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53. Autologous haematopoietic stem cell transplantation and other cellular therapy in multiple sclerosis and immune-mediated neurological diseases : updated guidelines and recommendations from the EBMT Autoimmune Diseases Working Party (ADWP) and the Joint Accreditation Committee of EBMT and ISCT (JACIE)

Author

Sharrack, Basil, Saccardi, Riccardo, Alexander, Tobias, Badoglio, Manuela, Burman, Joachim, Farge, Dominique, Greco, Raffaella, Jessop, Helen, Kazmi, Majid, Kirgizov, Kirill, Labopin, Myriam, Mancardi, Gianluigi, Martin, Roland, Moore, John, Muraro, Paolo A., Rovira, Montserrat, Sormani, Maria Pia, Snowden, John A., Snowden, John, McGrath, Eoin, Bambi, Franco, Sanchez-Guijo, Fermin, Worel, Nina, Badolglio, Manuela, Abinun, Mario, Arnold, Renate, Brierley, Charlotte, Castilla-Llorente, Cristina, Cooper, Nichola, Daikeler, Thomas, del Papa, Nicoletta, Finke, Jurgen, Hägglund, Hans, Henes, Joerg, Hiepe, Falk, Kiely, David, Marjanovic, Zora, Martin, Thierry, Ma, David, Miller, Paul, Muraro, Paolo, Oliveira, Maria-Carolina, Polushin, Alexey, Onida, Francesco, Simoes, Belinda, Puyade, Mathieu, Resnick, Igor, Saif, Muhammad, Sakellari, Ioanna, Snarski, Emilian, Scherer, Hans Ulrich, Sossa, Claudia, de Vries-Bouwstra, Jeska, Wulffraat, Nico, Zaccara, Eleanora, Sharrack, Basil, Saccardi, Riccardo, Alexander, Tobias, Badoglio, Manuela, Burman, Joachim, Farge, Dominique, Greco, Raffaella, Jessop, Helen, Kazmi, Majid, Kirgizov, Kirill, Labopin, Myriam, Mancardi, Gianluigi, Martin, Roland, Moore, John, Muraro, Paolo A., Rovira, Montserrat, Sormani, Maria Pia, Snowden, John A., Snowden, John, McGrath, Eoin, Bambi, Franco, Sanchez-Guijo, Fermin, Worel, Nina, Badolglio, Manuela, Abinun, Mario, Arnold, Renate, Brierley, Charlotte, Castilla-Llorente, Cristina, Cooper, Nichola, Daikeler, Thomas, del Papa, Nicoletta, Finke, Jurgen, Hägglund, Hans, Henes, Joerg, Hiepe, Falk, Kiely, David, Marjanovic, Zora, Martin, Thierry, Ma, David, Miller, Paul, Muraro, Paolo, Oliveira, Maria-Carolina, Polushin, Alexey, Onida, Francesco, Simoes, Belinda, Puyade, Mathieu, Resnick, Igor, Saif, Muhammad, Sakellari, Ioanna, Snarski, Emilian, Scherer, Hans Ulrich, Sossa, Claudia, de Vries-Bouwstra, Jeska, Wulffraat, Nico, and Zaccara, Eleanora

Abstract

These updated EBMT guidelines review the clinical evidence, registry activity and mechanisms of action of haematopoietic stem cell transplantation (HSCT) in multiple sclerosis (MS) and other immune-mediated neurological diseases and provide recommendations for patient selection, transplant technique, follow-up and future development. The major focus is on autologous HSCT (aHSCT), used in MS for over two decades and currently the fastest growing indication for this treatment in Europe, with increasing evidence to support its use in highly active relapsing remitting MS failing to respond to disease modifying therapies. aHSCT may have a potential role in the treatment of the progressive forms of MS with a significant inflammatory component and other immune-mediated neurological diseases, including chronic inflammatory demyelinating polyneuropathy, neuromyelitis optica, myasthenia gravis and stiff person syndrome. Allogeneic HSCT should only be considered where potential risks are justified. Compared with other immunomodulatory treatments, HSCT is associated with greater short-term risks and requires close interspeciality collaboration between transplant physicians and neurologists with a special interest in these neurological conditions before, during and after treatment in accredited HSCT centres. Other experimental cell therapies are developmental for these diseases and patients should only be treated on clinical trials.

