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

Author

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

Published
2015
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2. Prediction of improvement in skin fibrosis in diffuse cutaneous systemic sclerosis: a EUSTAR analysis

Author

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

Published
2016
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3. ELISA, protein immunoprecipitation and line blot assays for anti-TIF1-gamma autoantibody detection in cancer-associated dermatomyositis

Author

Selickaja, Sandra, Galindo-Feria, Angeles S., Dani, Lara, Mimori, Tsuneyo, Rönnelid, Johan, Holmqvist, Marie, Lundberg, Ingrid E., Venalis, Paulius, Selickaja, Sandra, Galindo-Feria, Angeles S., Dani, Lara, Mimori, Tsuneyo, Rönnelid, Johan, Holmqvist, Marie, Lundberg, Ingrid E., and Venalis, Paulius

Abstract

Objectives Anti-TIF1-gamma autoantibodies can be detected with immunoprecipitation (IP), line blot (LB) and ELISA. We compared assay performance in patients with DM and the potential of these assays to detect anti-TIF1-gamma positive cancer-associated DM (CADM). Methods We included sera from 131 patients with DM followed at Karolinska University Hospital, Stockholm, Sweden and 82 healthy controls. Serum samples taken at DM diagnosis were tested for anti-TIF1-gamma autoantibodies with IP, two ELISAs (in-house and commercial) and LB. Cancer diagnosis and dates were obtained from the Swedish national cancer register. CADM was defined as a malignancy that developed within 3 years of DM diagnosis. Results Anti-TIF1-gamma autoantibodies were detected in 19/101 (18.8%), 15/113 (13.2%), 34/131 (26%) and 45/131 (34.4%) of the patients with IP, LB, in-house and commercial ELISA, respectively. The anti-TIF1-gamma results from the in-house ELISA were confirmed with IP in 93 of 101 (92%) cases, kappa = 0.76, with a commercial ELISA in 110 of 131 (84%) cases, kappa = 0.63, and with LB in 101 of 113 (89.3%) cases, kappa = 0.67. Anti-TIF1-gamma results with IP were confirmed with LB in 85 of 92 (92.4%) cases, kappa = 0.73. For detecting CADM, the anti-TIF1-gamma in-house ELISA had a sensitivity of 58% and specificity of 86%, the commercial ELISA had a sensitivity of 63% and specificity of 82%, IP had a sensitivity of 52% and specificity of 92%, LB had a sensitivity of 40% and specificity of 96%. Conclusion The two anti-TIF1-gamma ELISA assays had advantages both for autoantibody detection and to identify anti-TIF1-gamma-positive CADM.

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

Author

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

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

Author

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

Published
2015
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8. Cardiomyopathy in Murine Models of Systemic Sclerosis

Author

Venalis, Paulius, Kumánovics, Gábor, Schulze-Koops, Hendrik, Distler, Alfiya, Dees, Clara, Zerr, Pawel, Palumbo-Zerr, Katrin, Czirják, László, Mackevic, Zygmunt, Lundberg, Ingrid E., Distler, Oliver, Schett, Georg, and Distler, Jörg H. W.

Published
2015
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16. Phenotypes determined by cluster analysis and their survival in the prospective European Scleroderma Trials and Research cohort of patients with systemic sclerosis

Author

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

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

OBJECTIVE: Systemic sclerosis (SSc) is a heterogeneous connective tissue disease that is typically subdivided into limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) depending on the extent of skin involvement. This subclassification may not capture the entire variability of clinical phenotypes. The European Scleroderma Trials and Research (EUSTAR) database includes data on a prospective cohort of SSc patients from 122 European referral centers. This study was undertaken to perform a cluster analysis of EUSTAR data to distinguish and characterize homogeneous phenotypes without any a priori assumptions, and to examine survival among the clusters obtained. METHODS: A total of 11,318 patients were registered in the EUSTAR database, and 6,927 were included in the study. Twenty-four clinical and serologic variables were used for clustering. RESULTS: Clustering analyses provided a first delineation of 2 clusters showing moderate stability. In an exploratory attempt, we further characterized 6 homogeneous groups that differed with regard to their clinical features, autoantibody profile, and mortality. Some groups resembled usual dcSSc or lcSSc prototypes, but others exhibited unique features, such as a majority of lcSSc patients with a high rate of visceral damage and antitopoisomerase antibodies. Prognosis varied among groups and the presence of organ damage markedly impacted survival regardless of cutaneous involvement. CONCLUSION: Our findings suggest that restricting subsets of SSc patients to only those based on cutaneous involvement may not capture the complete heterogeneity of the disease. Organ damage and antibody profile should be taken into consideration when individuating homogeneous groups of patients with a distinct prognosis. ispartof: ARTHRITIS & RHEUMATOLOGY vol:71 issue:9 pages:1553-1570 ispartof: location:United States status: published

