Search

Your search keyword '"Esthela Loyo"' showing total 21 results
Start Over Author "Esthela Loyo"
21 results on '"Esthela Loyo"'

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

Author

Pavel I Novikov, Thierry Martin, Eric Hachulla, Vanessa Smith, Maurizio Cutolo, Yannick Allanore, Camillo Ribi, Oliver Distler, Britta Maurer, Paolo Airo, Cosimo Bruni, Veronica Codullo, Luc Mouthon, Marie Vanthuyne, Elisabetta Zanatta, Simona Rednic, Ulf Müller-Ladner, Nemanja Damjanov, Anna-Maria Hoffmann-Vold, Michele Iudici, Suzana Jordan, Jean Sibilia, Mengtao Li, Roger Hesselstrand, Elise Siegert, László Czirják, Armando Gabrielli, Dominique Farge Bancel, Yolanda Braun-Moscovici, Nicolas Hunzelmann, Raffaele Pellerito, Jörg Henes, Bojana Stamenkovic, Carlo Francesco Selmi, Mohammed Tikly, Merete Engelhart, Valeria Riccieri, Cord Sunderkötter, Francesca Ingegnoli, Francesco Paolo Cantatore, Susanne Ullman, Maria Rosa Pozzi, Piotr Wiland, Juan Jose Alegre-Sancho, Brigitte Krummel-Lorenz, Ellen De Langhe, Branimir Anic, Carolina de Souza Müller, Simon Stebbings, Alessandra Vacca, Lisa Stamp, Kamal Solanki, Douglas Veale, Esthela Loyo, Edoardo Rosato, Cristina-Mihaela Tanaseanu, Rosario Foti, Codrina Ancuta, Ira Litinsky, Lesley Ann Saketkoo, Eduardo Kerzberg, Tim Schmeiser, Piercarlo Sarzi Puttini, Fabio Cacciapaglia, Marie-Elise Truchetet, Muriel Elhai, Juan José Alegre Sancho, Ivan Castellví, Serena Vettori, Vivien M Hsu, Masataka Kuwana, Sule Yavuz, Radim Becvar, Patricia E Carreira, Eugene J Kucharz, Bernard Coleiro, Paul Hasler, Carlos de la Puente, Svetlana Agachi, Thierry Zenone, Francesco Del Galdo, Doron Rimar, Alessandro Giollo, Lidia P Ananieva, Ina Kötter, Mislav Radic, Dominik Majewski, Kristine Herrmann, Goda Seskute, Lorinda S Chung, Laura Belloli, Ulrike Held, FJ López-Longo, Joerg H W Distler, Maria João Salvador, Simon Kuster, Klaus Steigmiller, Ana-Maria Gheorghiu, Katarzyna Romanowska-Prochnicka, Yuichiro Shirai, Chris Denton, Ruxandra M Ionescu, Stefan Heitmann Vera Ortiz-Santamaria, Walid Ahmed Abdel Atty Mohamed, Paloma Garcíadela Peña Lefebvre, and Vera Bernardino

Subjects
Medicine
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
Full Text
View/download PDF

2. Use of platelet inhibitors for digital ulcers related to systemic sclerosis: EUSTAR study on derivation and validation of the DU-VASC model

Author

Alexandru Garaiman 1, Klaus Steigmiller 2, Catherine Gebhard 3, Carina Mihai 1, Rucsandra Dobrota 1, Cosimo Bruni 4, Marco Matucci-Cerinic 5, Joerg Henes 6, Jeska de Vries-Bouwstra 7, Vanessa Smith 8, Andrea Doria 9, Yannick Allanore 10, Lorenzo Dagna 11, Branimir Anić 12, Carlomaurizio Montecucco 13, Otylia Kowal-Bielecka 14, Mickael Martin 15, Yoshiya Tanaka 16, Anna-Maria Hoffmann-Vold 17, Ulrike Held 2, Oliver Distler 1, Mike Oliver Becker 1, EUSTAR Silvia Bellando Randone, Gemma Lepri, Ulrich Walker, Florenzo Iannone, Suzana Jordan, Radim Becvar, Ewa Gindzienska-Sieskiewicz, Katarzyna Karaszewska, Maurizio Cutolo, Giovanna Cuomo, Elise Siegert, Simona Rednic, Jérome Avouac, Carole Desbas, Roberto Caporali, Lorenzo Cavagna, Patricia E Carreira, Srdan Novak, László Czirják, Michele Iudici, Eugene J Kucharz, Elisabetta Zanatta, Bernard Coleiro, Gianluca Moroncini, Dominique Farge Bancel, Paolo Airò, Roger Hesselstr, Mislav Radic, Alexandra Balbir-Gurman, Nicolas Hunzelmann, Raffaele Pellerito, Alessandro Giollo, Jadranka Morovic-Vergles, Christopher Denton, Nemanja Damjanov, Ann-Christian Pecher, Vera Ortiz Santamaria, Stefan Heitmann, Dorota Krasowska, Paul Hasler, Ivan Foeldvari, Maria João Salvador, Bojana Stamenkovic, Carlo Francesco Selmi, Lidia P Ananieva, Ariane Herrick, Ulf Müller-Ladner, Raffaele De Palma, Merete Engelhart, Gabriela Szücs, Carlos de la Puente, Øyvind Midtvedt, Torhild Garen, Håvard Fretheim, Eric Hachulla, Valeria Riccieri, Ruxandra Maria Ionescu, Ana Maria Gheorghiu, Cord Sunderkötter, Jörg Distler, Francesca Ingegnoli, Luc Mouthon, Francesco Paolo Cantatore, Susanne Ullman, Maria Rosa Pozzi, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Juan Jose Alegre-Sancho, Kristine Herrmann, Ellen De Langhe, Marko Baresic, Miroslav Mayer, Sule Yavuz, Brigitte Granel, Carolina de Souza Müller, Svetlana Agachi, Simon Stebbings, D'Alessandro Mathieu, Alessandra Vacca, Kamal Solanki, Douglas Veale, Esthela Loyo, Carmen Tineo, Mengtao Li, Edoardo Rosato, Fahrettin Oksel, Figen Yargucu, Cristina-Mihaela Tanaseanu, Rosario Foti, Codrina Ancuta, Britta Maurer, Jacob van Laar, Marzena Olesinska, Cristiane Kayser, Nihal Fathi, Paloma García de la Peña Lefebvre, Jorge Juan Gonzalez Martin, Jean Sibilia, Ira Litinsky, Francesco Del Galdo, Lesley Ann Saketkoo, Eduardo Kerzberg, Washington Bianch, Breno Valdetaro Bianchi, Ivan Castellví, Massimiliano Limonta, Doron Rimar, Maura Couto, François Spertini, Antonella Marcoccia, Sarah Kahl, Ivien M Hsu, Thierry Martin, Sergey Moiseev, Pavel Novikov, Lorinda S Chung, Tim Schmeiser, Dominik Majewski, Zbigniew Zdrojewski, Julia Martínez-Barrio, Vera Bernardino, Gabriela Riemekasten, Yair Levy, Elena Rezus, Omer Nuri Pamuk, Piercarlo Sarzi Puttini, Hadi Poormoghim, Ina Kötter, Francis Gaches, Laura Belloli, Petros Sfikakis, Daniel Furst, Ana-Maria Ramazan, H U Scherer, Tom W J Huizinga, Marie-Elise Truchetet, Alain Lescoat, Giacomo De Luca, Corrado Campochiaro, J M van Laar, Lidia Rudnicka, Susana Oliveira, Fabiola Atzeni, Masataka Kuwana, Arsene Mekinian, Cédric L, Mathieu Puyade, Pascal Roblot, Satoshi Kubo, Yasuyuki Todoroki, 1, Alexandru Garaiman, 2, Klaus Steigmiller, 3, Catherine Gebhard, 1, Carina Mihai, 1, Rucsandra Dobrota, 4, Cosimo Bruni, 5, Marco Matucci-Cerinic, 6, Joerg Hene, 7, Jeska de Vries-Bouwstra, 8, Vanessa Smith, 9, Andrea Doria, Allanore 10, Yannick, Dagna 11, Lorenzo, Anić 12, Branimir, Montecucco 13, Carlomaurizio, Kowal-Bielecka 14, Otylia, Martin 15, Mickael, Tanaka 16, Yoshiya, Hoffmann-Vold 17, Anna-Maria, 2, Ulrike Held, 1, Oliver Distler, 1, Mike Oliver Becker, Silvia Bellando Randone, Eustar, Lepri, Gemma, Walker, Ulrich, Iannone, Florenzo, Jordan, Suzana, Becvar, Radim, Gindzienska-Sieskiewicz, Ewa, Karaszewska, Katarzyna, Cutolo, Maurizio, Cuomo, Giovanna, Siegert, Elise, Rednic, Simona, Avouac, Jérome, Desbas, Carole, Caporali, Roberto, Cavagna, Lorenzo, E Carreira, Patricia, Novak, Srdan, Czirják, László, Iudici, Michele, J Kucharz, Eugene, Zanatta, Elisabetta, Coleiro, Bernard, Moroncini, Gianluca, Farge Bancel, Dominique, Airò, Paolo, Hesselstr, Roger, Radic, Mislav, Balbir-Gurman, Alexandra, Hunzelmann, Nicola, Pellerito, Raffaele, Giollo, Alessandro, Morovic-Vergles, Jadranka, Denton, Christopher, Damjanov, Nemanja, Pecher, Ann-Christian, Ortiz Santamaria, Vera, Heitmann, Stefan, Krasowska, Dorota, Hasler, Paul, Foeldvari, Ivan, João Salvador, Maria, Stamenkovic, Bojana, Francesco Selmi, Carlo, P Ananieva, Lidia, Herrick, Ariane, Müller-Ladner, Ulf, DE PALMA, Raffaele, Engelhart, Merete, Szücs, Gabriela, de la Puente, Carlo, Midtvedt, Øyvind, Garen, Torhild, Fretheim, Håvard, Hachulla, Eric, Riccieri, Valeria, Maria Ionescu, Ruxandra, Maria Gheorghiu, Ana, Sunderkötter, Cord, Distler, Jörg, Ingegnoli, Francesca, Mouthon, Luc, Paolo Cantatore, Francesco, Ullman, Susanne, Rosa Pozzi, Maria, Eyerich, Kilian, Wiland, Piotr, Vanthuyne, Marie, Jose Alegre-Sancho, Juan, Herrmann, Kristine, De Langhe, Ellen, Baresic, Marko, Mayer, Miroslav, Yavuz, Sule, Granel, Brigitte, de Souza Müller, Carolina, Agachi, Svetlana, Stebbings, Simon, Mathieu, D'Alessandro, Vacca, Alessandra, Solanki, Kamal, Veale, Dougla, Loyo, Esthela, Tineo, Carmen, Li, Mengtao, Rosato, Edoardo, Oksel, Fahrettin, Yargucu, Figen, Tanaseanu, Cristina-Mihaela, Foti, Rosario, Ancuta, Codrina, Maurer, Britta, van Laar, Jacob, Olesinska, Marzena, Kayser, Cristiane, Fathi, Nihal, García de la Peña Lefebvre, Paloma, Juan Gonzalez Martin, Jorge, Sibilia, Jean, Litinsky, Ira, Del Galdo, Francesco, Ann Saketkoo, Lesley, Kerzberg, Eduardo, Bianch, Washington, Valdetaro Bianchi, Breno, Castellví, Ivan, Limonta, Massimiliano, Rimar, Doron, Couto, Maura, Spertini, Françoi, Marcoccia, Antonella, Kahl, Sarah, M Hsu, Ivien, Martin, Thierry, Moiseev, Sergey, Novikov, Pavel, S Chung, Lorinda, Schmeiser, Tim, Majewski, Dominik, Zdrojewski, Zbigniew, Martínez-Barrio, Julia, Bernardino, Vera, Riemekasten, Gabriela, Levy, Yair, Rezus, Elena, Nuri Pamuk, Omer, Sarzi Puttini, Piercarlo, Poormoghim, Hadi, Kötter, Ina, Gaches, Franci, Belloli, Laura, Sfikakis, Petro, Furst, Daniel, Ramazan, Ana-Maria, U Scherer, H, J Huizinga, Tom W, Truchetet, Marie-Elise, Lescoat, Alain, De Luca, Giacomo, Campochiaro, Corrado, M van Laar, J, Rudnicka, Lidia, Oliveira, Susana, Atzeni, Fabiola, Kuwana, Masataka, Mekinian, Arsene, L, Cédric, Puyade, Mathieu, Roblot, Pascal, Kubo, Satoshi, Todoroki, Yasuyuki, and University of Zurich

Subjects
prognostic prediction model, Rheumatology, 10051 Rheumatology Clinic and Institute of Physical Medicine, digital ulcers, platelets inhibitors, 610 Medicine & health, Pharmacology (medical), SSc, 10060 Epidemiology, Biostatistics and Prevention Institute (EBPI)
Abstract

Objective To develop and validate the prognostic prediction model DU-VASC to assist the clinicians in decision-making regarding the use of platelet inhibitors (PIs) for the management of digital ulcers in patients with systemic sclerosis. Secondly, to assess the incremental value of PIs as predictor. Methods We analysed patient data from the European Scleroderma Trials and Research group registry (one time point assessed). Three sets of derivation/validation cohorts were obtained from the original cohort. Using logistic regression, we developed a model for prediction of digital ulcers (DUs). C-Statistics and calibration plots were calculated to evaluate the prediction performance. Variable importance plots and the decrease in C-statistics were used to address the importance of the predictors. Results Of 3710 patients in the original cohort, 487 had DUs and 90 were exposed to PIs. For the DU-VASC model, which includes 27 predictors, we observed good calibration and discrimination in all cohorts (C-statistic = 81.1% [95% CI: 78.9%, 83.4%] for the derivation and 82.3% [95% CI: 779.3%, 85.3%] for the independent temporal validation cohort). Exposure to PIs was associated with absence of DUs and was the most important therapeutic predictor. Further important factors associated with absence of DUs were lower modified Rodnan skin score, anti-Scl-70 negativity and normal CRP. Conversely, the exposure to phosphodiesterase-5 inhibitor, prostacyclin analogues or endothelin receptor antagonists seemed to be associated with the occurrence of DUs. Nonetheless, previous DUs remains the most impactful predictor of DUs. Conclusion The DU-VASC model, with good calibration and discrimination ability, revealed that PI treatment was the most important therapy-related predictor associated with reduced DU occurrence.