Published
2020
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54. Belimumab may decrease flare rate and allow glucocorticoid withdrawal in lupus nephritis (including dialysis and transplanted patient)

Author

Binda, V, Trezzi, B, Del Papa, N, Beretta, L, Frontini, G, Porata, G, Fabbrini, P, Pozzi, M, Messa, P, Sinico, R, Moroni, G, Binda, Valentina, Trezzi, Barbara, Del Papa, Nicoletta, Beretta, Lorenzo, Frontini, Giulia, Porata, Giulia, Fabbrini, Paolo, Pozzi, Maria Rosa, Messa, Piergiorgio, Sinico, Renato Alberto, Moroni, Gabriella, Binda, V, Trezzi, B, Del Papa, N, Beretta, L, Frontini, G, Porata, G, Fabbrini, P, Pozzi, M, Messa, P, Sinico, R, Moroni, G, Binda, Valentina, Trezzi, Barbara, Del Papa, Nicoletta, Beretta, Lorenzo, Frontini, Giulia, Porata, Giulia, Fabbrini, Paolo, Pozzi, Maria Rosa, Messa, Piergiorgio, Sinico, Renato Alberto, and Moroni, Gabriella

Abstract

Background: Belimumab (Benlysta) is currently approved for the treatment of active Lupus despite standard therapy. Few data are available on the efficacy of this drug in lupus nephritis (LN). Methods: 17 LN female followed in two Nephrology Italian Unit received belimumab for a median period of 36 months (range 6–42 months). The indications were: arthralgia in 3 patients, cutaneous manifestations in 2, residual proteinuria in 8, and the need to reduce steroids for severe side effects in 4. Of interest, 1 patient started therapy during Peritoneal Dialysis and continued after kidney transplantation due to non-responsive arthralgias. Results: Arthralgia and skin manifestations resolved in all patients. Proteinuria normalized in three patients and stabilized in all but one of the others. Steroids were indefinitely stopped in six patients (35%) and reduced to around 40% of the basal dosage in the other patients. During belimumab therapy, three extrarenal and one renal SLE flares were diagnosed accounting for a rate of renal flares of 0.02/patient/year. No major adverse events leading to therapy withdrawal occurred. Clinical case: Arthralgia resolved, immunological parameters improved and prednisone could be reduced within few months in the patient who started belimumab during peritoneal dialysis. After kidney transplantation belimumab was stopped but due to arthralgias unresponsive to standard immunosuppressive therapy it was restarted with success. Conclusions: Belimumab allows the achievement of complete response together with the withdrawal or the reduction of corticosteroids in almost all our patients. Of interest its satisfactory use in a patient in peritoneal dialysis and after kidney transplantation.

Published
2020

56. The Model for Early COvid-19 Recognition (MECOR) Score: A Proof-of-Concept for a Simple and Low-Cost Tool to Recognize a Possible Viral Etiology in Community-Acquired Pneumonia Patients during COVID-19 Outbreak

Author

Sambataro, Gianluca, primary, Giuffrè, Mauro, additional, Sambataro, Domenico, additional, Palermo, Andrea, additional, Vignigni, Giovanna, additional, Cesareo, Roberto, additional, Crimi, Nunzio, additional, Torrisi, Sebastiano Emanuele, additional, Vancheri, Carlo, additional, Malatino, Lorenzo, additional, Colaci, Michele, additional, Del Papa, Nicoletta, additional, Pignataro, Francesca, additional, Roman-Pognuz, Erik, additional, Fabbiani, Massimiliano, additional, Montagnani, Francesca, additional, Cassol, Chiara, additional, Cavagna, Lorenzo, additional, Zuccaro, Valentina, additional, Zerbato, Verena, additional, Maurel, Cristina, additional, Luzzati, Roberto, additional, and Di Bella, Stefano, additional