Published
2019

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

Author

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

Abstract

Objective To assess the safety and efficacy of rituximab in systemic sclerosis (SSc) in clinical practice. Methods We performed a prospective study including patients with SSc from the European Scleroderma Trials and Research (EUSTAR) network treated with rituximab and matched with untreated patients with SSc. The main outcomes measures were adverse events, skin fibrosis improvement, lung fibrosis worsening and steroids use among propensity score-matched patients treated or not with rituximab. Results 254 patients were treated with rituximab, in 58% for lung and in 32% for skin involvement. After a median follow-up of 2 years, about 70% of the patients had no side effect. Comparison of treated patients with 9575 propensity-score matched patients showed that patients treated with rituximab were more likely to have skin fibrosis improvement (22.7 vs 14.03 events per 100 person-years; OR: 2.79 [1.47-5.32]; p=0.002). Treated patients did not have significantly different rates of decrease in forced vital capacity (FVC)>10% (OR: 1.03 [0.55-1.94]; p=0.93) nor in carbon monoxide diffusing capacity (DLCO) decrease. Patients having received rituximab were more prone to stop or decrease steroids (OR: 2.34 [1.56-3.53], p<0.0001). Patients treated concomitantly with mycophenolate mofetil had a trend for better outcomes as compared with patients receiving rituximab alone (delta FVC: 5.22 [0.83-9.62]; p=0.019 as compared with controls vs 3 [0.66-5.35]; p=0.012). Conclusion Rituximab use was associated with a good safety profile in this large SSc-cohort. Significant change was observed on skin fibrosis, but not on lung. However, the limitation is the observational design. The potential stabilisation of lung fibrosis by rituximab has to be addressed by a randomised trial.

Published
2019

19. CD4+and CD8+CD28(null) T Cells Are Cytotoxic to Autologous Muscle Cells in Patients With Polymyositis

Author

Pandya, Jayesh M., Venalis, Paulius, Al-Khalili, Lubna, Hossain, Mohammad Shahadat, Stache, Vanessa, Lundberg, Ingrid E., Malmström, Vivianne, Fasth, Andreas E. R., Pandya, Jayesh M., Venalis, Paulius, Al-Khalili, Lubna, Hossain, Mohammad Shahadat, Stache, Vanessa, Lundberg, Ingrid E., Malmström, Vivianne, and Fasth, Andreas E. R.

Abstract

Objective. Inflammatory T cell infiltrates in the skeletal muscle tissue of patients with polymyositis are dominated by CD28-negative effector (CD28(null)) T cells of both the CD4 and CD8 lineage. These cells are potentially cytotoxic, and the aim of the present study was to develop a fully autologous cell culture system in which to investigate the functional contribution of such CD28(null) T cells to myotoxicity. Methods. In vitro cocultures of autologous skeletal muscle cells and T cell subsets obtained from 5 polymyositis patients were performed. Myotoxicity of T cells was quantified by calcein release and flow cytometric analyses. T cell degranulation was blocked with concanamycin A. Specific blocking of perforin, cytokines, and HLA was performed using antibodies. Results. Both CD4+CD28(null) and CD8+CD28(null) T cells induced more muscle cell death than did their CD28+ counterparts. Differentiated muscle cells (myotubes) were more sensitive to T cell-mediated cell death than were their precursors (myoblasts). Both CD8+ and CD4+ CD28(null) T cells displayed perforin polarization toward muscle cells and secreted higher levels of granzyme B and interferon-gamma (IFN gamma) in coculture than did CD28+ T cells. The myotoxic effects of CD28(null) T cells were reduced upon the blocking of perforin, cytokines, and HLA. Addition of IFN gamma or tumor necrosis factor did not induce skeletal muscle cell death in the absence of T cells; however, it did up-regulate HLA expression on muscle cells. Conclusion. Myotoxicity of CD4+ and CD8+ CD28(null) T cells is mediated by directed perforin-dependent killing and can be further influenced by IFN gamma-induced HLA expression on muscle cells. The data suggest that CD28(null) T cells are key effector cells that contribute to the muscle cell damage in polymyositis., QC 20161020