Published
2022

3. Phenotype of limited cutaneous systemic sclerosis patients with positive anti-topoisomerase I antibodies: data from the EUSTAR cohort

Author

Elisabetta Zanatta 1, Dörte Huscher 2, Augusta Ortolan 1, Jérôme Avouac 3, Paolo Airò 4, Alexandra Balbir-Gurman 5, Elise Siegert 6, Marco Matucci Cerinic 7, Franco Cozzi 8, Gabriela Riemekasten 9, Anna-Maria Hoffmann-Vold 10, Oliver Distler 11, Armando Gabrielli 12, Stefan Heitmann 13, Nicolas Hunzelmann 14, Carlomaurizio Montecucco 15, Jadranka Morovic-Vergles 16, Camillo Ribi 17, Andrea Doria 1, Yannick Allanore 3, EUSTAR collaborators, Giovanna Cuomo, Gianluca Moroncini, Jiri Stork, Fiorenzo Iannone, Ulrich Walker, Eugenia Bertoldo, Dorota Krasowska, Maria João Salvador, Mohammed Tikly, Eric Hachulla, Valeria Riccieri, Ami Sha, Ana Maria Gheorghiu, Cord Sunderkötter, Francesca Ingegnoli, Luc Mouthon, Vanessa Smith, Francesco Paolo Cantatore, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Branimir Anic, Maria Üprus, Brigitte Granel, Alessandra Vacca, Cristina-Mihaela Tanaseanu, Paloma García de la Peña Lefebvre, Jean Sibilia, Ira Litinsky, Lesley Ann Saketkoo, Eduardo Kerzberg, Massimiliano Limonta, Doron Rimar, Petros Sfikakis, Maurizio Cutolo, Patricia E Carreira, Rosario Foti, Srdan Novak, Michele Iudici, Mislav Radic, Raffaele Pellerito, Carlo Francesco Selmi, Lidia P Ananieva, Gabriela Szücs, Carlos de la Puente, Ruxandra Maria Ionescu, Jörg Distler, Maria Rosa Pozzi, Juan Jose Alegre-Sancho, Kristine Herrmann, Ellen De Langhe, Sule Yavuz, Carolina de Souza Müller, Svetlana Agachi, Douglas Veale, Esthela Loyo, Mengtao Li, Edoardo Rosato, Britta Maurer, Ivan Castellví, François Spertini, Kamal Solanki, Nicoletta Del Papa, Gerard Espinosa, László Czirják, Bernard Coleiro, Dominique Farge Bancel, Christopher Denton, Nemanja Damjanov, Jörg Henes, Vera Ortiz Santamaria, Michaela Kohm, Bojana Stamenkovic, 1, Elisabetta Zanatta, 2, Dörte Huscher, 1, Augusta Ortolan, 3, Jérôme Avouac, 4, Paolo Airò, 5, Alexandra Balbir-Gurman, 6, Elise Siegert, 7, Marco Matucci Cerinic, 8, Franco Cozzi, 9, Gabriela Riemekasten, Hoffmann-Vold 10, Anna-Maria, Distler 11, Oliver, Gabrielli 12, Armando, Heitmann 13, Stefan, Hunzelmann 14, Nicola, Montecucco 15, Carlomaurizio, Morovic-Vergles 16, Jadranka, Ribi 17, Camillo, 1, Andrea Doria, 3, Yannick Allanore, Collaborators, Eustar, Cuomo, Giovanna, Moroncini, Gianluca, Stork, Jiri, Iannone, Fiorenzo, Walker, Ulrich, Bertoldo, Eugenia, Krasowska, Dorota, João Salvador, Maria, Tikly, Mohammed, Hachulla, Eric, Riccieri, Valeria, Sha, Ami, Maria Gheorghiu, Ana, Sunderkötter, Cord, Ingegnoli, Francesca, Mouthon, Luc, Smith, Vanessa, Paolo Cantatore, Francesco, Eyerich, Kilian, Wiland, Piotr, Vanthuyne, Marie, Anic, Branimir, Üprus, Maria, Granel, Brigitte, Vacca, Alessandra, Tanaseanu, Cristina-Mihaela, García de la Peña Lefebvre, Paloma, Sibilia, Jean, Litinsky, Ira, Ann Saketkoo, Lesley, Kerzberg, Eduardo, Limonta, Massimiliano, Rimar, Doron, Sfikakis, Petro, Cutolo, Maurizio, E Carreira, Patricia, Foti, Rosario, Novak, Srdan, Iudici, Michele, Radic, Mislav, Pellerito, Raffaele, Francesco Selmi, Carlo, P Ananieva, Lidia, Szücs, Gabriela, de la Puente, Carlo, Maria Ionescu, Ruxandra, Distler, Jörg, Rosa Pozzi, Maria, Jose Alegre-Sancho, Juan, Herrmann, Kristine, De Langhe, Ellen, Yavuz, Sule, de Souza Müller, Carolina, Agachi, Svetlana, Veale, Dougla, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Maurer, Britta, Castellví, Ivan, Spertini, Françoi, Solanki, Kamal, Del Papa, Nicoletta, Espinosa, Gerard, Czirják, László, Coleiro, Bernard, Farge Bancel, Dominique, Denton, Christopher, Damjanov, Nemanja, Henes, Jörg, Ortiz Santamaria, Vera, Kohm, Michaela, and Stamenkovic, Bojana

Subjects
interstitial lung disease, Scleroderma, Systemic, Hypertension, Pulmonary, disease subset, anti-topoisomerase I, Scleroderma, Systemic sclerosis, cutaneous form, outcome, Phenotype, Rheumatology, Scleroderma, Limited, Antibodies, Antinuclear, Scleroderma, Diffuse, Humans, Pharmacology (medical), Lung Diseases, Interstitial
Abstract

Objectives To characterize patients with positive anti-topoisomerase I (ATA) in lcSSc. Methods SSc patients enrolled in the EUSTAR cohort with a disease duration of ≤3 years at database entry were considered. We assessed the risk of major organ involvement in the following groups: ATA-lcSSc vs ACA-lcSSc and vs ANA without specificity (ANA)-lcSSc, and ATA-lcSSc vs ATA-dcSSc. Cox regression models with time-dependent covariates were performed with the following outcomes: new-onset interstitial lung disease (ILD), ILD progression [forced vital capacity (FVC) decline ≥10% and ≥5% vs values at ILD diagnosis), primary myocardial involvement (PMI), pulmonary hypertension (PH), any organ involvement and all-cause mortality. Results We included 1252 patients [194 ATA-lcSSc (15.5%)], with 7.7 years (s.d. 3.5) of follow-up. ILD risk was higher in ATA-lcSSc vs ACA- and ANA-lcSSc and similar to ATA-dcSSc, although with less frequent restrictive lung disease. The risk of FVC decline ≥10% (35% of ATA-lcSSc) was lower in ATA-lcSSc than in ATA-dcSSc, whereas FVC decline ≥5% occurs similarly between ATA-lcSSc (58% of patients) and other SSc subsets, including ATA-dcSSc. The risk of PMI was similar in ATA-lcSSc and ANA-lcSSc but lower than in ACA-lcSSc; no difference in PH and mortality risk was observed among lcSSc subsets. The risk of any organ involvement, PMI and PH was lower and the mortality tended to be lower in ATA-lcSSc vs ATA-dcSSc. Conclusion ATA-lcSSc patients have a high risk of ILD, albeit with a lower risk of progression compared with ATA-dcSSc, supporting careful screening for ILD in this subgroup.

Published
2022

4. Myositis-Specific Antibodies and Myositis-Associated Antibodies in Patients With Idiopathic Inflammatory Myopathies From the PANLAR Myositis Study Group

Author

G Orozco-Barocio, Itzel Viridiana Reyes-Pérez, Alicia Cardenas-Anaya, Antonio Iglesias-Gamarra, Jose Eduardo Navarro-Zarza, Lilia Andrade-Ortega, Marco A. Maradiaga-Ceceña, Mónica Vázquez-Del Mercado, Marvin J. Fritzler, Graciela Gómez, Armando Rojo-Mejia, Paola Gottschalk, Esthela Loyo, Kelly Vega, Gabriel Medrano-Ramírez, Abraham García-Kutzbach, Ignacio Garcia-Valladares, Daniel García-Cerda, Cilia Rojas, Yelitza Concepción González-Bello, Rubén D. Mantilla, Ignacio García-De La Torre, and Arnulfo Hernán Nava-Zavala

Subjects
Adult, Male, medicine.medical_specialty, Gastroenterology, Polymyositis, Dermatomyositis, Rheumatology, Internal medicine, medicine, Humans, In patient, Myositis, Autoantibodies, Immunoassay, biology, business.industry, Autoantibody, medicine.disease, Cross-Sectional Studies, Idiopathic inflammatory myopathies, biology.protein, Female, Antibody, business, Rheumatism
Abstract

Background Dermatomyositis (DM) and polymyositis (PM) are forms of idiopathic inflammatory myopathies (IIMs), which are associated with the production of autoantibodies that are useful in the diagnosis and prognosis of the disease. Objective The aim of this study was to determine the frequency of antinuclear autoantibodies (ANAs), myositis-specific autoantibodies (MSAs), and myositis-associated autoantibodies (MAAs) in 6 Latin American countries. Methods Two hundred ten patients with IIM were included in this cross-sectional study from 2014 to 2017: 112 from Mexico, 46 from Colombia, 20 from Peru, 16 from the Dominican Republic, 10 from Argentina, and 6 from Guatemala. Antinuclear autoantibodies were detected by indirect immunofluorescence on HEp-2 cells. MSAs and MAAs were tested by a line immunoassay method. Mann-Whitney U and χ tests were used for statistical analysis. Results Of the 210 IIM patients, 139 (66.2%) had DM, 59 (28%) PM, and 12 (5.7%) juvenile DM. The mean age was 43.5 (6-79 years); 158 (75.2%) were female, and 52 (24.8%) were male. The overall frequency of ANA was 60%. The most frequent patterns were fine speckled (AC-4) (78.3%) and cytoplasmic (AC-19) (6.45%). The most frequent MSA were anti-Mi-2 (38.5%) and anti-Jo-1 (11.9%). Anti-Mi-2 was more frequent in patients from Colombia (40.1%). The MAA more frequent were anti-Ro-52/TRIM21 (17.6%) and anti-PM-Scl75 (7.5%). Conclusions This is the first study of ANA, MSA, and MAA in patients from 6 countries from the Panamerican League against Rheumatism myositis study group. We observed a general prevalence of 60% of ANA. In relation to MSA and MAA, anti-Mi-2 was the more frequent (38.5%).

Published
2021

5. Glucocorticoids prescribing practices in systemic sclerosis: an analysis of the EUSTAR database

Author

Iudici, Michele, Mongin, Denis, Siegert, Elise, Carreira, Patricia E, Distler, Jörg, Henes, Jörg, Zanatta, Elisabetta, Hachulla, Eric, De Luca, Giacomo, Müller, Carolina de Souza, Santiago, Tânia, Tandaipan, José-Luis, Bianchi, Breno Valdetaro, De Santis, Maria, Hoffmann-Vold, Anna-Maria, Gabrielli, Armando, Distler, Oliver, Courvoisier, Delphine Sophie, Giovanna Cuomo, Gianluca Moroncini, Jiri Stork, Fiorenzo Iannone, Ulrich Walker, Eugenia Bertoldo, Dorota Krasowska, Maria João Salvador, Mohammed Tikly, Valeria Riccieri, Ami Sha, Ana Maria Gheorghiu, Cord Sunderkötter, Francesca Ingegnoli, Luc Mouthon, Vanessa Smith, Francesco Paolo Cantatore, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Branimir Anic, Maria Üprus, Brigitte Granel, Alessandra Vacca, Cristina-Mihaela Tanaseanu, Paloma García de la Peña Lefebvre, Jean Sibilia, Ira Litinsky, Lesley Ann Saketkoo, Eduardo Kerzberg, Massimiliano Limonta, Doron Rimar, Petros Sfikakis, Maurizio Cutolo, Rosario Foti, Srdan Novak, Mislav Radic, Raffaele Pellerito, Carlo Francesco Selmi Rozzano, Lidia P Ananieva, Gabriela Szűcs, Carlos de la Puente, Ruxandra Maria Ionescu, Maria Rosa Pozzi, Juan Jose Alegre-Sancho, Kristine Herrmann, Ellen De Langhe, Sule Yavuz Altunizade, Svetlana Agachi, Douglas Veale, Esthela Loyo, Mengtao Li, Edoardo Rosato, Britta Maurer, Iván Castellví, François Spertini, Kamal Solanki, Nicoletta Del Papa, Gerard Espinosa, László Czirják, Bernard Coleiro, Dominique Farge Bancel, Christopher Denton, Nemanja Damjanov, Vera Ortiz Santamaria Granollers, Michaela Kohm, Bojana Stamenkovic, Yannick Allanore, Paolo Airo, Alexandra Balbir-Gurman, Marco Matucci Cerinic, Gabriela Riemekasten, Stefan Heitmann, Nicolas Hunzelmann, Carlomaurizio Montecucco, Jadranka Morovic-Vergles, Camillo Ribi, Michele, Iudici, Denis, Mongin, Elise, Siegert, Patricia E, Carreira, Jörg, Distler, Jörg, Hene, Elisabetta, Zanatta, Eric, Hachulla, Giacomo, De Luca, Carolina de Souza, Müller, Tânia, Santiago, José-Luis, Tandaipan, Breno Valdetaro, Bianchi, Maria, De Santi, Anna-Maria, Hoffmann-Vold, Armando, Gabrielli, Oliver, Distler, Courvoisier, Sophie, Delphine, Cuomo, Giovanna, Moroncini, Gianluca, Stork, Jiri, Iannone, Fiorenzo, Walker, Ulrich, Bertoldo, Eugenia, Krasowska, Dorota, João Salvador, Maria, Tikly, Mohammed, Riccieri, Valeria, Sha, Ami, Maria Gheorghiu, Ana, Sunderkötter, Cord, Ingegnoli, Francesca, Mouthon, Luc, Smith, Vanessa, Paolo Cantatore, Francesco, Eyerich, Kilian, Wiland, Piotr, Vanthuyne, Marie, Anic, Branimir, Üprus, Maria, Granel, Brigitte, Vacca, Alessandra, Tanaseanu, Cristina-Mihaela, García de la Peña Lefebvre, Paloma, Sibilia, Jean, Litinsky, Ira, Ann Saketkoo, Lesley, Kerzberg, Eduardo, Limonta, Massimiliano, Rimar, Doron, Sfikakis, Petro, Cutolo, Maurizio, Foti, Rosario, Novak, Srdan, Radic, Mislav, Pellerito, Raffaele, Francesco Selmi Rozzano, Carlo, P Ananieva, Lidia, Szűcs, Gabriela, de la Puente, Carlo, Maria Ionescu, Ruxandra, Rosa Pozzi, Maria, Jose Alegre-Sancho, Juan, Herrmann, Kristine, De Langhe, Ellen, Yavuz Altunizade, Sule, Agachi, Svetlana, Veale, Dougla, Loyo, Esthela, Li, Mengtao, Rosato, Edoardo, Maurer, Britta, Castellví, Iván, Spertini, Françoi, Solanki, Kamal, Del Papa, Nicoletta, Espinosa, Gerard, Czirják, László, Coleiro, Bernard, Farge Bancel, Dominique, Denton, Christopher, Damjanov, Nemanja, Ortiz Santamaria Granollers, Vera, Kohm, Michaela, Stamenkovic, Bojana, Allanore, Yannick, Airo, Paolo, Balbir-Gurman, Alexandra, Matucci Cerinic, Marco, Riemekasten, Gabriela, Heitmann, Stefan, Hunzelmann, Nicola, Montecucco, Carlomaurizio, Morovic-Vergles, Jadranka, and Ribi, Camillo

Subjects
Rheumatology, systemic sclerosis, Pharmacology (medical), epidemiology, glucocorticoid
Abstract

Objectives To estimate the prevalence of long-term exposure to glucocorticoids (GCs) and to identify factors associated with, and variations in prescribing practices over time and across recruiting countries. Methods We included patients with SSc having a visit recorded in the EUSTAR database from January 2013 onward. We analysed the prevalence and the main features of GCs users, their exposure to GCs over time, and their GCs dosages. Multivariable linear regression was used to analyse the factors identified as associated with GCs intake duration. Time trends, and variations in GCs utilization across recruiting countries were explored. Missing data were imputed using multiple imputation with chained equations. Results The 9819 patients included were mostly females (85%), the majority had lcSSc (73%), and the median age was 58 years. At baseline, 34% of patients (n = 2769/8109) (48% dcSSc vs 29% lcSSc) were on GCs, and the median dose was 7.5 mg/day. GCs users were more frequently males and anti-Scl70 positive, and more commonly had dcSSc and more severe disease. On average, GCs users spent 25% of their follow-up time (median 33.2 months) on GCs, with no significant between-subsets difference. Notably, 33% (n = 971/2959) and 22% (n = 647/2959) of patients followed up for >1 year had received GCs for >6 and >12 months, respectively. Multivariable analysis showed that patient and disease characteristics poorly explained the variability in GCs exposure (adjusted-R2 = 0.06, P Conclusions GCs are widely and long-term prescribed in SSc, with significant between-countries and within-country differences. A gradual decrease in their utilization has been observed.

Published
2022

6. Stratification in systemic sclerosis according to autoantibody status versus skin involvement: a study of the prospective EUSTAR cohort

Author

Muriel Elhai, Nanthara Sritharan, Marouane Boubaya, Alexandra Balbir-Gurman, Elise Siegert, Eric Hachulla, Jeska de Vries-Bouwstra, Gabriela Riemekasten, Jörg H W Distler, Edoardo Rosato, Francesco Del Galdo, Fabian A Mendoza, Daniel E Furst, Carlos de la Puente, Anna-Maria Hoffmann-Vold, Armando Gabrielli, Oliver Distler, Coralie Bloch-Queyrat, Yannick Allanore, Marco Matucci Cerinic, Ulrich Walker, Florenzo Iannone, Suzana Jordan, Radim Becvar, Otylia Kowal Bielecka, Maurizio Cutolo, Giovanna Cuomo, Claudia Kedor, Simona Rednic, Jérome Avouac, P. Vlachoyiannopoulos, C. Montecucco, Jiri Stork, Murat Inanc, Patricia E. Carreira, Srdan Novak, László Czirják, Michele Iudici, Eugene J. Kucharz, Elisabetta Zanatta, Katja Perdan-Pirkmajer, Bernard Coleiro, Gianluca Moroncini, Dominique Farge Bancel, Paolo Airò, Roger Hesselstrand, Mislav Radic, Yolanda Braun-Moscovici, Andrea Lo Monaco, Nicolas Hunzelmann, Raffaele Pellerito, Alessandro Giollo, Jadranka Morovic-Vergles, Christopher Denton, Madelon Vonk, Nemanja Damjanov, Jörg Henes, Vera Ortiz Santamaria, Stefan Heitmann, Dorota Krasowska, Paul Hasler, Michaela Kohm, Ivan Foeldvari, Gianluigi Bajocchi, Maria João Salvador, Bojana Stamenkovic, Carlo Francesco Selmi, Mohammed Tikly, Lidia P. Ananieva, Ariane Herrick, Ulf Müller-Ladner, Raffaele De Palma, Merete Engelhart, Gabriela Szücs, Cristina Sobrino Grande, Øyvind Midtvedt, David Launay, Valeria Riccieri, Ruxandra Maria Ionescu, Ami Sha, Ana Maria Gheorghiu, Cord Sunderkötter, Francesca Ingegnoli, Luc Mouthon, Vanessa Smith, Francesco Paolo Cantatore, Susanne Ullman, Carlos Alberto von Mühlen, Maria Rosa Pozzi, Kilian Eyerich, Piotr Wiland, Marie Vanthuyne, Juan Jose Alegre-Sancho, Kristine Herrmann, Ellen De Langhe, Branimir Anic, Maria Üprus, Sule Yavuz, Brigitte Granel, Carolina de Souza Müller, Joanna Busquets, Svetlana Agachi, Simon Stebbings, D'Alessandro Mathieu, Percival D. Sampaio-Barros, Lisa Stamp, Kamal Solanki, Douglas Veale, Esthela Loyo, Mengtao Li, Walid Ahmed Abdel Atty Mohamed, Antonietta Gigante, Fahrettin Oksel, Cristina-Mihaela Tanaseanu, Rosario Foti, Codrina Ancuta, Britta Maurer, Jacob van Laar, Cristiane Kayser, Nihal Fathi, Paloma García de la Peña Lefebvre, Jean Sibilia, Ira Litinsky, Giuseppina Abignano, Goda Seskute, Lesley Ann Saketkoo, Eduardo Kerzberg, Washington Bianchi, Ivan Castellví, Massimiliano Limonta, Doron Rimar, Maura Couto, François Spertini, Antonella Marcoccia, Sarah Kahl, Ivien M. Hsu, Thierry Martin, Sergey Moiseev, Lorinda S. Chung, Tim Schmeiser, Dominik Majewski, Zbigniew Zdrojewski, Julia Martínez-Barrio, Vera Bernardino, Sabine Sommerlatte, Yair Levy, Elena Rezus, Omer Nuri Pamuk, Piercarlo Sarzi Puttini, Hadi Poormoghim, Ina Kötter, Francis Gaches, Laura Belloli, Petros Sfikakis, Juliana Markus, Gary R Feldman, Ana-Maria Ramazan, H.U. Scherer, Marie-Elise Truchetet, Alain Lescoat, Lorenzo Dagna, J.M. van Laar, Lidia Rudnicka, Susana Oliveira, Fabiola Atzeni, Masataka Kuwana, Arsene Mekinian, Mickaël Martin, Yoshiya Tanaka, Elhai, M., Sritharan, N., Boubaya, M., Balbir-Gurman, A., Siegert, E., Hachulla, E., de Vries-Bouwstra, J., Riemekasten, G., Distler, J. H. W., Rosato, E., Del Galdo, F., Mendoza, F. A., Furst, D. E., de la Puente, C., Hoffmann-Vold, A. -M., Gabrielli, A., Distler, O., Bloch-Queyrat, C., Allanore, Y., Matucci Cerinic, M., Walker, U., Iannone, F., Jordan, S., Becvar, R., Kowal Bielecka, O., Cutolo, M., Cuomo, G., Kedor, C., Rednic, S., Avouac, J., Vlachoyiannopoulos, P., Montecucco, C., Stork, J., Inanc, M., Carreira, P. E., Novak, S., Czirjak, L., Iudici, M., Kucharz, E. J., Zanatta, E., Perdan-Pirkmajer, K., Coleiro, B., Moroncini, G., Farge Bancel, D., Airo, P., Hesselstrand, R., Radic, M., Braun-Moscovici, Y., Lo Monaco, A., Hunzelmann, N., Pellerito, R., Giollo, A., Morovic-Vergles, J., Denton, C., Vonk, M., Damjanov, N., Henes, J., Ortiz Santamaria, V., Heitmann, S., Krasowska, D., Hasler, P., Kohm, M., Foeldvari, I., Bajocchi, G., Salvador, M. J., Stamenkovic, B., Selmi, C. F., Tikly, M., Ananieva, L. P., Herrick, A., Muller-Ladner, U., De Palma, R., Engelhart, M., Szucs, G., Sobrino Grande, C., Midtvedt, O., Launay, D., Riccieri, V., Ionescu, R. M., Sha, A., Gheorghiu, A. M., Sunderkotter, C., Ingegnoli, F., Mouthon, L., Smith, V., Cantatore, F. P., Ullman, S., Alberto von Muhlen, C., Pozzi, M. R., Eyerich, K., Wiland, P., Vanthuyne, M., Alegre-Sancho, J. J., Herrmann, K., De Langhe, E., Anic, B., Uprus, M., Yavuz, S., Granel, B., de Souza Muller, C., Busquets, J., Agachi, S., Stebbings, S., Mathieu, D. A., Sampaio-Barros, P. D., Stamp, L., Solanki, K., Veale, D., Loyo, E., Li, M., Abdel Atty Mohamed, W. A., Gigante, A., Oksel, F., Tanaseanu, C. -M., Foti, R., Ancuta, C., Maurer, B., van Laar, J., Kayser, C., Fathi, N., Garcia de la Pena Lefebvre, P., Sibilia, J., Litinsky, I., Abignano, G., Seskute, G., Saketkoo, L. A., Kerzberg, E., Bianchi, W., Castellvi, I., Limonta, M., Rimar, D., Couto, M., Spertini, F., Marcoccia, A., Kahl, S., Hsu, I. M., Martin, T., Moiseev, S., Chung, L. S., Schmeiser, T., Majewski, D., Zdrojewski, Z., Martinez-Barrio, J., Bernardino, V., Sommerlatte, S., Levy, Y., Rezus, E., Nuri Pamuk, O., Sarzi Puttini, P., Poormoghim, H., Kotter, I., Gaches, F., Belloli, L., Sfikakis, P., Markus, J., Feldman, G. R., Ramazan, A. -M., Scherer, H. U., Truchetet, M. -E., Lescoat, A., Dagna, L., van Laar, J. M., Rudnicka, L., Oliveira, S., Atzeni, F., Kuwana, M., Mekinian, A., Martin, M., and Tanaka, Y.

Subjects
Rheumatology, Immunology, Immunology and Allergy
Abstract

Background: The current subclassification of systemic sclerosis into cutaneous subtypes does not fully capture the heterogeneity of the disease. We aimed to compare the performances of stratification into LeRoy's cutaneous subtypes versus stratification by autoantibody status in systemic sclerosis. Methods: For this cohort study, we assessed people with systemic sclerosis in the multicentre international European Scleroderma Trials and Research (EUSTAR) database. Individuals positive for systemic-sclerosis autoantibodies of two specificities were excluded, and remaining individuals were classified by cutaneous subtype, according to their systemic sclerosis-specific autoantibodies, or both. We assessed the performance of each model to predict overall survival, progression-free survival, disease progression, and different organ involvement. The three models were compared by use of the area under the curve (AUC) of the receiver operating characteristic and the net reclassification improvement (NRI). Missing data were imputed. Findings: We assessed the database on July 26, 2019. Of 16 939 patients assessed for eligibility, 10 711 patients were included: 1647 (15·4%) of 10 709 were male, 9062 (84·6%) were female, mean age was 54·4 (SD 13·8) years, and mean disease duration was 7·9 (SD 8·2) years. Information regarding cutaneous subtype was available for 10 176 participants and antibody data were available for 9643 participants. In the prognostic analysis, there was no difference in AUC for overall survival (0·82, 95% CI 0·81–0·84 for cutaneous only vs 0·84, 0·82–0·85 for antibody only vs 0·84, 0·83–0·86 for combined) or for progression-free survival (0·70, 0·69–0·71 vs 0·71, 0·70–0·72 vs 0·71, 0·70–0·72). However, at 4 years the NRI showed substantial improvement for the antibody-only model compared with the cutaneous-only model in prediction of overall survival (0·57, 0·46–0·71 for antibody only vs 0·29, 0·19–0·39 for cutaneous only) and disease progression (0·36, 0·29–0·46 vs 0·21, 0·14–0·28). The antibody-only model did better than the cutaneous-only model in predicting renal crisis (AUC 0·72, 0·70–0·74 for antibody only vs 0·66, 0·64–0·69 for cutaneous only) and lung fibrosis leading to restrictive lung function (AUC 0·76, 0·75–0·77 vs 0·71, 0·70–0·72). The combined model improved the prediction of digital ulcers and elevated systolic pulmonary artery pressure, but did poorly for cardiac involvement. Interpretation: The autoantibody-only model outperforms cutaneous-only subsetting for risk stratifying people with systemic sclerosis in the EUSTAR cohort. Physicians should be aware of these findings at the time of decision making for patient management. Funding: World Scleroderma Foundation.

Published
2022

7. Genetic Alleles Associated with SLE Susceptibility and Clinical Manifestations in Hispanic Patients from the Dominican Republic

Author

Yangsheng Yu, Michelene Hearth-Holmes, Esthela Loyo, Persio David López, Zheng Liu, Yinshi Yue, Wei-Neng Fu, Carmen Tineo, Kaihong Su, and Glenny Paulino

Subjects
Adult, Male, 0301 basic medicine, Population, Lupus nephritis, Single-nucleotide polymorphism, Polymorphism, Single Nucleotide, Biochemistry, 03 medical and health sciences, 0302 clinical medicine, HLA Antigens, immune system diseases, Odds Ratio, medicine, Humans, Lupus Erythematosus, Systemic, SNP, Genetic Predisposition to Disease, Allele, skin and connective tissue diseases, education, Molecular Biology, Genotyping, Alleles, Genetic Association Studies, 030203 arthritis & rheumatology, education.field_of_study, business.industry, Dominican Republic, Haplotype, Hispanic or Latino, General Medicine, Middle Aged, Tag SNP, medicine.disease, Phenotype, 030104 developmental biology, Haplotypes, Case-Control Studies, Immunology, Molecular Medicine, Female, business
Abstract

Purpose: Systemic lupus erythematosus (SLE) is a complex autoimmune disease with marked disparities in prevalence and disease severity among different ethnic groups. The purpose of this study is to characterize a Latin American cohort and identify genetic risk factors for developing SLE and its end-organ manifestations in this Latin Hispanic cohort. Methods: In this cohort, SNP rs9271366 (tag SNP for HLA-DRB1*15:01) confers the highest risk for SLE among the 13 MHC gene alleles that display association with SLE (p = 8.748E-10; OR = 3.5). Among the 26 non-MHC gene alleles analyzed, SNP rs2476601 in PTPN22 gene confers the highest risk for SLE (p = 0.0001; OR = 5.6). ITGAM, TNFSF4, TNIP1, STAT4, CARD11, BLK, and TNXB gene alleles were confirmed as SLE-susceptible alleles in the DR cohort. However, IRF5 and TNFAIP3 gene alleles, established risk factors for SLE in populations of European and Asian ancestry, are not significantly associated with SLE in this cohort. We also defined a novel HLA-DRA haplotype that confers an increased risk for lupus nephritis (LN) and alleles in HLA-DRA2 and TNFSF4 genes as genetic risk factors for developing neuropsychiatric (NP) SLE. Results: In this cohort, SNP rs9271366 (tag SNP for HLA-DRB1*15:01) confers the highest risk for SLE among the 13 MHC gene alleles that display association with SLE (p = 8.748E-10; OR = 3.5). Among the 26 non-MHC gene alleles analyzed, SNP rs2476601 in PTPN22 gene confers the highest risk for SLE (p = 0.0001; OR = 5.6). ITGAM, TNFSF4, TNIP1, STAT4, CARD11, BLK, and TNXB gene alleles were confirmed as SLE-susceptible alleles in the DR cohort. However, IRF5 and TNFAIP3 gene alleles, established risk factors for SLE in populations of European and Asian ancestry, are not significantly associated with SLE in this cohort. We also defined a novel HLA-DRA haplotype that confers an increased risk for lupus nephritis (LN) and alleles in HLA-DRA2 and TNFSF4 genes as genetic risk factors for developing neuropsychiatric (NP) SLE. Conclusion: Our data suggest that the Latin American population shares some common genetic risk factors for SLE as other populations, but also has distinct risk gene alleles that contribute to SLE susceptibility and development of LN and NPSLE. This is the first study focusing on genetic risk factors for SLE in the DR, a Latin American population that has never been characterized before.

Published
2019

8. Significant weight loss in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database

Author

Michael Hughes, Calvin Heal, Elise Siegert, Eric Hachulla, Paolo Airó, Antonella Riccardi, Oliver Distler, Marco Matucci-Cerinic, Andrea Doria, Lorenzo Baretta, Alexandra Balbir-Gurman, Patricia E Carreira, Vanessa Smith, Carlos Alberto, Jörg Distler von Mühlen, Ulf Müller-Ladner, Lidia P Ananieva, László Czirják, Jörg Henes, Jeska de Vries-Bouwstra, Mengtao Li, Fabian Mendoza, Nemanja Damjanov, Ivan Castellví, Alessandro Giollo, Stefan Heitmann, Edoardo Rosato, Lorenzo Dagna, Christopher P Denton, Marie Vanthuyne, Fabio Cacciapaglia, Valeria Riccieri, Nicolas Hunzelmann, Ami Shah, Carlomaurizio Montecucco, Raffaele Pellerito, Ruxandra Maria Ionescu, Simona Rednic, Ulrich Walker, Maria Rosa Pozzi, Anna-Maria Hoffmann-Vold, Marie-Elise Truchetet, Susanne Ullman, Carolina de Souza Müller, Juan Jose Alegre-Sancho, Eduardo Kerzberg, Francesco Del Galdo, Gabriela Riemekasten, Branimir Anic, Marko Baresic, Miroslav Mayer, Fahrettin Oksel, Figen Yargucu, Ellen De Langhe, Ina Kötter, Mohammed Tikly, Radim Becvar, Douglas Veale, Dorota Krasowska, Andrea Lo Monaco, Lidia Rudnicka, Ana Maria Gheorghiu, Piercarlo Sarzi Puttini, Mislav Radic, Armando Gabrielli, Maria João Salvador, Carlos de la Puente, Gabriela Szücs, Sule Yavuz, Rosario Foti, Otylia Kowal Bielecka, Codrina Ancuta, Peter Villiger, Sabine Adler, Patrick Jego, Michaela Kohm, Eugene J Kucharz, Dominique Farge Bancel, Tim Schmeiser, Alberto Cauli, Alessandra Vacca, Kamal Solanki, Piotr Wiland, Paloma García de la Peña Lefebvre, Jorge Juan Gonzalez Martin, Sergio Jimenez, Lesley Ann Saketkoo, Roger Hesselstrand, Francesca Ingegnoli, Jean Sibilia, Merete Engelhart, Esthela Loyo, Carmen Tineo, Francesco Paolo Cantatore, Brigitte Krummel-Lorenz, Petros Sfikakis, Cristiane Kayser, Vera Ortiz Santamaria, Bojana Stamenkovic, Giovanna Cuomo, Francesco Puppo, Thierry Zenone, Nihal Fathi, Ira Litinsky, Carlo Chizzolini, Monika Swacha, Washington Bianchi, Breno Valdetaro Bianchi, Maria Üprus, Kati Otsa, Masataka Kuwana, Panayiotis Vlachoyiannopoulos, Sarah Kahl, Bernard Coleiro, François Spertini, Walid Ahmed Abdel Atty Mohamed, Sergey Moiseev, Pavel Novikov, Dominik Majewski, Simon Stebbings, Svetlana Agachi, Massimiliano Limonta, Carlo Francesco Selmi, Elena Rezus, Kristine Herrmann, Brigitte Granel, Goda Seskute, Matthias Seidel, Paul Hasler, Maurizio Cutolo Vera Bernardino, Carmen Pizzorni, Jadranka Morovic-Vergles, Daniel Furst, Ana-Maria Ramazan, Gianluigi Bajocchi, Lisa Stamp, Doron Rimar, Antonella Marcoccia, Srdan Novak, Luc Mouthon, Jiri Stork, Lorinda S Chung, Hadi Poormoghim, Francis Gaches, Laura Belloli, Cristina-Mihaela Tanaseanu, Fabiola Atzeni, Kilian Eyerich, Ivien M Hsu, Jacob van Laar, Mary Ellen Csuka, Omer Nuri Pamuk, Maura Couto, Arsene Mekinian, Murat Inanc, Ivan Foeldvari, Julia Martínez-Barrio, Yair Levy, Juliana Markus, Susana Oliveira, Hughes, Michael, Heal, Calvin, Siegert, Elise, Hachulla, Eric, Airó, Paolo, Riccardi, Antonella, Distler, Oliver, Matucci-Cerinic, Marco, Doria, Andrea, Baretta, Lorenzo, Balbir-Gurman, Alexandra, E Carreira, Patricia, Smith, Vanessa, Alberto, Carlo, Distler von Mühlen, Jörg, Müller-Ladner, Ulf, P Ananieva, Lidia, Czirják, László, Henes, Jörg, de Vries-Bouwstra, Jeska, Li, Mengtao, Mendoza, Fabian, Damjanov, Nemanja, Castellví, Ivan, Giollo, Alessandro, Heitmann, Stefan, Rosato, Edoardo, Dagna, Lorenzo, P Denton, Christopher, Vanthuyne, Marie, Cacciapaglia, Fabio, Riccieri, Valeria, Hunzelmann, Nicola, Shah, Ami, Montecucco, Carlomaurizio, Pellerito, Raffaele, Maria Ionescu, Ruxandra, Rednic, Simona, Walker, Ulrich, Rosa Pozzi, Maria, Hoffmann-Vold, Anna-Maria, Truchetet, Marie-Elise, Ullman, Susanne, de Souza Müller, Carolina, Jose Alegre-Sancho, Juan, Kerzberg, Eduardo, Del Galdo, Francesco, Riemekasten, Gabriela, Anic, Branimir, Baresic, Marko, Mayer, Miroslav, Oksel, Fahrettin, Yargucu, Figen, De Langhe, Ellen, Kötter, Ina, Tikly, Mohammed, Becvar, Radim, Veale, Dougla, Krasowska, Dorota, Lo Monaco, Andrea, Rudnicka, Lidia, Maria Gheorghiu, Ana, Sarzi Puttini, Piercarlo, Radic, Mislav, Gabrielli, Armando, João Salvador, Maria, de la Puente, Carlo, Szücs, Gabriela, Yavuz, Sule, Foti, Rosario, Kowal Bielecka, Otylia, Ancuta, Codrina, Villiger, Peter, Adler, Sabine, Jego, Patrick, Kohm, Michaela, J Kucharz, Eugene, Farge Bancel, Dominique, Schmeiser, Tim, Cauli, Alberto, Vacca, Alessandra, Solanki, Kamal, Wiland, Piotr, García de la Peña Lefebvre, Paloma, Juan Gonzalez Martin, Jorge, Jimenez, Sergio, Ann Saketkoo, Lesley, Hesselstrand, Roger, Ingegnoli, Francesca, Sibilia, Jean, Engelhart, Merete, Loyo, Esthela, Tineo, Carmen, Paolo Cantatore, Francesco, Krummel-Lorenz, Brigitte, Sfikakis, Petro, Kayser, Cristiane, Ortiz Santamaria, Vera, Stamenkovic, Bojana, Cuomo, Giovanna, Puppo, Francesco, Zenone, Thierry, Fathi, Nihal, Litinsky, Ira, Chizzolini, Carlo, Swacha, Monika, Bianchi, Washington, Valdetaro Bianchi, Breno, Üprus, Maria, Otsa, Kati, Kuwana, Masataka, Vlachoyiannopoulos, Panayioti, Kahl, Sarah, Coleiro, Bernard, Spertini, Françoi, Ahmed Abdel Atty Mohamed, Walid, Moiseev, Sergey, Novikov, Pavel, Majewski, Dominik, Stebbings, Simon, Agachi, Svetlana, Limonta, Massimiliano, Francesco Selmi, Carlo, Rezus, Elena, Herrmann, Kristine, Granel, Brigitte, Seskute, Goda, Seidel, Matthia, Hasler, Paul, Cutolo Vera Bernardino, Maurizio, Pizzorni, Carmen, Morovic-Vergles, Jadranka, Furst, Daniel, Ramazan, Ana-Maria, Bajocchi, Gianluigi, Stamp, Lisa, Rimar, Doron, Marcoccia, Antonella, Novak, Srdan, Mouthon, Luc, Stork, Jiri, S Chung, Lorinda, Poormoghim, Hadi, Gaches, Franci, Belloli, Laura, Tanaseanu, Cristina-Mihaela, Atzeni, Fabiola, Eyerich, Kilian, M Hsu, Ivien, van Laar, Jacob, Ellen Csuka, Mary, Nuri Pamuk, Omer, Couto, Maura, Mekinian, Arsene, Inanc, Murat, Foeldvari, Ivan, Martínez-Barrio, Julia, Levy, Yair, Markus, Juliana, and Oliveira, Susana

Subjects
Adult, Male, 0301 basic medicine, medicine.medical_specialty, weight loss, systemic sclerosis, nutrition, Immunology, Disease, computer.software_genre, General Biochemistry, Genetics and Molecular Biology, Scleroderma, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Risk Factors, Weight loss, Internal medicine, Weight Loss, Epidemiology, medicine, Humans, Immunology and Allergy, 610 Medicine & health, Aged, 030203 arthritis & rheumatology, Scleroderma, Systemic, Database, business.industry, Middle Aged, medicine.disease, 030104 developmental biology, Mood, Databases as Topic, Female, Outcomes research, medicine.symptom, business, computer, Rheumatism
Abstract

Gastrointestinal (GI) involvement is almost universal in patients with systemic sclerosis (SSc) and is associated with significant disease-related morbidity and mortality.1 The entire GI tract can be involved and other disease features (eg, low mood, terminal organ failure and functional hand impairment) can result in significant nutritional impairment. Severe GI involvement has been reported to occur in ~10% of patients with SSc and often occurs early in the course of the disease.2 However, identification of patients at high risk of clinically significant weight loss is extremely challenging, including from the high prevalence of GI symptoms in patients with SSc. Therefore, there is a need to understand high-risk patients including potentially modifiable risk factors, with a view to early intervention strategies. Against this background, the aim of this study was to examine potential clinical risk factors of significant weight loss in patients with SSc. We performed an analysis of patients with SSc enrolled in the multinational, longitudinal European League Against Rheumatism (EULAR) Scleroderma Trials and Research (EUSTAR) database. In our study, we defined significant weight loss as 4.5 kg and/or least 5% of their body weight at 5 months onwards.3 Patients with a recorded second visit after 3 months and before 12 months were included in the analysis. We adopted a pragmatic approach (relevant to clinical practice) in …

Published
2020
Full Text
View/download PDF

9. AB0788C PSORIASTIC ARTHRITIS IN PATIENTS DIAGNOSED WITH PSORIASIS ASSISTING DERMATOLOGY CONSULTATION IN A COHORT OF THE DOMINICAN REPUBLIC

Author

Catherine Rodríguez, Yamel Camilo Uribe, Yanela Suriel Ayala, Franchesca alvarez Polanco, Glenny Paulino, Carmen Tineo, Anllely altagracia Tapia Báez, Piery Guzmán, Diana Rozón Rozón, Esthela Loyo de López, and Idelfi Sánchez

Subjects
medicine.medical_specialty, education.field_of_study, Oligoarthritis, business.industry, Population, Arthritis, medicine.disease, Dermatology, Rheumatology, Psoriatic arthritis, Internal medicine, Psoriasis, Epidemiology, medicine, Polyarthritis, business, education
Abstract

Background: Psoriatic arthritis (PsA) is a disease of an inflammatory, heterogeneous nature that involves skin, nails, peripheral and axial joints, as well as the entheses. Due to its systemic involvement, it makes diagnosis and therapy a challenge in clinical practice. (1) It has been estimated that around 6% to 41% of patients with Psoriasis (Pso) will develop PsA, finding an accumulated incidence of 1.7% at 5 years of diagnosis of Psoriasis, 3.1% at 10 years and 5.1% at 20 years (2). In the Dominican Republic we do not have studies that characterize this pathology, so it would be interesting to be able to specify the clinical course, as well as the risk factors and associated prognoses for the development of this entity. Objectives: To determine the factors associated with psoriatic arthritis in patients diagnosed with psoriasis attending the Dermatology service. Methods: A multicenter, descriptive study of primary and secondary source data collection was conducted at the Psoriasis clinic of the Regional University Hospital Jose Maria Cabral and Baez, Cibao Regional Dermatological institute and the Foundation to support patients with psoriasis and psoriatic arthritis (FUNAPAPSO), in the period of august-December 2018, the universe consisted of 304 patients, 103 patients met inclusion criteria. After signing informed consent, a form was completed that included general, clinical, serological and image data. Analysis of the variables was performed and Chi 2 was used, considering statistical significance p Results: Of a total of 103 patients with psoriasis, the mean age was 40 ± 13 years, 63.1% male, 36.9% female. The prevalence of psoriatic arthritis by CASPAR criteria was 36.89%. The presentation of arthritis was asymmetric oligoarthritis in 56.52%, symmetric polyarthritis 21.74% and distal interphalangeal arthritis 13.04%, radiographic findings were present in 7.77% (p: 0.00) of patients with PsA. The forms of presentation of Pso that were most associated with arthritis were plaques 48.57% (p: 0.000) and drop 12.5% (p: 0.01). The quality of life was average in 39.47% and poor in 18.42%. Psoriasic onychopathy was present in 40.23% (p: 0.048) of patients with arthritis. 39% of patients had some type of comorbidity, and of these arterial hypertension obtained 34% (n = 13), obesity 26% (n = 10) and diabetes mellitus 21% (n = 8). Conclusion: The presence of psoriatic arthritis in the population with psoriasis in our environment is high. This is related to the presence of psoriatic onicopathy, radiographic findings and deterioration in the quality of life of patients. References [1] S Raychaudhuri, RW, aS, SR, EM. Management of psoriatic arthritis: Early diagnosis, monitoring of disease severity and cutting edge therapies. Journal of autoimmunity. 2016Octubre. [2] a Ogdie P. The Epidemiology of Psoriatic arthritis. Rheum Dis Clin N am.2015 [3] P Helliwel. Natural history, prognosis and socioeconomic aspects of Psoriatic arthrits. Rheum Dis Clin N am. 2015 [4] M. Boutet, aGC. Role of the IL-23/IL-17 axis in Psoriasis and Psoriatic arthritis: the clinic importance of its divergence in skin and joints. Int J Molc Sci 2018; 19(2):p.530 [5] L. Sakkas. Are psoriasis and psoriatic arthritis the same disease? autoimmunity Review.2017Enero; 16(1): p10-15 [6] J Torre. Utilidad y aplicacion en la practica clinica de los criterios CASPAR. Reumatol Clin2010; 6(1):p 18-21 [7] L Gossec, LM, aS, PC, DJ. Management of psoriatic arthritis in 2016: a comparison of EULAR and GRAPPA recommendations. Nature Reviews Rheumatology. 2016 Disclosure of interests: None declared

Published
2019

10. Autoantibodies to Mi-2 alpha and Mi-2 beta in patients with idiopathic inflammatory myopathy

Author

Mónica Vázquez-Del Mercado, Esthela Loyo, Gabriel Medrano-Ramírez, Andrea Seaman, Graciela Gómez, Armando Rojo-Mejia, Martial Koenig, Marvin J. Fritzler, Lilia Andrade-Ortega, Jose Eduardo Navarro-Zarza, Michaelin Richards, Michael Mahler, Marco A. Maradiaga-Ceceña, Yelitza Concepción González-Bello, and Ignacio García-De La Torre

Subjects
Male, medicine.medical_specialty, Alpha (ethology), Context (language use), Gastroenterology, Dermatomyositis, Rheumatology, Internal medicine, medicine, Humans, Pharmacology (medical), Autoantibodies, Immunoassay, Receiver operating characteristic, medicine.diagnostic_test, biology, Myositis, business.industry, Area under the curve, Autoantibody, Reproducibility of Results, Polymyositis, ROC Curve, Case-Control Studies, biology.protein, Female, Antibody, business, Kappa, Biomarkers, Mi-2 Nucleosome Remodeling and Deacetylase Complex
Abstract

Objectives The objective of this study was to compare the results obtained from different assays for the detection of anti-Mi-2 antibodies, which are important markers in the diagnosis of DM. Methods The study included 82 patients (68 females/14 males), most of whom had DM (n = 57), followed by PM (n = 16) and juvenile DM (n = 9). All samples were tested using a novel particle-based multi-analyte technology (PMAT) (Inova Diagnostics, research use only) in parallel with a line immunoassay (LIA: Euroimmun). To assess clinical specificity for the PMAT assay, a total of 775 disease and healthy controls were tested. Results 29 samples were positive by at least one test for anti-Mi-2 antibodies. Of those, 24 were Mi-2β LIA+, five were Mi-2α LIA+ and 23 Mi-2 PMAT+. The comparison shows varying agreement between the different methods (kappa 0.27–0.77). When LIA results were used as reference for receiver operating characteristics analysis, high area under the curve values were found for both PMAT vs LIA Mi-2α and LIA Mi-2β. When analysing the results in the context of the myositis phenotype, PMAT associated closest with the DM phenotype. In the control group, 3/775 controls (all low levels) were anti-Mi-2+ resulting in a sensitivity and specificity of 28.1% and 99.6%, respectively. Conclusion Overall, good agreement was found between LIA and PMAT for anti-Mi-2 antibodies, which is important for the standardization of autoantibodies. Anti-Mi-2β antibodies measured by PMAT tend be more highly associated with the clinical phenotype of DM.

Published
2018

11. Smoking in Systemic Sclerosis: a Longitudinal European Scleroderma Trials and Research Group Study

Author

Jaeger, Vk, Valentini, G, Hachulla, E, Cozzi, F, Distler, O, Airó, P, Czirják, L, Allanore, Y, Siegert, E, Rosato, E, Matucci-Cerinic, M, Caimmi, C, Henes, J, Carreira, Pe, Smith, V, Del Galdo, F, Denton, Cp, Ullman, S, Langhe, E, Riccieri, V, Alegre-Sancho, Jj, Rednic, S, Müller-Ladner, U, Walker, Ua, Ananieva, Lidia P., Florenzo, Iannone, Distler, JeorgH. W., Eduardo, Kerzberg, Bojana, Stamenkovic, José Antonio Pereira Da Silva, Ivan, Catellvì, Suleyavuz, Kamal, Solanki, Branimir, Anic, Esthela, Loyo, Tim, Schmeiser, Ruxandra Maria Ionescu, Peter, Villiger, Francesco Paolo Cantatore, Paloma Garcia de la Pena Lefebvre, Carina, Mihai, Dominique Farge Bancel, Pierluigi, Meroni, Thierry, Martin, Stefan, Heitmann, Nicolas, Hunzelmann, Fahrettin, Oksel, Washington, Bianchi, Sara, Bongiovanni, Radim, Becvar, Thierry, Zenone, Alexandra, Balbir-Gurman, Piotr, Wiland, Pawel, Zebryk, Francßoisspertini, Lori, Chung, Alessandra, Vacca, Carlo, Chizzolini, Carlo Francesco Selmi, Cristina-Mihaela, Tanaseanu, Ira, Litinsky, Houssiau, Frederic A., Brigitte, Krummel-Lorenz, Hsu, Vivien M., Francisca, Fontes, Alexandra Maria Burlui, Duska, Martinovic, Sergei, Popa, Itzhak, Rosner, Carmel, Mallia, Rosario, Foti, Luc, Mouthon, Martin, Aringer, Jacob van Laar, Roger, Hesselstrand, Maria Rosa Pozzi, Gabriela, Riemekasten, Cutolo, Maurizio, Paul, Hasler, Zea Mendoza Antonio, Cord, Sunderkotter, Codrina, Ancuta, Jean, Sibilia, Roberto, Caporali, Armando, Gabrielli, Raffaele, Pellerito, Ariane, Herrick, Øyvind, Midtvedt, Kilian, Eyerich, Brigitte, Granel, Lesley Ann Saketkoo, Maura, Couto, Yael, Eizikovits, and Giuseppina, Abignano.

Published
2018

12. AB0788C PSORIASTIC ARTHRITIS IN PATIENTS DIAGNOSED WITH PSORIASIS ASSISTING DERMATOLOGY CONSULTATION IN A COHORT OF THE DOMINICAN REPUBLIC

Author

Tapia Báez, Anllely altagracia, primary, Polanco, Franchesca alvarez, additional, López, Esthela Loyo de, additional, Tineo, Carmen, additional, Paulino, Glenny, additional, Rodríguez, Catherine, additional, Rozón, Diana Rozón, additional, Ayala, Yanela Suriel, additional, Uribe, Yamel Camilo, additional, Guzmán, Piery, additional, and Sánchez, Idelfi, additional

Published
2019
Full Text
View/download PDF

13. Scleroderma in the Caribbean: Characteristics in a Dominican Case Series

Author

Paola Gottschalk, Esthela Loyo, Persio David López, Carmen Tineo, Ricardo Vásquez, and Jossiell Then

Subjects
Adult, Male, medicine.medical_specialty, Pediatrics, Adolescent, Population, Disease, Scleroderma, Young Adult, Epidemiology, Prevalence, medicine, Humans, Age of Onset, education, Aged, Retrospective Studies, Series (stratigraphy), education.field_of_study, Scleroderma, Systemic, Traditional medicine, business.industry, Dominican Republic, General Medicine, Middle Aged, medicine.disease, Caribbean Region, Female, Rheumatology department, Age of onset, business, Rare disease
Abstract

Introduction Scleroderma is a rare disease with limited data in Latin America. Preliminary genetic studies suggest a strong African ascendance in the Dominican Republic, which could modulate the expression of the disease. The objective of this study is to describe the clinical and demographic characteristics of scleroderma in a series of 26 Dominican patients. Materials and methods Patients who fulfilled the EULAR/ACR criteria for scleroderma were selected from the Rheumatology Department of a tertiary health center; systemic sclerosis subtypes were defined according to the EULAR classification. Clinical and demographic information was obtained retrospectively from clinical records. Results Mean age at time of onset was 32.6±15 years; 68% of patients had 40 years of age or less. 73% of patients were females, with a female:male ratio of 2.7:1. The most affected systems were pulmonary and gastrointestinal; renal affection was scarce. Anti-Scl-70 antibodies were positive in 64% of patients, sometimes in coexistence with anti-centromere antibodies. Conclusions The prevalence of systemic sclerosis is lower in the Dominican population than was reported elsewhere. The age of onset of the disease seems to be lower in the Dominican population than that reported in literature. A different pattern of autoantibodies is observed in this population.

Published
2014

14. Esclerodermia en el Caribe: características en una serie de casos dominicana

Author

Jossiell Then, Ricardo Vásquez, Persio David López, Esthela Loyo, Carmen Tineo, and Paola Gottschalk

Subjects
Rheumatology, business.industry, Medicine, business, Humanities
Abstract

Resumen Introduccion La esclerodermia es una enfermedad rara, de la cual existe informacion limitada en America Latina. Estudios preliminares en genetica muestran que la ascendencia de Republica Dominicana tiene fuerte influencia africana, lo cual podria modular la expresion de la enfermedad. El proposito de este estudio es describir las caracteristicas clinicas y demograficas de esclerodermia en una serie de pacientes dominicanos. Materiales y metodos Se seleccionaron los pacientes que cumplieron con los criterios del EULAR/ACR para esclerosis sistemica de la base de datos del servicio de reumatologia de un centro terciario. Se definieron los subtipos de esclerodermia de acuerdo a la clasificacion EULAR. La informacion clinica y demografica al momento del diagnostico fue obtenida de forma retrospectiva de los expedientes medicos. Resultados La prevalencia fue estimada de 9,3 por millon de habitantes. Veinte y seis pacientes entraron al estudio. La edad media al momento del primer sintoma fue 32,6 ± 15 anos; el 68% de los pacientes tenia 40 anos de edad o menos cuando aparecieron los sintomas. El 73,1% de los pacientes fue de sexo femenino, con una relacion mujer:hombre 2,7:1. Los sistemas organicos mas afectados fueron el pulmonar y el gastrointestinal; la afectacion renal fue rara. Los anticuerpos anti-Scl-70 se encontraron positivos en el 64% de los casos y, en 2 casos, en coexistencia con anticentromero. Conclusiones La prevalencia de esclerosis sistemica es menor en la poblacion dominicana que la reportada internacionalmente. La edad de inicio de la enfermedad parece ser menor en la poblacion dominicana que la reportada en la literatura. Un patron distinto de autoanticuerpos es observado en esta poblacion.

Published
2014

15. Mapping and predicting mortality from systemic sclerosis

Author

Kamal Solanki, Roger Hesselstrand, Ulrich A Walker, Jiri Stork, Carlo Chizzolini, Stefan Heitmann, Vanessa Smith, Maurizio Cutolo, Simona Rednic, Carolina de Souza Müller, Jörg H W Distler, Luc Mouthon, Nicolas Hunzelmann, Edoardo Rosato, Mohammed Tikly, Franco Cozzi, Nemanja Damjanov, Paola Caramaschi, Florenzo Iannone, Sule Yavuz, Alexandra Balbir-Gurman, Marco Matucci-Cerinic, Duska Martinovic, Carlos Alberto Von Mühlen, Beatriz Joven, Carina Mihai, Oliver Distler, Paolo Airò, Emmanuel Chatelus, Paloma García de la Peña Lefebvre, Gabriela Riemekasten, Grégoire Rey, Marouane Boubaya, Jörg Henes, Alessandra Vacca, Ulf Müller-Ladner, Thierry Zenone, Armando Gabrielli, Srdan Novak, Ellen De Langhe, Juan José Alegre-Sancho, Walter Alberto Sifuentes-Giraldo, R. Becvar, Jacob M van Laar, Matthias Seidel, Carlomaurizio Montecucco, Daniela Opris, Esthela Loyo, Kilian Eyerich, Vera Ortiz Santamaria, Otylia Kowal-Bielecka, L. Ananieva, Muriel Elhai, Valeria Riccieri, Vanesa Cosentino, Christophe Meune, Panayiotis G. Vlachoyiannopoulos, Jérôme Avouac, Maria De Santis, Branimir Anić, Eric Hachulla, Susanne Ullman, Francesca Ingegnoli, Gabriella Szücs, Bojana Stamenkovic, Yannick Allanore, Serena Vettori, Maria João Salvador, László Czirják, Lisa K. Stamp, Elhai, Muriel, Meune, Christophe, Boubaya, Marouane, Avouac, Jã©rã´me, Hachulla, Eric, Balbir gurman, Alexandra, Riemekasten, Gabriela, Airã², Paolo, Joven, Beatriz, Vettori, Serena, Cozzi, Franco, Ullman, Susanne, Czirjã¡k, Lã¡szlã³, Tikly, Mohammed, Mã¼ller 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, Szã¼cs, Gabriella, Walker, Ulrich A, Hunzelmann, Nicola, Martinovic, Duska, Smith, Vanessa, Mã¼ller, Carolina De Souza, Montecucco, Carlo Maurizio, Opris, Daniela, Ingegnoli, Francesca, Vlachoyiannopoulos, Panayiotis G, Stamenkovic, Bojana, Rosato, Edoardo, Heitmann, Stefan, Distler, Jã¶rg H. W, Zenone, Thierry, Seidel, Matthia, Vacca, Alessandra, Langhe, Ellen De, Novak, Srdan, Cutolo, Maurizio, Mouthon, Luc, Henes, Jã¶rg, Chizzolini, Carlo, Mã¼hlen, 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, De La Peã±a Lefebvre, Paloma Garcia, Eyerich, Kilian, Cosentino, Vanesa, Alegre sancho, Juan Jose, Kowal bielecka, Otylia, Rey, Grã©goire, Matucci cerinic, Marco, and Allanore, Yannick

Subjects
pulmonary fibrosi, 0301 basic medicine, Male, Genetics and Molecular Biology (all), Time Factors, Databases, Factual, systemic sclerosis, Disease, Biochemistry, Scleroderma, 0302 clinical medicine, cardiovascular disease, Risk Factors, Cause of Death, Epidemiology, Immunology and Allergy, skin and connective tissue diseases, Cause of death, BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti. Interna medicina, ddc:616, education.field_of_study, Framingham Risk Score, integumentary system, Orvostudományok, Middle Aged, Prognosis, 3. Good health, Quartile, Cohort, epidemiology, Female, France, systemic sclerosi, pulmonary fibrosis, Aged, Death Certificates, Humans, Proportional Hazards Models, Scleroderma, Systemic, Rheumatology, Immunology, Biochemistry, Genetics and Molecular Biology (all), Human, medicine.medical_specialty, Time Factor, Prognosi, Population, Klinikai orvostudományok, General Biochemistry, Genetics and Molecular Biology, 03 medical and health sciences, Databases, Internal medicine, cardiovascular disease, epidemiology, pulmonary fibrosis, systemic sclerosis, medicine, education, BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences. Internal Medicine, Factual, 030203 arthritis & rheumatology, Proportional hazards model, business.industry, Risk Factor, Systemic, Surgery, 030104 developmental biology, Death Certificate, Proportional Hazards Model, business
Abstract

ObjectivesTo determine the causes of death and risk factors in systemic sclerosis (SSc).MethodsBetween 2000 and 2011, we examined the death certificates of all French patients with SSc to determine causes of death. Then we examined causes of death and developed a score associated with all-cause mortality from the international European Scleroderma Trials and Research (EUSTAR) database. Candidate prognostic factors were tested by Cox proportional hazards regression model by single variable analysis, followed by a multiple variable model stratified by centres. The bootstrapping technique was used for internal validation.ResultsWe identified 2719 French certificates of deaths related to SSc, mainly from cardiac (31%) and respiratory (18%) causes, and an increase in SSc-specific mortality over time. Over a median follow-up of 2.3 years, 1072 (9.6%) of 11 193 patients from the EUSTAR sample died, from cardiac disease in 27% and respiratory causes in 17%. By multiple variable analysis, a risk score was developed, which accurately predicted the 3-year mortality, with an area under the curve of 0.82. The 3-year survival of patients in the upper quartile was 53%, in contrast with 98% in the first quartile.ConclusionCombining two complementary and detailed databases enabled the collection of an unprecedented 3700 deaths, revealing the major contribution of the cardiopulmonary system to SSc mortality. We also developed a robust score to risk-stratify these patients and estimate their 3-year survival. With the emergence of new therapies, these important observations should help caregivers plan and refine the monitoring and management to prolong these patients’ survival.

Published
2017

16. Autoimmunity in connection with a metal implant: a case of autoimmune/autoinflammatory syndrome induced by adjuvants

Author

Luis J. Jara, Ana Carolina Puig, Esthela Loyo, and Persio David López

Subjects
medicine.medical_specialty, Adult onset Still’s disease, Proteinuria, Anti-nuclear antibody, business.industry, Anemia, Immunology, Hepatosplenomegaly, Case Report, Neutropenia, medicine.disease, Autoinflammatory Syndrome, medicine.disease_cause, Dermatology, Autoimmunity, Metal hypersensitivity, Rheumatology, Antiphospholipid syndrome, ASIA syndrome, medicine, Lupus-like syndrome, medicine.symptom, business
Abstract

Autoimmune/autoinflammatory syndrome induced by adjuvants (ASIA) has been recently proposed by Shoenfeld and Agmon-Levin as a new entity that comprises several conditions: the macrophagic-myofasciitis syndrome, the Gulf War syndrome, silicosis and post-vaccination phenomena, autoimmunity related to infectious fragments, hormones, aluminum, silicone, squalene oil, and pristane. We report the case of a 23-year-old woman who developed serial episodes of high fever, extreme fatigue, transient thrombocytopenia, multiple cervical adenopathies, hepatosplenomegaly, anemia, neutropenia, severe proteinuria and urine sediment abnormalities, elevated serum ferritin levels, and transient low positive antinuclear antibodies 1 year after she had a nickel-titanium chin implant for cosmetic reasons. The clinical picture simulated a variety of probable diseases: systemic lupus erythematosus, Kikuchi-Fujimoto syndrome, adult onset Still's disease, antiphospholipid syndrome, and hemophagocytic syndrome, among others, so she underwent an extensive medical investigation including two lymph node biopsies. She received treatment accordingly with steroids, methotrexate, and mofetil mycophenolate, with initial improvement of her symptoms, which recurred every time the dose was reduced. Two and a half years later the patient decided to retire the chin implant and afterwards all her systemic symptoms have disappeared. She remains in good health, without recurrence of any symptom and off medications until today. Albeit this patient fulfills proposed major ASIA criteria, to our knowledge it would be the first description of systemic features of autoinflammation in connection with a metal implant.

Published
2012

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

Author

Antonio C. Zea Mendoza, Jean Sibilia, Kamal Solanki, Cristina Mihaela Tanaseanu, Fredrick M. Wigley, Guido Valesini, M. Govoni, Lisa K. Stamp, Christopher P. Denton, Yolanda Braun-Moscovici, Ruxandra Ionescu, Øyvind Midtvedt, Ileana Nicoara, Aleksandra Stanković, Rüdiger Hein, Alina Dumitrascu, Susanne Ullman, Alan Tyndall, Sergio A. Jimenez, Irena Butrimiene, Alan Doube, Eugene J. Kucharz, Mohammed Tikly, Pier Luigi Meroni, Simon Stebbings, Renata Sokolik, Alexandra Balbir Gurman, Roger Hesselstrand, Kirsten Damgaard, Francesco Paolo Cantatore, Razvan Ionitescu, Silvana Zeni, Marco Matucci-Cerinic, Maria Rosa Pozzi, Jacques-Eric Gottenberg, Srdan Novak, Ana Maria Gherghe, David Launay, Liliana Groppa, Carlomaurizio Montecucco, Roxana Sfrent Cornateanu, Stefan Heitmann, Paloma García de la Peña Lefebvre, Daniela Opris, Peter T. Chapman, Line V. Iversen, Bernard Coleiro, Ulf Müller-Ladner, John Highton, Mara Oleszowsky, Gabriella Szücs, Magdalena Kopec-Medrek, Carlos De La Puente Buijdos, Paola Caramaschi, Magdalena Szmyrka-Kaczmarek, Rucsandra Dobrota, Gabriele Valentini, Fabiana Montoya, Blaz Rozman, Alberto Sulli, Hélène Chifflot, Raffaella Scorza, Patricia Carreira, Paulius Venalis, Lisa Maria Bambara, Torhild Garen, Isabela Tiglea, Agneta Scheja, Duska Martinovic, Jörg H W Distler, Jörg Henes, Giovanni Lapadula, Luc Mouthon, Diana Karpec, Douglas J. Veale, Valeria Riccieri, Nicolas Hunzelmann, Muriel Elhai, Serena Guiducci, Codrina Ancuta, Simonetta Pisarri, Thierry Zenone, Esthela Loyo, Branimir Anić, Claudia Günther, R. Becvar, Eugen Russu, Serena Vettori, Carlo Chizzolini, Vanessa Smith, Mengtao Li, Stanislaw Sierakowsky, Carmel Mallia, Małgorzata Widuchowska, Carolina de Souza Müller, László Czirják, Algirdas Venalis, Adrian Hij, Marta Valero Exposito, Simona Rednic, Miroslav Mayer, Laura Groseanu, Walter Alberto Sifuentes Giraldo, Murray Baron, Dominique Farge, Anna Kotulska, Marko Baresic, Svetlana Agachi, Martin Aringer, Merete Engelhart, John L. O'Donnell, Jérôme Avouac, Filip De Keyser, Ulrich A. Walker, Roberto Caporali, Harald Burkhardt, P. G. Vlachoyiannopoulos, Maurizio Cutolo, Frank A. Wollheim, Edoardo Rosato, Suzanne Kafaja, Valderílio Feijó Azevedo, Kilian Eyerich, Paolo Airò, Emmanuel Chatelus, L. Ananieva, Ira Litinsky, Andrea Lo Monaco, Vanesa Cosentino, Rita Rugiene, Eric Hachulla, P. Saar, Bojana Stamenkovic, Brigitte Krummel-Lorenz, Yannick Allanore, Elisabeth Knott, Oliver Distler, Matthias Seidel, Silvia Rodriguez Rubio, Franco Cozzi, Mihai Bojinca, Nemanja Damjanov, Maria João Salvador, Joanna Busquets, Otylia Kowal Bielecka, André Kahan, Jacek Szechiński, Daniel E. Furst, C. Mihai, Rodica Chirieac, Ewa Morgiel, Georg Schett, Armando Gabrielli, Giovanna Cuomo, Piotr Wiland, Maya N. Starovoytova, Sebastião Cezar Radominski, Gitte Strauss, Lealea Chiaburu, Florenzo Iannone, Carol M. Black, Andrea Himsel, Eduardo Kerzberg, Cecília Varjú, Vera Ortiz Santamaria, Gabriela Riemekasten, Dorota Krasowska, Marilena Gorga, Monica Popescu, Marie O'Rourke, Henrik Nielsen, Raffaele Pellerito, Ada Corrado, Elhai, M, Avouac, J, Walker, Ua, Matucci Cerinic, M, Riemekasten, G, Airò, P, Hachulla, E, Valentini, Gabriele, Carreira, Pe, Cozzi, F, Balbir Gurman, A, Braun Moscovici, Y, Damjanov, N, Ananieva, Lp, Scorza, R, Jimenez, S, Busquets, J, Li, M, Müller Ladner, U, Kahan, A, Distler, O, Allanore, Y, EUSTAR co, Author, EUSTAR co, Authors, Matucci-Cerinic, M, Airo, P, Valentini, G, Gurman, Ab, Braun-Moscovici, Y, Mt, Li, Muller-Ladner, U, Allanore, Y EUSTAR co-authors: Serena Guiducci, Alan, Tyndall, Giovanni, Lapadula, Florenzo, Iannone, Radim, Becvar, Stanislaw, Sierakowsky, Otylia Kowal Bielecka, Maurizio, Cutolo, Alberto, Sulli, Cuomo, Giovanna, Vettori, Serena, Simona, Rednic, Ileana, Nicoara, Vlachoyiannopoulos, P, Montecucco, C, Roberto, Caporali, Srdan, Novak, László, Czirják, Cecilia, Varju, Carlo, Chizzolini, Eugene, J Kucharz, Anna, Kotulska, Magdalena, Kopec-Medrek, Malgorzata, Widuchowska, Blaz, Rozman, Carmel, Mallia, Bernard, Coleiro, Armando, Gabrielli, Dominique, Farge, Adrian, Hij, Roger, Hesselstrand, Agneta, Scheja, Frank, Wollheim, Duska, Martinovic, Govoni, M, Andrea Lo Monaco, Nicolas, Hunzelmann, Raffaele, Pellerito, Lisa Maria Bambara, Paola, Caramaschi, Carol, Black, Christopher, Denton, Jörg, Hene, Vera Ortiz Santamaria, Stefan, Heitmann, Dorota, Krasowska, Matthias, Seidel, Mara, Oleszowsky, Harald, Burkhardt, Andrea, Himsel, Maria, J Salvador, Bojana, Stamenkovic, Aleksandra, Stankovic, Mohammed, Tikly, Maya, N Starovoytova, Merete, Engelhart, Gitte, Strau, Henrik, Nielsen, Kirsten, Damgaard, Gabriella, Szüc, Antonio Zea Mendoza, Carlos de la Puente Buijdos, Walter, A Sifuentes Giraldo, Øyvind, Midtvedt, Torhild, Garen, David, Launay, Guido, Valesini, Valeria, Riccieri, Ruxandra Maria Ionescu, Daniela, Opri, Laura, Groseanu, Fredrick, M Wigley, Carmen, M Mihai, Roxana Sfrent Cornateanu, Razvan, Ionitescu, Ana Maria Gherghe, Marilena, Gorga, Rucsandra, Dobrota, Mihai, Bojinca, Georg, Schett, Jörg Hw Distler, Pierluigi, Meroni, Silvana, Zeni, Luc, Mouthon, Filip De Keyser, Vanessa, Smith, Francesco, P Cantatore, Ada, Corrado, Susanne, Ullman, Line, Iversen, Maria, R Pozzi, Kilian, Eyerich, Rüdiger, Hein, Elisabeth, Knott, Jacek, Szechinski, Piotr, Wiland, Magdalena, Szmyrka-Kaczmarek, Renata, Sokolik, Ewa, Morgiel, Brigitte, Krummel-Lorenz, Petra, Saar, Martin, Aringer, Claudia, Günther, Branimir, Anic, Marko, Baresic, Miroslav, Mayer, Sebastião, C Radominski, Carolina de Souza Müller, Valderílio, F Azevedo, Svetlana, Agachi, Liliana, Groppa, Lealea, Chiaburu, Eugen, Russu, Thierry, Zenone, Simon, Stebbing, John, Highton, Lisa, Stamp, Peter, Chapman, Murray, Baron, John, O'Donnell, Kamal, Solanki, Alan, Doube, Douglas, Veale, Marie, O'Rourke, Esthela, Loyo, Edoardo, Rosato, Simonetta, Pisarri, Cristina-Mihaela, Tanaseanu, Monica, Popescu, Alina, Dumitrascu, Isabela, Tiglea, Rodica, Chirieac, Codrina, Ancuta, Daniel, E Furst, Suzanne, Kafaja, Paloma García de la Peña Lefebvre, Silvia Rodriguez Rubio, Marta Valero Exposito, Jean, Sibilia, Emmanuel, Chatelu, Jacques Eric Gottenberg, Hélène, Chifflot, Ira, Litinsky, Algirdas, Venali, Irena, Butrimiene, Paulius, Venali, Rita, Rugiene, Diana, Karpec, Eduardo, Kerzberg, Fabiana, Montoya, Vanesa, Cosentino, and Chizzolini, Carlo

Subjects
0301 basic medicine, Male, heart dysfunction, Databases, Factual, Epidemiology, autoimmune diseases, epidemiology, systemic sclerosis, Kaplan-Meier Estimate, 0302 clinical medicine, Cardiovascular Disease, Immunology and Allergy, Prospective Studies, Age of Onset, skin and connective tissue diseases, Prospective cohort study, ddc:616, Orvostudományok, Middle Aged, Prognosis, Connective tissue disease, 3. Good health, Europe, Cardiovascular Diseases, Cohort, Disease Progression, Female, Autoimmune Diseases, Systemic Sclerosis, Human, Adult, medicine.medical_specialty, Prognosi, Immunology, Socio-culturale, Klinikai orvostudományok, Autoimmune Disease, General Biochemistry, Genetics and Molecular Biology, Follow-Up Studie, 03 medical and health sciences, Sex Factors, Rheumatology, Internal medicine, medicine, Humans, Sex Distribution, Systemic Sclerosi, Aged, 030203 arthritis & rheumatology, Lupus erythematosus, Scleroderma, Systemic, business.industry, medicine.disease, Pulmonary hypertension, Prospective Studie, 030104 developmental biology, Heart failure, Age of onset, business, Follow-Up Studies
Abstract

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

Published
2014

18. Rituximab in the treatment of inflammatory myopathies: a review

Author

Patrick Gordon, Raouf Hajji, David A. Isenberg, Esthela Loyo, and Serena Fasano

Subjects
medicine.medical_specialty, Polymyositis, Gastroenterology, Autoantigens, Dermatomyositis, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Refractory, Internal medicine, Medicine, Humans, Pharmacology (medical), In patient, Myositis, Autoantibodies, 030203 arthritis & rheumatology, business.industry, Significant difference, Autoantibody, medicine.disease, Treatment Outcome, Antibodies, Antinuclear, Antirheumatic Agents, Immunology, Rituximab, business, 030217 neurology & neurosurgery, medicine.drug, Mi-2 Nucleosome Remodeling and Deacetylase Complex
Abstract

Several uncontrolled studies have encouraged the use of rituximab (RTX) in patients with myositis. Unfortunately, the first placebo-phase trial to assess the efficacy of RTX in refractory myositis did not show a significant difference between the two treatment groups, and doubts have been expressed about its study design. In this review we present an up-to-date overview of the reported experiences of RTX therapy in myositis. A PubMed search was performed to find all the available cases of refractory myositis patients treated with RTX up to July 2015. The following terms were assessed: inflammatory myopathies OR anti-synthetase syndrome OR polymyositis OR dermatomyositis AND RTX. A total of 48 studies were included. We identified 458 patients with myositis treated with RTX. We found a rate of response to RTX of 78.3%. RTX can play a role in the management of patients with myositis, at least in those with positive myositis-specific autoantibodies.

Published
2015

19. A comparison between nailfold capillaroscopy patterns in adulthood in juvenile and adult-onset systemic sclerosis: A EUSTAR exploratory study

Author

F. Lauffer, Kati Otsa, Oliver Distler, S. Zeni, Marco Matucci Cerinic, Maria Rosa Pozzi, Margarita Pileckyte, John Highton, Paola Caramaschi, Jacek Szechiński, Maria João Salvador, Diana Karpec, Maurizio Cutolo, Codrina Ancuta, Patrizia Boracchi, Simonetta Pisarri, Fabiana Montoya, Vanessa Smith, Mengtao Li, Carolina de Souza Müller, Patricia Carreira, C. Mihai, Henrik Nielsen, Luc Mouthon, L. Denisov, Marc Frerix, Pier Luigi Meroni, Øyvind Midtvedt, Francesco Paolo Cantatore, Ada Corrado, Sebastião Cezar Radominski, Serena Guiducci, Francesco Puppo, Simon Stebbings, Armando Gabrielli, Giovanna Cuomo, Irena Butrimiene, Piotr Wiland, Ira Litinsky, Maria Uprus, Merete Engelhart, Roger Hesselstrand, Ulrich A Walker, Rodica Chirieac, Ulf Müller-Ladner, David Launay, Kirsten Damgaard, Kamal Solanki, Cristina Mihaela Tanaseanu, Torhild Garen, Isabela Tiglea, Aleksandra Stanković, L. Ananieva, Francesca Ingegnoli, Magdalena Szmyrka-Kaczmarek, Jörg Henes, Alan Tyndall, Roberta Gualtierotti, Rüdiger Hein, Ewa Morgiel, Edoardo Rosato, Ivan Foeldvari, Valderílio Feijó Azevedo, Gitte Strauss, Valeria Riccieri, Anna Kotulska, Marta Valero Exposito, R. Becvar, José António Pereira da Silva, Blaz Rozman, Vera Ortiz-Santamaria, Paloma García de la Peña Lefebvre, Szilvia Szamosi, Małgorzata Widuchowska, Gabriella Szücs, Martin Aringer, Paulius Venalis, Roberto Caporali, Kilian Eyerich, Florenzo Iannone, Alina Dumitrascu, Eugene J. Kucharz, Laura Groseanu, Alessandra Vacca, Monica Popescu, Cristiane Kayser, Yannick Allanore, Brigitte Krummel-Lorenz, P. Saar, Mihai Bojinca, Magdalena Kopec-Medrek, Eduardo Kerzberg, Cecília Varjú, Nemanja Damjanov, Luis Eduardo Coelho Andrade, Rita Rugiene, Paolo Airò, Filip De Keyser, Nicola Ughi, Bojana Stamenkovic, Claudia Günther, Ruxandra Ionescu, László Czirják, Matthias Seidel, Silvia Rodriguez Rubio, Paola Gottschalk, Dirk M. Wuttge, Alan Doube, Vanesa Cosentino, Thierry Zenone, Dominique Farge-Bancel, Esthela Loyo, Algirdas Venalis, Renata Sokolik, Alberto Sulli, Rosario Foti, Stefan Heitmann, Eric Hachulla, Juan José Alegre-Sancho, Carlomaurizio Montecucco, Daniela Opris, Ingegnoli, F, Boracchi, P, Gualtierotti, R, Smith, V, Cutolo, M, Foeldvari, I, Airò, P, Alegre-Sancho, Jj, Allanore, Y, Ananieva, Lp, Ancuta, C, Andrade, Le, Aringer, M, Becvar, R, Bojinca, M, Butrimiene, I, Cantatore, Fp, Caporali, R, Caramaschi, P, Carreira, Pe, Chirieac, R, Corrado, A, Cosentino, V, Cuomo, G, Czirjak, L, Da Silva, Ja, la Peña Lefebvre, Pg, De Keyser, F, de Souza Müller, C, Damgaard, K, Damjanov, N, Denisov, Ln, Distler, O, Doube, A, Dumitrascu, A, Engelhart, M, Exposito, Mv, Eyerich, K, Farge-Bancel, D, Azevedo, Vf, Foti, R, Frerix, M, Gabrielli, A, Garen, T, Gottschalk, P, Groseanu, L, Guiducci, S, Günther, C, Hachulla, Hein, R, Heitmann, S, Henes, J, Hesselstrand, R, Highton, J, Iannone, F, Ionescu, Rm, Kayser, C, Karpec, D, Kerzberg, E, Kotulska, A, Kopec-Medrek, M, Kucharz, E, Krummel-Lorenz, B, Lauffer, F, Launay, D, Li, M, Litinsky, I, Loyo, E, Cerinic, Mm, Meroni, P, Midtvedt, Ø, Mihai, Cm, Montecucco, C, Montoya, F, Morgiel, E, Mouthon, L, Müller-Ladner, U, Nielsen, H, Opris, D, Ortiz-Santamaria, V, Otsa, K, Pileckyte, M, Pisarri, S, Popescu, M, Pozzi, Mr, Puppo, F, Radominski, Sc, Riccieri, V, Rosato, E, Rozman, B, Rubio, Sr, Rugiene, R, Saar, P, Salvador, Mj, Seidel, M, Sokolik, R, Solanki, K, Stamenkovic, B, Stankovic, A, Stebbings, S, Strauss, G, Sulli, A, Szamosi, S, Szechinski, J, Szmyrka-Kaczmarek, M, Szücs, G, Tanaseanu, Cm, Tiglea, I, Tyndall, A, Ughi, N, Uprus, M, Vacca, A, Varju, C, Venalis, A, Venalis, P, Walker, Ua, Widuchowska, M, Wiland, P, Wuttge, Dm, Zeni, S, and Zenone, T.

Subjects
Adult, Male, medicine.medical_specialty, Pathology, Adolescent, Klinikai orvostudományok, Biochemistry, Juvenile systemic sclerosi, Scleroderma, Microscopic Angioscopy, Systemic sclerosi, Scleroderma, Localized, Young Adult, Medicine, Juvenile, Humans, Young adult, Age of Onset, skin and connective tissue diseases, Child, Nailfold Capillaroscopy, Videocapillaroscopy, Aged, Retrospective Studies, EUSTAR, Scleroderma, Systemic, integumentary system, Capillaroscopy, business.industry, Similar distribution, Microcirculation, Autoantibody, Retrospective cohort study, Orvostudományok, Cell Biology, Middle Aged, medicine.disease, Dermatology, Capillaries, Nailfold capillaroscopy, Female, Age of onset, Cardiology and Cardiovascular Medicine, business, Juvenile systemic sclerosis, Systemic sclerosis
Abstract

Objective: Qualitative capillaroscopy patterns in juvenile- and adult-onset systemic sclerosis (SSc) were studied in adulthood using data from the EULAR Scleroderma Trials and Research (EUSTAR) database. Methods: Data collected between June 2004 and April 2013 were examined with focus on capillaroscopy. In this retrospective exploratory study, series of patients with juvenile-onset SSc were matched with series of adult-onset SSc having the same gender and autoantibody profile. Results: 30 of 123 patients with juvenile-onset and 2108 of 7133 with adult-onset SSc had data on capillaroscopy. Juvenile-onset SSc showed scleroderma pattern more frequently than adult-onset SSc (93.3% and 88%). The OR was 2.44 and 95% Cl 0.57-10.41. An active scleroderma pattern was present in 58% of juvenile- and 61% of adult-onset SSc. The OR was 0.91 and 95% Cl 0.28-2.93. The late scleroderma pattern was present in 61% of juvenile- and 55.5% of adult-onset SSc. The OR was 1.06 and 95% Cl 0.34-3.56. Conclusion: This is the first exploratory study on the comparison of capillaroscopy between juvenile- and adult-onset SSc in adulthood. Juvenile-onset SSc had an increase prevalence of sderoderma pattern, but a similar distribution of the three patterns was suggested. Further studies are needed to define this issue. (C) 2015 Elsevier Inc. All rights reserved.

Published
2015

20. Nailfold capillaroscopy in systemic sclerosis: data from the EULAR scleroderma trials and research (EUSTAR) database

Author

Francesca Ingegnoli, Ilaria Ardoino, Patrizia Boracchi, Maurizio Cutolo, Paolo Airò, Lidia P. Ananieva, Codrina Ancuta, Luis Eduardo Andrade, Radim Becvar, Alessia Benenati, Paola Caramaschi, Patricia E. Carreira, Giovanna Cuomo, Nemanja Damjanov, Oliver Distler, Rosario Foti, Serena Guiducci, Eric Hachulla, John Highton, Cristiane Kayser, Chiara Lubatti, Marco Matucci Cerinic, Florian Meier, Pier Luigi Meroni, Carmen Marina Mihai, Ewa Morgiel, Ulf Müller-Ladner, Esthela Loyo, Vera Ortiz, Valeria Riccieri, Agneta Scheja, Simona Skacelova, Vanessa Smith, Bojana Stamenkovic, Simon Stebbings, Viktor Stoica, Alberto Sulli, Jacek Szechinski, Alan Tyndall, Gabriele Valentini, Ulrich Walker, Silvana Zeni, Maja Zlatanovic, Ingegnoli, Francesca, Ardoino, Ilaria, Boracchi, Patrizia, Cutolo, Maurizio, Airã², Paolo, Ananieva, Lidia P., Ancuta, Codrina, Andrade, Luis Eduardo, Becvar, Radim, Benenati, Alessia, Caramaschi, Paola, Carreira, Patricia E., Cuomo, Giovanna, Damjanov, Nemanja, Distler, Oliver, Foti, Rosario, Guiducci, Serena, Hachulla, Eric, Highton, John, Kayser, Cristiane, Lubatti, Chiara, Cerinic, Marco Matucci, Meier, Florian, Meroni, Pier Luigi, Mihai, Carmen Marina, Morgiel, Ewa, Müller Ladner, Ulf, Loyo, Esthela, Ortiz, Vera, Riccieri, Valeria, Scheja, Agneta, Skacelova, Simona, Smith, Vanessa, Stamenkovic, Bojana, Stebbings, Simon, Stoica, Viktor, Sulli, Alberto, Szechinski, Jacek, Tyndall, Alan, Valentini, Gabriele, Walker, Ulrich, Zeni, Silvana, and Zlatanovic, Maja

Subjects
Male, VIDEOCAPILLAROSCOPIC PATTERNS, Databases, Factual, RAYNAUDS-PHENOMENON, systemic sclerosis, International Cooperation, Nailfold videocapillaroscopy, Disease, computer.software_genre, ANTINUCLEAR ANTIBODIES, Biochemistry, Scleroderma, Microscopic Angioscopy, Cohort Studies, Surveys and Questionnaires, Medicine and Health Sciences, Surveys and Questionnaire, Medicine, ORGAN INVOLVEMENT, Nailfold Capillaroscopy, Clinical Trials as Topic, integumentary system, Database, ABNORMALITIES, MICROSCOPY, Middle Aged, Phenotype, Cohort, Disease Progression, Female, Cardiology and Cardiovascular Medicine, Human, Adult, Disease cluster, Nailfold capillaroscopy, Humans, In patient, Aged, Cross-Sectional Studie, Scleroderma, Systemic, business.industry, Cell Biology, medicine.disease, Capillaries, ACTIVITY CRITERIA, Capillarie, Cross-Sectional Studies, SEVERITY, DENSITY, Organ involvement, CONNECTIVE-TISSUE DISEASE, Cohort Studie, business, computer
Abstract

Objective The aims of this study were to obtain cross-sectional data on capillaroscopy in an international multi-center cohort of Systemic Sclerosis (SSc) and to investigate the frequency of the capillaroscopic patterns and their disease-phenotype associations. Methods Data collected between June 2004 and October 2011 in the EULAR Scleroderma Trials and Research (EUSTAR) registry were examined. Patients' profiles based on clinical and laboratory data were obtained by cluster analysis and the association between profiles and capillaroscopy was investigated by multinomial logistic regression. Results 62 of the 110 EUSTAR centers entered data on capillaroscopy in the EUSTAR database. 376 of the 2754 patients (13.65%) were classified as scleroderma pattern absent, but non-specific capillary abnormalities were noted in 55.48% of the cases. Four major patients' profiles were identified characterized by a progressive severity for skin involvement, as well as an increased number of systemic manifestations. The “early” and “active” scleroderma patterns were generally observed in patients with mild/moderate skin involvement and a low number of disease manifestations, while the “late” scleroderma pattern was found more frequently in the more severe forms of the disease. Conclusion These data indicate the importance of capillaroscopy in SSc management and that capillaroscopic patterns are directly related to the extent of organ involvement.

Published
2013

21. Effects and safety of rituximab in systemic sclerosis : An analysis from the European Scleroderma Trial and Research (EUSTAR) group

Author

Veronica Codullo, Thierry Zenone, Ulrich A. Walker, Dominique Farge-Bancel, Jacob M van Laar, Matija Tomšič, Simona Rednic, Francesco Paolo Cantatore, Sule Yavuz, Tore K Kvien, Sergiu Popa, Lidia Ananjeva, Suzana Jordan, Marco Matucci-Cerinic, Gabriela Szucs, Paolo Airò, Paola Caramaschi, Armando Gabrielli, Emmanuel Chatelus, Codrina Michaela Ancuta, Agneta Scheja, Francesca Ingegnoli, Gabriela Riemekasten, Gabriele Valentini, Vanessa Smith, Edoardo Rosato, Esthela Loyo, Martin Aringer, Alexandra Balbir-Gurman, Ulf Müller-Ladner, I. Herrgott, Britta Maurer, Yannick Allanore, Juan José Alegre Sancho, Dörte Huscher, Rosario Foti, Rene Westhovens, Øyvind Palm, Jörg H W Distler, Oliver Distler, M. Saracco, Carina Mihai, Jörg Henes, Florenzo Iannone, Walid Ahmed Abdel Atty Mohamed, Jordan, Suzana, Distler, Jörg H. W., Maurer, Britta, Huscher, Dörte, Van Laar, Jacob M., Allanore, Yannick, Distler, Oliver, Kvien, Tore K., Airo, Paolo, Sancho, Juan José Alegre, Ananjeva, Lidia, Ancuta, Codrina Michaela, Aringer, Martin, Balbir Gurman, Alexandra, Cantatore, Francesco Paolo, Caramaschi, Paola, Chatelus, Emmanuel, Codullo, Veronica, Farge Bancel, Dominique, Foti, Rosario, Gabrielli, Armando, Henes, Jörg, Herrgott, Ilka, Iannone, Florenzo, Ingegnoli, Francesca, Loyo, Esthela, Matucci Cerinić, Marco, Mohamed, Walid Ahmed Abdel Atty, Müller Ladner, Ulf, Palm, Øyvind, Popa, Sergiu, Riemekasten, Gabriela, Rednic, Simona, Rosato, Edoardo, Saracco, Marta, Scheja, Agneta, Smith, Vanessa, Mihai, Carina, Szucs, Gabriela, Tomšić, Matija, Valentini, Gabriele, Walker, Ulrich A., Westhovens, Rene, Yavuz, Sule Kurhan, and Zenone, Thierry

Subjects
Male, Vital capacity, Databases, Factual, Fibrosi, Vital Capacity, Gastroenterology, Severity of Illness Index, Biochemistry, Scleroderma, Cohort Studies, Antibodies, Monoclonal, Murine-Derived, Immunosuppressive Agent, Immunology and Allergy, Lung, Skin, Medicine(all), B cell, integumentary system, Medicine (all), Interstitial lung disease, Antirheumatic Agent, Orvostudományok, Middle Aged, Connective tissue disease, Rheumatoid arthritis, Antirheumatic Agents, Rituximab, Female, Case-Control Studie, Immunosuppressive Agents, medicine.drug, Human, medicine.medical_specialty, Immunology, Observational Study, Klinikai orvostudományok, General Biochemistry, Genetics and Molecular Biology, FEV1/FVC ratio, Rheumatology, Internal medicine, medicine, Journal Article, Humans, Systemic Sclerosi, Scleroderma, Systemic, Biochemistry, Genetics and Molecular Biology (all), business.industry, Biochemistry, Genetics and Molecular Biology(all), medicine.disease, Fibrosis, Treatment, Case-Control Studies, Cohort Studie, business, Lung Diseases, Interstitial, Genetics and Molecular Biology(all)
Abstract

ObjectivesTo assess the effects of Rituximab (RTX) on skin and lung fibrosis in patients with systemic sclerosis (SSc) belonging to the European Scleroderma Trial and Research (EUSTAR) cohort and using a nested case-control design.MethodsInclusion criteria were fulfilment of American College of Rheumatology classification criteria for SSc, treatment with RTX and availability of follow-up data. RTX-treated patients were matched with control patients from the EUSTAR database not treated with RTX. Matching parameters for skin/lung fibrosis were the modified Rodnan Skin Score (mRSS), forced vital capacity (FVC), follow-up duration, scleroderma subtype, disease duration and immunosuppressive co-treatment. The primary analysis was mRSS change from baseline to follow-up in the RTX group compared with the control group. Secondary analyses included change of FVC and safety measures.Results63 patients treated with RTX were included in the analysis. The case-control analysis in patients with severe diffuse SSc showed that mRSS changes were larger in the RTX group versus matched controls (N=25; −24.0±5.2% vs −7.7±4.3%; p=0.03). Moreover, in RTX-treated patients, the mean mRSS was significantly reduced at follow-up compared with baseline (26.6±1.4 vs 20.3±1.8; p=0.0001). In addition, in patients with interstitial lung disease, RTX prevented significantly the further decline of FVC compared with matched controls (N=9; 0.4±4.4% vs −7.7±3.6%; p=0.02). Safety measures showed a good profile consistent with previous studies in autoimmune rheumatic diseases.ConclusionsThe comparison of RTX treated versus untreated matched-control SSc patients from the EUSTAR cohort demonstrated improvement of skin fibrosis and prevention of worsening lung fibrosis, supporting the therapeutic concept of B cell inhibition in SSc.

Published
2015

Catalog

Books, media, physical & digital resources
See catalog results