lev N.; Müller Ladner, Ulf; Frerix, Marc; Tarner, Ingo; Scorza, Raffaella; 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 H. W.; Beyer, Christian; Meroni, Pierluigi; Ingegnoli, Francesca; Mouthon, Luc; Keyser, Filip De; 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; Saar, Petra; 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; Stebbings, Simon; Highton, John; Stamp, Lisa; Chapman, Peter; O'Donnell, John; Solanki, Kamal; Doube, Alan; Veale, Douglas; O'Rourke, Marie; Loyo, Esthela; Li, Mengtao; Rosato, Edoardo; Amoroso, Antonio; Gigante, Antonietta; Tanaseanu, Cristina Mihaela; Popescu, Monica; Dumitrascu, Alina; Tiglea, Isabela; Foti, Rosario; Chirieac, Rodica; Ancuta, Codrina; Villiger, Peter; Adler, Sabine; de la Peña Lefebvre, Paloma García; 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; 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; Gorse, Audre

Update on the profile of the EUSTAR cohort: an analysis of the EULAR Scleroderma Trials and Research group database

Objectives Systemic sclerosis (SSc) is a rare disease requiring multicentre collaboration to reveal comprehensive details of disease-related causes for morbidity and mortality.Methods The European League Against Rheumatism (EULAR) Scleroderma Trials and Research (EUSTAR) group initiated a database to prospectively gather key data of patients with SSc using a minimal essential dataset that was reorganised in 2008 introducing new items. Baseline visit data of patients who were registered between 2004 and 2011 were analysed using descriptive statistics.Results In June 2011, 7655 patients (2838 with diffuse cutaneous (dc) and 4481 with limited cutaneous (lc) SSc who fulfilled the American College of Rheumatology diagnostic criteria had been registered in 174 centres, mainly European. The most prominent hallmarks of disease were Raynaud's phenomenon (96.3%), antinuclear antibodies (93.4%) and a typical capillaroscopic pattern (90.9%). Scleroderma was more common on fingers and hands than on any other part of the skin. Proton pump inhibitors (65.2%), calcium channel blockers (52.7%), and corticosteroids (45.3%) were most often prescribed. Among the immunosuppressant agents, cyclophosphamide was used more often in dcSSc than in lcSSc.Conclusions The EUSTAR database provides an abundance of information on the true clinical face of SSc that will be helpful in improving the classification of SSc and its subsets and for developing more specific therapeutic recommendations