Published
2016
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21. Antifibrozinių priemonių paieška preklinikiniuose sisteminės sklerozės modeliuose

Author

Venalis, Paulius, Butrimienė, Irena, Rugienė, Rita, Valančiūtė, Angelė, Lesauskaitė, Vaiva, Ivanovienė, Laima, Mackevič, Zygmunt, Gedminienė, Genovaitė, Dadonienė, Jolanta, Distler , Jörg, Vilnius University, and Distler, Jörg

Subjects
TGF-β, Imatinib mesylate, Imatinibo mezilatas, Systemic sclerosis, fibrosis, PDGF, imatinib mesylate, Medicine, Sisteminė sklerozė, Fibrozė, Fibrosis
Abstract

Systemic sclerosis (SSc) – is one of the most complicated and fatal systemic diseases, and the lack of effective therapy is very evident. The tyrosine kinase inhibitor imatinib mesylate (IM) was shown to inhibit TGF-β and PDGF signaling pathways and prevent the development of dermal fibrosis upon challenge with bleomycin in murine model of SSc. The aim of therapy is not only to stop disease progression, but even induce regression of preexisting fibrosis. On other hand, blocking TGF-β and PDGF signaling in angiogenesis might worsen the vascular manifestations of SSc. We found important to evaluate effectiveness of IM for the treatment of pre-established tissue fibrosis and to exclude that the anti-fibrotic effects of IM are complicated by inhibitory effects on endothelial cell functions. Aim of the study: assess the effect of IM on the process of fibrosis and endothelium in experimental models of systemic sclerosis and cell cultures. Objectives of the study: assess the effectiveness of IM on murine models of established fibrosis; evaluate if IM has an effect on basal functions of endothelial cells; assess effect of IM on the process of angiogenesis. We have shown that IM exerts potent antifibrotic effects in two different models of SSc. Imatinib was effective for prevention of fibrosis and for treatment of established dermal fibrosis. We’ve demonstrated that IM does not inhibit major functions of endothelial cells. Thus, IM might not augment further the preexisting vascular... [to full text] Sisteminė sklerozė (SSc) – viena sunkiausių ir fatališkiausių autoimuninių sisteminių reumatinių ligų, o bazinių vaistų stygius, šiai ligai gydyti, itin didelis. Į onkologinę klinikinę praktiką įdiegtas tirozinkinazių inhibitorius – imatinibo mezilatas(IM). IM blokuoja TGF-β ir PDGF intraląstelinio signalo perdavimą ir taip sąlygoja fibrozės prevenciją SSc pelių modelyje. Mums buvo svarbu išsiaiškinti, ar imatinibas gali turėti įtakos ne tik prevencijai, bet ir susiformavusiai fibrozei. Be to TGF-β ir PDGF blokavimas angiogenezėje, galėtų riboti daug žadančio fibrozės inhibitoriaus IM naudojimą gydant SSc. Darbo tikslas: įvertinti imatinibo mezilato poveikį fibrozės procesui ir endoteliui sisteminės sklerozės eksperimentiniuose modeliuose ir ląstelių kultūrose. Darbo uždaviniai: įvertinti imatinibo efektyvumą neuždegiminiame SSc modelyje ir patikrinti imatinibo mezilato efektyvumą uždegiminiame suformuotos fibrozės modelyje; ištirti, ar terapinės imatinibo mezilato koncentracijos daro neigiamą poveikį gyvybinėms endotelio funkcijoms; įvertinti imatinibo mezilato poveikį angiogenezės etapams. Mūsų gauti duomenys rodo, kad: IM ne tik sustabdė bet ir paskatino jau egzistuojančios (bleomicino sukeltos) odos firbrozės regresiją; IM ryškiai sumažino poodžio ir odos storį, bei normalizavo miofibroblastų skaičių Tsk-1 pelėse; IM neturėjo poveikio endotelio ląstelių bazinėms funkcijoms; IM neturėjo neigiamo poveikio angiogenezės etapams.

Published
2010

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

Author

Ingegnoli, Francesca, primary, Boracchi, Patrizia, additional, Gualtierotti, Roberta, additional, Smith, Vanessa, additional, Cutolo, Maurizio, additional, Foeldvari, Ivan, additional, Airò, Paolo, additional, Alegre-Sancho, Juan Jose, additional, Allanore, Yannick, additional, Ananieva, Lidia P., additional, Ancuta, Codrina, additional, Andrade, Luis Eduardo, additional, Aringer, Martin, additional, Becvar, Radim, additional, Bojinca, Mihai, additional, Butrimiene, Irena, additional, Cantatore, Francesco Paolo, additional, Caporali, Roberto, additional, Caramaschi, Paola, additional, Carreira, Patricia E., additional, Chirieac, Rodica, additional, Corrado, Ada, additional, Cosentino, Vanesa, additional, Cuomo, Giovanna, additional, Czirjak, Laszlo, additional, Da Silva, José Antonio Pereira, additional, la Peña Lefebvre, Paloma García de, additional, De Keyser, Filip, additional, de Souza Müller, Carolina, additional, Damgaard, Kirsten, additional, Damjanov, Nemanja, additional, Denisov, Lev N., additional, Distler, Oliver, additional, Doube, Alan, additional, Dumitrascu, Alina, additional, Engelhart, Merete, additional, Exposito, Marta Valero, additional, Eyerich, Kilian, additional, Farge-Bancel, Dominique, additional, Azevedo, Valderílio Feijó, additional, Foti, Rosario, additional, Frerix, Marc, additional, Gabrielli, Armando, additional, Garen, Torhild, additional, Gottschalk, Paola, additional, Groseanu, Laura, additional, Guiducci, Serena, additional, Günther, Claudia, additional, Hachulla, Eric, additional, Hein, Rüdiger, additional, Heitmann, Stefan, additional, Henes, Jörg, additional, Hesselstrand, Roger, additional, Highton, John, additional, Iannone, Florenzo, additional, Ionescu, Ruxandra Maria, additional, Kayser, Cristiane, additional, Karpec, Diana, additional, Kerzberg, Eduardo, additional, Kotulska, Anna, additional, Kopec-Medrek, Magdalena, additional, Kucharz, Eugene, additional, Krummel-Lorenz, Brigitte, additional, Lauffer, Felix, additional, Launay, David, additional, Li, Mengtao, additional, Litinsky, Ira, additional, Loyo, Esthela, additional, Cerinic, Marco Matucci, additional, Meroni, Pierluigi, additional, Midtvedt, Øyvind, additional, Mihai, Carmen Marina, additional, Montecucco, Carlomaurizio, additional, Montoya, Fabiana, additional, Morgiel, Ewa, additional, Mouthon, Luc, additional, Müller-Ladner, Ulf, additional, Nielsen, Henrik, additional, Opris, Daniela, additional, Ortiz-Santamaria, Vera, additional, Otsa, Kati, additional, Pileckyte, Margarita, additional, Pisarri, Simonetta, additional, Popescu, Monica, additional, Pozzi, Maria Rosa, additional, Puppo, Francesco, additional, Radominski, Sebastião Cezar, additional, Riccieri, Valeria, additional, Rosato, Edoardo, additional, Rozman, Blaz, additional, Rubio, Silvia Rodriguez, additional, Rugiene, Rita, additional, Saar, Petra, additional, Salvador, Maria João, additional, Seidel, Matthias, additional, Sokolik, Renata, additional, Solanki, Kamal, additional, Stamenkovic, Bojana, additional, Stankovic, Aleksandra, additional, Stebbings, Simon, additional, Strauss, Gitte, additional, Sulli, Alberto, additional, Szamosi, Szilvia, additional, Szechinski, Jacek, additional, Szmyrka-Kaczmarek, Magdalena, additional, Szücs, Gabriella, additional, Tanaseanu, Cristina-Mihaela, additional, Tiglea, Isabela, additional, Tyndall, Alan, additional, Ughi, Nicola, additional, Uprus, Maria, additional, Vacca, Alessandra, additional, Varju, Cecilia, additional, Venalis, Algirdas, additional, Venalis, Paulius, additional, Walker, Ulrich A., additional, Widuchowska, Malgorzata, additional, Wiland, Piotr, additional, Wuttge, Dirk M., additional, Zeni, Silvana, additional, and Zenone, Thierry, additional