Published
2020
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59. Nailfold Videocapillaroscopy Is a Useful Tool to Recognize Definite Forms of Systemic Sclerosis and Idiopathic Inflammatory Myositis in Interstitial Lung Disease Patients

Author

Sambataro, Domenico, primary, Sambataro, Gianluca, additional, Libra, Alessandro, additional, Vignigni, Giovanna, additional, Pino, Fabio, additional, Fagone, Evelina, additional, Fruciano, Mary, additional, Gili, Elisa, additional, Pignataro, Francesca, additional, Del Papa, Nicoletta, additional, and Vancheri, Carlo, additional

Published
2020
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61. Patients with Interstitial Lung Disease Secondary to Autoimmune Diseases: How to Recognize Them?

Author

Sambataro, Domenico, primary, Sambataro, Gianluca, additional, Pignataro, Francesca, additional, Zanframundo, Giovanni, additional, Codullo, Veronica, additional, Fagone, Evelina, additional, Martorana, Emanuele, additional, Ferro, Francesco, additional, Orlandi, Martina, additional, Del Papa, Nicoletta, additional, Cavagna, Lorenzo, additional, Malatino, Lorenzo, additional, Colaci, Michele, additional, and Vancheri, Carlo, additional

Published
2020
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63. Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course

Author

Cavagna, Lorenzo, primary, Trallero-Araguás, Ernesto, additional, Meloni, Federica, additional, Cavazzana, Ilaria, additional, Rojas-Serrano, Jorge, additional, Feist, Eugen, additional, Zanframundo, Giovanni, additional, Morandi, Valentina, additional, Meyer, Alain, additional, Pereira da Silva, Jose, additional, Matos Costa, Carlo, additional, Molberg, Oyvind, additional, Andersson, Helena, additional, Codullo, Veronica, additional, Mosca, Marta, additional, Barsotti, Simone, additional, Neri, Rossella, additional, Scirè, Carlo, additional, Govoni, Marcello, additional, Furini, Federica, additional, Lopez-Longo, Francisco, additional, Martinez-Barrio, Julia, additional, Schneider, Udo, additional, Lorenz, Hanns-Martin, additional, Doria, Andrea, additional, Ghirardello, Anna, additional, Ortego-Centeno, Norberto, additional, Confalonieri, Marco, additional, Tomietto, Paola, additional, Pipitone, Nicolò, additional, Rodriguez Cambron, Ana, additional, Blázquez Cañamero, María, additional, Voll, Reinhard, additional, Wendel, Sarah, additional, Scarpato, Salvatore, additional, Maurier, Francois, additional, Limonta, Massimiliano, additional, Colombelli, Paolo, additional, Giannini, Margherita, additional, Geny, Bernard, additional, Arrigoni, Eugenio, additional, Bravi, Elena, additional, Migliorini, Paola, additional, Mathieu, Alessandro, additional, Piga, Matteo, additional, Drott, Ulrich, additional, Delbrueck, Christiane, additional, Bauhammer, Jutta, additional, Cagnotto, Giovanni, additional, Vancheri, Carlo, additional, Sambataro, Gianluca, additional, De Langhe, Ellen, additional, Sainaghi, Pier, additional, Monti, Cristina, additional, Gigli Berzolari, Francesca, additional, Romano, Mariaeva, additional, Bonella, Francesco, additional, Specker, Christof, additional, Schwarting, Andreas, additional, Villa Blanco, Ignacio, additional, Selmi, Carlo, additional, Ceribelli, Angela, additional, Nuno, Laura, additional, Mera-Varela, Antonio, additional, Perez Gomez, Nair, additional, Fusaro, Enrico, additional, Parisi, Simone, additional, Sinigaglia, Luigi, additional, Del Papa, Nicoletta, additional, Benucci, Maurizio, additional, Cimmino, Marco, additional, Riccieri, Valeria, additional, Conti, Fabrizio, additional, Sebastiani, Gian, additional, Iuliano, Annamaria, additional, Emmi, Giacomo, additional, Cammelli, Daniele, additional, Sebastiani, Marco, additional, Manfredi, Andreina, additional, Bachiller-Corral, Javier, additional, Sifuentes Giraldo, Walter, additional, Paolazzi, Giuseppe, additional, Saketkoo, Lesley, additional, Giorgi, Roberto, additional, Salaffi, Fausto, additional, Cifrian, Jose, additional, Caporali, Roberto, additional, Locatelli, Francesco, additional, Marchioni, Enrico, additional, Pesci, Alberto, additional, Dei, Giulia, additional, Pozzi, Maria, additional, Claudia, Lomater, additional, Distler, Jorg, additional, Knitza, Johannes, additional, Schett, George, additional, Iannone, Florenzo, additional, Fornaro, Marco, additional, Franceschini, Franco, additional, Quartuccio, Luca, additional, Gerli, Roberto, additional, Bartoloni, Elena, additional, Bellando Randone, Silvia, additional, Zampogna, Giuseppe, additional, Gonzalez Perez, Montserrat, additional, Mejia, Mayra, additional, Vicente, Esther, additional, Triantafyllias, Konstantinos, additional, Lopez-Mejias, Raquel, additional, Matucci-Cerinic, Marco, additional, Selva-O’Callaghan, Albert, additional, Castañeda, Santos, additional, Montecucco, Carlomaurizio, additional, and Gonzalez-Gay, Miguel, additional

Published
2019
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65. Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course

Author

Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrián, José Manuel, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I., Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, González-Gay, Miguel A., Universitat Autònoma de Barcelona, Universidad de Cantabria, Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, and Gonzalez-Gay, M

Subjects
medicine.medical_specialty, antisynthetase antibodies, antisynthetase syndrome, arthritis, interstitial lung disease, myositis, Medizin, Arthritis, lcsh:Medicine, Antisynthetase syndrome, Interstitial lung disease, Article, NO, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, medicine, antisynthetase antibodies, antisynthetase syndrome, arthritis, interstitial lung disease, myositis, ddc:610, Myositis, 030203 arthritis & rheumatology, Antisynthetase antibodies, biology, business.industry, lcsh:R, Autoantibody, General Medicine, medicine.disease, arthriti, 030228 respiratory system, Time course, Cohort, biology.protein, Antibody, business, antisynthetase antibodie
Abstract

Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies that are addressed to different aminoacyl tRNA synthetases (ARS). Until now, it has been unknown whether the presence of a different ARS might affect the clinical presentation, evolution, and outcome of ASSD. In this study, we retrospectively recorded the time of onset, characteristics, clustering of triad findings, and survival of 828 ASSD patients (593 anti-Jo1, 95 anti-PL7, 84 anti-PL12, 38 anti-EJ, and 18 anti-OJ), referring to AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group's cohort. Comparisons were performed first between all ARS cases and then, in the case of significance, while using anti-Jo1 positive patients as the reference group. The characteristics of triad findings were similar and the onset mainly began with a single triad finding in all groups despite some differences in overall prevalence. The "ex-novo" occurrence of triad findings was only reduced in the anti-PL12-positive cohort, however, it occurred in a clinically relevant percentage of patients (30%). Moreover, survival was not influenced by the underlying anti-aminoacyl tRNA synthetase antibodies' positivity, which confirmed that antisynthetase syndrome is a heterogeneous condition and that antibody specificity only partially influences the clinical presentation and evolution of this condition. ispartof: JOURNAL OF CLINICAL MEDICINE vol:8 issue:11 ispartof: location:Switzerland status: published

Published
2019

66. Sjögrenʼs syndrome disease damage index and disease activity index: Scoring systems for the assessment of disease damage and disease activity in Sjögrenʼs syndrome, derived from an analysis of a cohort of Italian patients

Author

Vitali, Claudio, Palombi, Gianluigi, Baldini, Chiara, Benucci, Maurizio, Bombardieri, Stefano, Covelli, Michele, Del Papa, Nicoletta, De Vita, Salvatore, Epis, Oscar, Franceschini, Franco, Gerli, Roberto, Govoni, Marcello, Bongi, Susanna Maddali, Maglione, Wanda, Migliaresi, Sergio, Montecucco, Carlomaurizio, Orefice, Maddalena, Priori, Roberta, Tavoni, Antonio, and Valesini, Guido

Published
2007

67. Bone Marrow Endothelial Progenitors are Defective in Systemic Sclerosis

Author

Del Papa, Nicoletta, Quirici, Nadia, Soligo, Davide, Scavullo, Cinzia, Cortiana, Michela, Borsotti, Chiara, Maglione, Wanda, Comina, Denise P., Vitali, Claudio, Fraticelli, Paolo, Gabrielli, Armando, Cortelezzi, Agostino, and Lambertenghi-Deliliers, Giorgio

Published
2006

71. Influence of Antisynthetase Antibodies Specificities on Antisynthetase Syndrome Clinical Spectrum Time Course

Author

Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, Gonzalez-Gay, M, Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrian, Jose, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I, Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, Gonzalez-Gay, Miguel Angel, Cavagna, L, Trallero-Araguás, E, Meloni, F, Cavazzana, I, Rojas-Serrano, J, Feist, E, Zanframundo, G, Morandi, V, Meyer, A, Pereira da Silva, J, Matos Costa, C, Molberg, O, Andersson, H, Codullo, V, Mosca, M, Barsotti, S, Neri, R, Scirè, C, Govoni, M, Furini, F, Lopez-Longo, F, Martinez-Barrio, J, Schneider, U, Lorenz, H, Doria, A, Ghirardello, A, Ortego-Centeno, N, Confalonieri, M, Tomietto, P, Pipitone, N, Rodriguez Cambron, A, Blázquez Cañamero, M, Voll, R, Wendel, S, Scarpato, S, Maurier, F, Limonta, M, Colombelli, P, Giannini, M, Geny, B, Arrigoni, E, Bravi, E, Migliorini, P, Mathieu, A, Piga, M, Drott, U, Delbrueck, C, Bauhammer, J, Cagnotto, G, Vancheri, C, Sambataro, G, De Langhe, E, Sainaghi, P, Monti, C, Gigli Berzolari, F, Romano, M, Bonella, F, Specker, C, Schwarting, A, Villa Blanco, I, Selmi, C, Ceribelli, A, Nuno, L, Mera-Varela, A, Perez Gomez, N, Fusaro, E, Parisi, S, Sinigaglia, L, Del Papa, N, Benucci, M, Cimmino, M, Riccieri, V, Conti, F, Sebastiani, G, Iuliano, A, Emmi, G, Cammelli, D, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Paolazzi, G, Saketkoo, L, Giorgi, R, Salaffi, F, Cifrian, J, Caporali, R, Locatelli, F, Marchioni, E, Pesci, A, Dei, G, Pozzi, M, Claudia, L, Distler, J, Knitza, J, Schett, G, Iannone, F, Fornaro, M, Franceschini, F, Quartuccio, L, Gerli, R, Bartoloni, E, Bellando Randone, S, Zampogna, G, Gonzalez Perez, M, Mejia, M, Vicente, E, Triantafyllias, K, Lopez-Mejias, R, Matucci-Cerinic, M, Selva-O'Callaghan, A, Castañeda, S, Montecucco, C, Gonzalez-Gay, M, Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Pereira da Silva, Jose Antonio, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo, Govoni, Marcello, Furini, Federica, Lopez-Longo, Francisco Javier, Martinez-Barrio, Julia, Schneider, Udo, Lorenz, Hanns-Martin, Doria, Andrea, Ghirardello, Anna, Ortego-Centeno, Norberto, Confalonieri, Marco, Tomietto, Paola, Pipitone, Nicolò, Rodriguez Cambron, Ana Belen, Blázquez Cañamero, María Ángeles, Voll, Reinhard Edmund, Wendel, Sarah, Scarpato, Salvatore, Maurier, Francois, Limonta, Massimiliano, Colombelli, Paolo, Giannini, Margherita, Geny, Bernard, Arrigoni, Eugenio, Bravi, Elena, Migliorini, Paola, Mathieu, Alessandro, Piga, Matteo, Drott, Ulrich, Delbrueck, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Gigli Berzolari, Francesca, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Perez Gomez, Nair, Fusaro, Enrico, Parisi, Simone, Sinigaglia, Luigi, Del Papa, Nicoletta, Benucci, Maurizio, Cimmino, Marco Amedeo, Riccieri, Valeria, Conti, Fabrizio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Emmi, Giacomo, Cammelli, Daniele, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Paolazzi, Giuseppe, Saketkoo, Lesley Ann, Giorgi, Roberto, Salaffi, Fausto, Cifrian, Jose, Caporali, Roberto, Locatelli, Francesco, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Pozzi, Maria Rosa, Claudia, Lomater, Distler, Jorg, Knitza, Johannes, Schett, George, Iannone, Florenzo, Fornaro, Marco, Franceschini, Franco, Quartuccio, Luca, Gerli, Roberto, Bartoloni, Elena, Bellando Randone, Silvia, Zampogna, Giuseppe, Gonzalez Perez, Montserrat I, Mejia, Mayra, Vicente, Esther, Triantafyllias, Konstantinos, Lopez-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O'Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, and Gonzalez-Gay, Miguel Angel

Abstract

Antisynthetase syndrome (ASSD) is a rare clinical condition that is characterized by the occurrence of a classic clinical triad, encompassing myositis, arthritis, and interstitial lung disease (ILD), along with specific autoantibodies that are addressed to different aminoacyl tRNA synthetases (ARS). Until now, it has been unknown whether the presence of a different ARS might affect the clinical presentation, evolution, and outcome of ASSD. In this study, we retrospectively recorded the time of onset, characteristics, clustering of triad findings, and survival of 828 ASSD patients (593 anti-Jo1, 95 anti-PL7, 84 anti-PL12, 38 anti-EJ, and 18 anti-OJ), referring to AENEAS (American and European NEtwork of Antisynthetase Syndrome) collaborative group's cohort. Comparisons were performed first between all ARS cases and then, in the case of significance, while using anti-Jo1 positive patients as the reference group. The characteristics of triad findings were similar and the onset mainly began with a single triad finding in all groups despite some differences in overall prevalence. The "ex-novo" occurrence of triad findings was only reduced in the anti-PL12-positive cohort, however, it occurred in a clinically relevant percentage of patients (30%). Moreover, survival was not influenced by the underlying anti-aminoacyl tRNA synthetase antibodies' positivity, which confirmed that antisynthetase syndrome is a heterogeneous condition and that antibody specificity only partially influences the clinical presentation and evolution of this condition.

Published
2019

74. List of Contributors

Author

Abramsky, Oded, primary, Abu-Shakra, Mahmoud, additional, Aderka, Dan, additional, Aharoni, Rina, additional, Ansar Ahmed, S., additional, Alarcón-Segovia, Donato, additional, Amit, Micha, additional, Arepally, Gowthami, additional, Arnon, Ruth, additional, Bach, Jean-François, additional, Balestrieri, Genesio, additional, Barak, Vivian, additional, Ben-Chetrit, Eldad, additional, Ben-Nun, Avraham, additional, Blank, Miri, additional, Bombardieri, Stefano, additional, Brégégère, François, additional, Buskila, Dan, additional, Cabral, Antonio R., additional, Carbotte, Ramona M., additional, Cervera, Ricard, additional, Ones, Douglas B., additional, Cohen, Irun R., additional, Cohen Tervaert, J.W., additional, Coppel, Ross L., additional, Davies, Terry F., additional, Deckmann, M., additional, Del Papa, Nicoletta, additional, Della Rossa, A., additional, Denburg, Judah A., additional, Denburg, Susan D., additional, Deutsch, Moti, additional, Ehrenfeld, Michael, additional, Feltkamp, T.E.W., additional, Ferri, C., additional, Finemesser, Maora, additional, Fishman, Pnina, additional, Franitza, Suzanne, additional, Fridkis-Hareli, Masha, additional, Fuchs, Sara, additional, Gerbat, Sunadar, additional, Gershon, Harriet, additional, Eric Gershwin, M., additional, Heeringa, P., additional, Hermann, M., additional, Hughes, Graham R.V., additional, Jamin, C., additional, Kalden, Joachim R., additional, Kallenberg, Cees G.M., additional, Karussis, Demitrius, additional, Kaveri, Srini V., additional, Kaufman, M., additional, Kazatchkine, Michel D., additional, de Rosbo, Nicole Kerlero, additional, Khamashta, Munther A., additional, Kiefer, Hélène, additional, Kita, Masako, additional, Koike, Takao, additional, Kraiem, Zachi, additional, Krause, Ilan, additional, Lahat, Nitza, additional, Langevitz, Pnina, additional, Lerner, Aaron, additional, Leykin, I., additional, Lider, Ofer, additional, Lydyard, Peter M., additional, Matsuura, Eiji, additional, Merimsky, Ofer, additional, Meroni, Pier Luigi, additional, Métézeau, Philippe, additional, Michel, Beno, additional, Migliaccio, Christopher T., additional, Milner, Yoram, additional, Mor, A., additional, Mor, Felix, additional, Moutsopoulos, Haralampos M., additional, Mozes, Edna, additional, Munoz, Santiago, additional, Naparstek, Yaakov, additional, Pers, J.O., additional, Polihronis, Mary, additional, Putterman, Chaim, additional, Rapoport, Micha, additional, Raschi, Elena, additional, Rose, Noel R., additional, Roubey, Robert A.S., additional, Sabbadini, Maria Grazia, additional, Sakic, Boris, additional, Sela, Michael, additional, Sgonc, Roswitha, additional, Shapiro, Sarah, additional, Shinitzky, Meir, additional, Shoenfeld, Yehuda, additional, Sikuler, Emanuel, additional, Slavin, Shimon, additional, Spatola, Laura, additional, David Stollar, B., additional, Szechtman, Henry, additional, Talal, Norman, additional, Tapinos, Nikolaos I., additional, Teitelbaum, Dvora, additional, Tincani, Angela, additional, Tishler, Moshe, additional, Tzioufas, Athanasios G., additional, Van de Water, Judy, additional, Vlase, Horia, additional, Wang, Xin, additional, Wick, Georg, additional, Wiik, Allan, additional, Winkler, Th., additional, Youinou, Pierre, additional, Ziporen, Lea, additional, Zlotkin, Marina, additional, and Zurgil, Naomi, additional

Published
1999
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79. Adipose-derived mesenchymal stromal/stem cells in systemic sclerosis: Alterations in function and beneficial effect on lung fibrosis are regulated by caveolin-1

Author

Lee, Rebecca, primary, Del Papa, Nicoletta, additional, Introna, Martin, additional, Reese, Charles F, additional, Zemskova, Marina, additional, Bonner, Michael, additional, Carmen-Lopez, Gustavo, additional, Helke, Kristi, additional, Hoffman, Stanley, additional, and Tourkina, Elena, additional

Published
2019
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81. Improving Treatment Related Mortality over Time - Data from the Non-Observational Trial on Autologous Stem Cell Transplantation in Systemic Sclerosis By the EBMT Autoimmune Diseases Working Party (ADWP)

Author

Farge, Dominique, primary, Snowden, John A, additional, Badoglio, Manuela, additional, Labopin, Myriam, additional, Scherer, Hans Ulrich, additional, Del Papa, Nicoletta, additional, Kanz, Lothar, additional, Schmalzing, Marc, additional, Martin, Thierry, additional, Rovira, Montserrat, additional, Daikeler, Thomas, additional, Pugnet, Gregory, additional, and Henes, Joerg, additional

Published
2018
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87. Mesenchymal stromal cells from human umbilical cord prevent the development of lung fibrosis in immunocompetent mice

Author

Moroncini, Gianluca, primary, Paolini, Chiara, additional, Orlando, Fiorenza, additional, Capelli, Chiara, additional, Grieco, Antonella, additional, Tonnini, Cecilia, additional, Agarbati, Silvia, additional, Mondini, Eleonora, additional, Saccomanno, Stefania, additional, Goteri, Gaia, additional, Svegliati Baroni, Silvia, additional, Provinciali, Mauro, additional, Introna, Martino, additional, Del Papa, Nicoletta, additional, and Gabrielli, Armando, additional

Published
2018
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88. State of the art in interstitial pneumonia with autoimmune features: a systematic review on retrospective studies and suggestions for further advances

Author

Sambataro, Gianluca, primary, Sambataro, Domenico, additional, Torrisi, Sebastiano Emanuele, additional, Vancheri, Ada, additional, Pavone, Mauro, additional, Rosso, Roberta, additional, Schisano, Matteo, additional, Crimi, Claudia, additional, Pignataro, Francesca, additional, Fischer, Aryeh, additional, Del Papa, Nicoletta, additional, and Vancheri, Carlo, additional

Published
2018
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91. The cumulative number of micro-haemorrhages and micro-thromboses in nailfold videocapillaroscopy is a good indicator of disease activity in systemic sclerosis: a validation study of the NEMO score

Author

Andracco, Romina, primary, Irace, Rosaria, additional, Zaccara, Eleonora, additional, Vettori, Serena, additional, Maglione, Wanda, additional, Riccardi, Antonella, additional, Pignataro, Francesca, additional, Ferrara, Roberta, additional, Sambataro, Domenico, additional, Sambataro, Gianluca, additional, Vitali, Claudio, additional, Valentini, Gabriele, additional, and Del Papa, Nicoletta, additional

Published
2017
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92. Phenotypical and Functional Characteristics of in Vitro-Expanded Adipose-Derived Mesenchymal Stromal Cells from Patients with Systematic Sclerosis

Author

Capelli, Chiara, primary, Zaccara, Eleonora, additional, Cipriani, Paola, additional, Di Benedetto, Paola, additional, Maglione, Wanda, additional, Andracco, Romina, additional, Di Luca, Gabriele, additional, Pignataro, Francesca, additional, Giacomelli, Roberto, additional, Introna, Martino, additional, Vitali, Claudio, additional, and Del Papa, Nicoletta, additional

Published
2017
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94. Autologous Hematopoietic Stem Cell Transplantation vs Intravenous Pulse Cyclophosphamide in Diffuse Cutaneous Systemic Sclerosis

Author

van Laar, Jacob, Farge, Dominique, Sont, Jacob, Naraghi, Kamran, Marjanovic, Zora, Larghero, Jérôme, Schuerwegh, Annemie, Marijt, Erik, Vonk, Madelon, Schattenberg, Anton, Matucci-Cerinic, Marco, Voskuyl, Alexandre, van de Loosdrecht, Arjan, Daikeler, Thomas, Kötter, Ina, Schmalzing, Marc, Martin, Thierry, Lioure, Bruno, Weiner, Stefan, Kreuter, Alexander, Deligny, Christophe, DURAND, