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1. 2023 Brazilian Society of Rheumatology guidelines for the treatment of systemic sclerosis

Author

Kayser, Cristiane, de Oliveira Delgado, Sandra Maximiano, Zimmermann, Adriana Fontes, Horimoto, Alex Magno Coelho, Del Rio, Ana Paula Toledo, de Souza Müller, Carolina, Camargo, Cintia Zumstein, Lupo, Cristiano Michelini, de Moraes, Daniela Aparecida, Do Rosário E Souza, Eduardo José, Santos, Flávia Patrícia Sena Teixeira, Sekiyama, Juliana Yuri, Lonzetti, Lilian Scussel, de Oliveira Martins, Lucas Victória, Bezerra, Mailze Campos, Bredemeier, Markus, Oliveira, Maria Carolina, da Fonseca Salgado, Maria Cecília, Miossi, Renata, de Araújo Fontenele, Sheila Márcia, Hax, Vanessa, Dantas, Andrea Tavares, and Sampaio-Barros, Percival Degrava

Published
2024
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2. CASE REPORT: IGG4-RELATED DISEASE IN A PATIENT WITH SJÖGREN SYNDROME, PSORIATIC ARTHRITIS AND AUTOIMMUNE HEPATITIS

Author

Bischoff, Laura Marcon, primary, Gonçalves, Luciana Machado Kurtz, additional, Denti, Leonardo André, additional, Pádua, Mateus Camozzato de, additional, Silveira, Letícia Fioravante da, additional, Lopes, Maria Lucia Lemos, additional, Tourinho, Tatiana Freitas, additional, Coradin, Rafael, additional, Lonzetti, Lilian Scussel, additional, and Willers, Thiago, additional

Published
2023
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3. IGG-4 RELATED MIKULICZ SYNDROME MASQUERADING AS LYMPHOCYTIC LYMPHOMA - A CASE REPORT HIGHLIGHTING THE IMPORTANCE OF MEDICAL HISTORY AND PHYSICAL EXAMINATION IN PATIENT DIAGNOSIS

Author

Pádua, Mateus Camozzato de, primary, Silveira, Leticia Fioravante da, additional, Denti, Leonardo André, additional, Gonçalves, Luciana Machado Kurtz, additional, Lopes, Maria Lúcia Lemos, additional, Tourinho, Tatiana Freitas, additional, Lonzetti, Lilian Scussel, additional, Willers, Thiago, additional, Coradin, Rafael, additional, and Soveral, Rodolfo Tome, additional

Published
2023
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5. SURVIVING A CATASTROPHE - A CASE REPORT

Author

Alves, Laura da Silva, primary, Denti, Leonardo André, additional, Silveira, Letícia Fioravante da, additional, Padua, Mateus Camozzato de, additional, Gonçalves, Luciana Machado Kurtz, additional, Willers, Thiago, additional, Tourinho, Tatiana Freitas, additional, Lonzetti, Lilian Scussel, additional, Lopes, Maria Lucia Lemos, additional, and Coradin, Rafael, additional

Published
2023
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6. CASE REPORT: ACUTE LIVER FAILURE IN A YOUNG PATIENT WITH SYSTEMIC LUPUS ERYTHEMATOSUS

Author

Fontanive, Giuliano, primary, Santos, Taina Mafalda dos, additional, Gonçalves, Luciana Machado Kurtz, additional, Denti, Leonardo André, additional, Pádua, Mateus Camozzato de, additional, Tourinho, Tatiana Freitas, additional, Lopes, Maria Lúcia Lemos, additional, Lonzetti, Lilian Scussel, additional, Coradin, Rafael, additional, and Willers, Thiago, additional

Published
2023
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7. SEVERE AND REFRACTORY PYODERMA GANGRENOSUM IN A PATIENT WITH BEHCET S DISEASE: SATISFACTORY RESPONSE TO CYCLOPHOSPHAMIDE THERAPY

Author

Denti, Leonardo André, primary, Gonçalves, Luciana Machado Kurtz, additional, Silveira, Leticia Fioravante da, additional, Padua, Mateus Camozzato de, additional, Lopes, Maria Lúcia Lemos, additional, Tourinho, Tatiana Freitas, additional, Duquia, Rodrigo, additional, Bonamigo, Renan Rangel, additional, Lonzetti, Lilian Scussel, additional, Willers, Thiago, additional, Coradin, Rafael, additional, and Saldanha, Carla Forgiarini, additional

Published
2023
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10. 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

11. CORRELATION BETWEEN DIGITAL ULCERS AND SSA ANTIBODIES IN SYSTEMIC SCLEROSIS PATIENTS: A PRELIMINARY STUDY.

Author

WILLERS, THIAGO, additional, LONZETTI, LILIAN SCUSSEL, additional, TOURINHO, TATIANA FREITAS, additional, LOPES, MARIA LÚCIA LEMOS, additional, HILÁRIO, MARIA ODETE ESTEVES, additional, SCHNEIDER, MATHEUS HENRIQUE, additional, CORADIN, RAFAEL, additional, SCANAGATTA, GILBERTO, additional, FIGUEIREDO, RAISSA VELASQUES DE, additional, and CARLOS, LUANA RIBEIRO, additional

Published
2019
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12. 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

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