Published
2015
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23. The performance of antifibrotic agents in preclinical models of systemic sclerosis

Author

Venalis, Paulius and Dadonienė, Jolanta

Subjects
systemic sclerosis, fibrosis, TGF-β, PDGF, imatinib mesylate
Abstract

Systemic sclerosis (SSc) – is one of the most complicated and fatal systemic diseases, and the lack of effective therapy is very evident. The tyrosine kinase inhibitor imatinib mesylate (IM) was shown to inhibit TGF-β and PDGF signaling pathways and prevent the development of dermal fibrosis upon challenge with bleomycin in murine model of SSc. The aim of therapy is not only to stop disease progression, but even induce regression of preexisting fibrosis. On other hand, blocking TGF-β and PDGF signaling in angiogenesis might worsen the vascular manifestations of SSc. We found important to evaluate effectiveness of IM for the treatment of pre-established tissue fibrosis and to exclude that the anti-fibrotic effects of IM are complicated by inhibitory effects on endothelial cell functions. Aim of the study: assess the effect of IM on the process of fibrosis and endothelium in experimental models of systemic sclerosis and cell cultures. Objectives of the study: assess the effectiveness of IM on murine models of established fibrosis; evaluate if IM has an effect on basal functions of endothelial cells; assess effect of IM on the process of angiogenesis. We have shown that IM exerts potent antifibrotic effects in two different models of SSc. Imatinib was effective for prevention of fibrosis and for treatment of established dermal fibrosis. We’ve demonstrated that IM does not inhibit major functions of endothelial cells. Thus, IM might not augment further the preexisting vascular damage in SSc.

Published
2010

26. This title is unavailable for guests, please login to see more information.

Author

Akhmetshina, Alfiya, Palumbo, Katrin, Dees, Clara, Bergmann, Christina, Venalis, Paulius, Zerr, Pawel, Horn, Angelika, Kireva, Trayana, Beyer, Christian, Zwerina, Jochen, Schneider, Holm, Sadowski, Anika, Riener, Marc-Oliver, MacDougald, Ormond A, Distler, Oliver, Schett, Georg, Distler, Jörg H W, Akhmetshina, Alfiya, Palumbo, Katrin, Dees, Clara, Bergmann, Christina, Venalis, Paulius, Zerr, Pawel, Horn, Angelika, Kireva, Trayana, Beyer, Christian, Zwerina, Jochen, Schneider, Holm, Sadowski, Anika, Riener, Marc-Oliver, MacDougald, Ormond A, Distler, Oliver, Schett, Georg, and Distler, Jörg H W

Published
2012

27. Antifibrozinių priemonių paieška preklinikiniuose sisteminės sklerozės modeliuose

Author

Butrimienė, Irena, Rugienė, Rita, Valančiūtė, Angelė, Lesauskaitė, Vaiva, Ivanovienė, Laima, Mackevič, Zygmunt, Gedminienė, Genovaitė, Dadonienė, Jolanta, Distler , Jörg, Vilnius University, Venalis, Paulius, Butrimienė, Irena, Rugienė, Rita, Valančiūtė, Angelė, Lesauskaitė, Vaiva, Ivanovienė, Laima, Mackevič, Zygmunt, Gedminienė, Genovaitė, Dadonienė, Jolanta, Distler , Jörg, Vilnius University, and Venalis, Paulius

Abstract

Sisteminė sklerozė (SSc) – viena sunkiausių ir fatališkiausių autoimuninių sisteminių reumatinių ligų, o bazinių vaistų stygius, šiai ligai gydyti, itin didelis. Į onkologinę klinikinę praktiką įdiegtas tirozinkinazių inhibitorius – imatinibo mezilatas(IM). IM blokuoja TGF-β ir PDGF intraląstelinio signalo perdavimą ir taip sąlygoja fibrozės prevenciją SSc pelių modelyje. Mums buvo svarbu išsiaiškinti, ar imatinibas gali turėti įtakos ne tik prevencijai, bet ir susiformavusiai fibrozei. Be to TGF-β ir PDGF blokavimas angiogenezėje, galėtų riboti daug žadančio fibrozės inhibitoriaus IM naudojimą gydant SSc. Darbo tikslas: įvertinti imatinibo mezilato poveikį fibrozės procesui ir endoteliui sisteminės sklerozės eksperimentiniuose modeliuose ir ląstelių kultūrose. Darbo uždaviniai: įvertinti imatinibo efektyvumą neuždegiminiame SSc modelyje ir patikrinti imatinibo mezilato efektyvumą uždegiminiame suformuotos fibrozės modelyje; ištirti, ar terapinės imatinibo mezilato koncentracijos daro neigiamą poveikį gyvybinėms endotelio funkcijoms; įvertinti imatinibo mezilato poveikį angiogenezės etapams. Mūsų gauti duomenys rodo, kad: IM ne tik sustabdė bet ir paskatino jau egzistuojančios (bleomicino sukeltos) odos firbrozės regresiją; IM ryškiai sumažino poodžio ir odos storį, bei normalizavo miofibroblastų skaičių Tsk-1 pelėse; IM neturėjo poveikio endotelio ląstelių bazinėms funkcijoms; IM neturėjo neigiamo poveikio angiogenezės etapams., Systemic sclerosis (SSc) – is one of the most complicated and fatal systemic diseases, and the lack of effective therapy is very evident. The tyrosine kinase inhibitor imatinib mesylate (IM) was shown to inhibit TGF-β and PDGF signaling pathways and prevent the development of dermal fibrosis upon challenge with bleomycin in murine model of SSc. The aim of therapy is not only to stop disease progression, but even induce regression of preexisting fibrosis. On other hand, blocking TGF-β and PDGF signaling in angiogenesis might worsen the vascular manifestations of SSc. We found important to evaluate effectiveness of IM for the treatment of pre-established tissue fibrosis and to exclude that the anti-fibrotic effects of IM are complicated by inhibitory effects on endothelial cell functions. Aim of the study: assess the effect of IM on the process of fibrosis and endothelium in experimental models of systemic sclerosis and cell cultures. Objectives of the study: assess the effectiveness of IM on murine models of established fibrosis; evaluate if IM has an effect on basal functions of endothelial cells; assess effect of IM on the process of angiogenesis. We have shown that IM exerts potent antifibrotic effects in two different models of SSc. Imatinib was effective for prevention of fibrosis and for treatment of established dermal fibrosis. We’ve demonstrated that IM does not inhibit major functions of endothelial cells. Thus, IM might not augment further the preexisting vascular... [to full text]

Published
2010

28. The performance of antifibrotic agents in preclinical models of systemic sclerosis

Author

Butrimienė, Irena, Rugienė, Rita, Valančiūtė, Angelė, Lesauskaitė, Vaiva, Ivanovienė, Laima, Mackevič, Zygmunt, Gedminienė, Genovaitė, Dadonienė, Jolanta, Distler, Jörg, Vilnius University, Venalis, Paulius, Butrimienė, Irena, Rugienė, Rita, Valančiūtė, Angelė, Lesauskaitė, Vaiva, Ivanovienė, Laima, Mackevič, Zygmunt, Gedminienė, Genovaitė, Dadonienė, Jolanta, Distler, Jörg, Vilnius University, and Venalis, Paulius

Abstract

Systemic sclerosis (SSc) – is one of the most complicated and fatal systemic diseases, and the lack of effective therapy is very evident. The tyrosine kinase inhibitor imatinib mesylate (IM) was shown to inhibit TGF-β and PDGF signaling pathways and prevent the development of dermal fibrosis upon challenge with bleomycin in murine model of SSc. The aim of therapy is not only to stop disease progression, but even induce regression of preexisting fibrosis. On other hand, blocking TGF-β and PDGF signaling in angiogenesis might worsen the vascular manifestations of SSc. We found important to evaluate effectiveness of IM for the treatment of pre-established tissue fibrosis and to exclude that the anti-fibrotic effects of IM are complicated by inhibitory effects on endothelial cell functions. Aim of the study: assess the effect of IM on the process of fibrosis and endothelium in experimental models of systemic sclerosis and cell cultures. Objectives of the study: assess the effectiveness of IM on murine models of established fibrosis; evaluate if IM has an effect on basal functions of endothelial cells; assess effect of IM on the process of angiogenesis. We have shown that IM exerts potent antifibrotic effects in two different models of SSc. Imatinib was effective for prevention of fibrosis and for treatment of established dermal fibrosis. We’ve demonstrated that IM does not inhibit major functions of endothelial cells. Thus, IM might not augment further the preexisting vascular... [to full text], Sisteminė sklerozė (SSc) – viena sunkiausių ir fatališkiausių autoimuninių sisteminių reumatinių ligų, o bazinių vaistų stygius, šiai ligai gydyti, itin didelis. Į onkologinę klinikinę praktiką įdiegtas tirozinkinazių inhibitorius – imatinibo mezilatas(IM). IM blokuoja TGF-β ir PDGF intraląstelinio signalo perdavimą ir taip sąlygoja fibrozės prevenciją SSc pelių modelyje. Mums buvo svarbu išsiaiškinti, ar imatinibas gali turėti įtakos ne tik prevencijai, bet ir susiformavusiai fibrozei. Be to TGF-β ir PDGF blokavimas angiogenezėje, galėtų riboti daug žadančio fibrozės inhibitoriaus IM naudojimą gydant SSc. Darbo tikslas: įvertinti imatinibo mezilato poveikį fibrozės procesui ir endoteliui sisteminės sklerozės eksperimentiniuose modeliuose ir ląstelių kultūrose. Darbo uždaviniai: įvertinti imatinibo efektyvumą neuždegiminiame SSc modelyje ir patikrinti imatinibo mezilato efektyvumą uždegiminiame suformuotos fibrozės modelyje; ištirti, ar terapinės imatinibo mezilato koncentracijos daro neigiamą poveikį gyvybinėms endotelio funkcijoms; įvertinti imatinibo mezilato poveikį angiogenezės etapams. Mūsų gauti duomenys rodo, kad: IM ne tik sustabdė bet ir paskatino jau egzistuojančios (bleomicino sukeltos) odos firbrozės regresiją; IM ryškiai sumažino poodžio ir odos storį, bei normalizavo miofibroblastų skaičių Tsk-1 pelėse; IM neturėjo poveikio endotelio ląstelių bazinėms funkcijoms; IM neturėjo neigiamo poveikio angiogenezės etapams.

Published
2010

29. CD4+ and CD8+ CD28null T Cells Are Cytotoxic to Autologous Muscle Cells in Patients With Polymyositis.

Author

Pandya, Jayesh M., Venalis, Paulius, Al‐Khalili, Lubna, Shahadat Hossain, Mohammad, Stache, Vanessa, Lundberg, Ingrid E., Malmström, Vivianne, and Fasth, Andreas E. R.

Subjects
*APOPTOSIS, *BIOPSY, *CELL culture, *FLOW cytometry, *FLUORESCENT antibody technique, *GENE expression, *MICROSCOPY, *POLYMYOSITIS, *RESEARCH funding, *STATISTICS, *T cells, *DATA analysis, *DATA analysis software, *SKELETAL muscle, *CYTOTOXINS, *DESCRIPTIVE statistics, *IN vitro studies, *MANN Whitney U Test
Abstract

Objective Inflammatory T cell infiltrates in the skeletal muscle tissue of patients with polymyositis are dominated by CD28-negative effector (CD28null) T cells of both the CD4 and CD8 lineage. These cells are potentially cytotoxic, and the aim of the present study was to develop a fully autologous cell culture system in which to investigate the functional contribution of such CD28null T cells to myotoxicity. Methods In vitro cocultures of autologous skeletal muscle cells and T cell subsets obtained from 5 polymyositis patients were performed. Myotoxicity of T cells was quantified by calcein release and flow cytometric analyses. T cell degranulation was blocked with concanamycin A. Specific blocking of perforin, cytokines, and HLA was performed using antibodies. Results Both CD4+CD28null and CD8+CD28null T cells induced more muscle cell death than did their CD28+ counterparts. Differentiated muscle cells (myotubes) were more sensitive to T cell-mediated cell death than were their precursors (myoblasts). Both CD8+ and CD4+ CD28null T cells displayed perforin polarization toward muscle cells and secreted higher levels of granzyme B and interferon-γ (IFNγ) in coculture than did CD28+ T cells. The myotoxic effects of CD28null T cells were reduced upon the blocking of perforin, cytokines, and HLA. Addition of IFNγ or tumor necrosis factor did not induce skeletal muscle cell death in the absence of T cells; however, it did up-regulate HLA expression on muscle cells. Conclusion Myotoxicity of CD4+ and CD8+ CD28null T cells is mediated by directed perforin-dependent killing and can be further influenced by IFNγ-induced HLA expression on muscle cells. The data suggest that CD28null T cells are key effector cells that contribute to the muscle cell damage in polymyositis. [ABSTRACT FROM AUTHOR]

Published
2016
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30. Activation of canonical Wnt signalling is required for TGF-β-mediated fibrosis

Author

Akhmetshina, Alfiya, primary, Palumbo, Katrin, additional, Dees, Clara, additional, Bergmann, Christina, additional, Venalis, Paulius, additional, Zerr, Pawel, additional, Horn, Angelika, additional, Kireva, Trayana, additional, Beyer, Christian, additional, Zwerina, Jochen, additional, Schneider, Holm, additional, Sadowski, Anika, additional, Riener, Marc-Oliver, additional, MacDougald, Ormond A., additional, Distler, Oliver, additional, Schett, Georg, additional, and Distler, Jörg H.W., additional

Published
2012
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33. Lack of inhibitory effects of the anti-fibrotic drug imatinib on endothelial cell functionsin vitroandin vivo

Author

Venalis, Paulius, primary, Maurer, Britta, additional, Akhmetshina, Alfiya, additional, Busch, Nicole, additional, Dees, Clara, additional, Stürzl, Michael, additional, Zwerina, Jochen, additional, Jüngel, Astrid, additional, Gay, Steffen, additional, Schett, Georg, additional, Distler, Oliver, additional, and Distler, Jörg H.W., additional

Published
2009
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36. Serum levels of transforming growth factor ß1 (TGF-ß1) in patients with rheumatoid arthritis and Sjögren's syndrome.

Author

Mieliauskaitė, Diana, Venalis, Paulius, Dumalakienė, Irena, Gražienė, Vida, Kirdaitė, Gailutė, and Venalis, Algirdas

Subjects
*SERUM, *RHEUMATOID arthritis, *TRANSFORMING growth factors, *BIOMARKERS, *AUTOIMMUNE diseases, *MEDICAL research
Abstract

Background. Autoimmune diseases, including rheumatoid arthritis (RA) and Sjögren's syndrome, are characterized by loss of immunological tolerance to self-antigens. Transforming growth factor (TGF-β1) is a pleiotropic cytokine that regulates cell growth, adhesion, and differentiation in a wide variety of cell types. Studies of animal models and human patients have revealed a critical function for TGF-β1 in regulating leukocyte functions in autoimmune diseases. Measurement of TGF-β1 is becoming an important screening tool for identifying biomarkers of autoimmune diseases. The purpose of this study was to assess and to compare serum levels of TGF-β1 in patients with rheumatoid arthritis (RA), Sjögren's syndrome secondary to rheumatoid arthritis (sSS + RA) and primary Sjögren's syndrome (pSS). Material and methods. Sera were obtained from 23 patients with RA, 23 patients with sSS + RA, 23 patients with pSS and 20 healthy individuals. Serum levels of TGF-β1 were measured using the respective commercially available BIOSOURCE Immunoassay kit (BioSource International, Inc., USA). Results. Comparison of the serum levels of TGF-β1 in patients with RA, sSS + RA, pSS and healthy individuals showed significant differences of TGF-β1 concentration between four groups (respectively, 51736 ± 12483 pg/ml vs 32953 ± 3090 pg/ml vs 17500 ± 3773 pg/ml and vs 31600 ± 2040 pg/ml, p < 0.001). Conclusion. The appropriate serum levels of TGF-β1 may reflect an ongoing autoimmune inflammation in the different target organs. [ABSTRACT FROM AUTHOR]

Published
2008

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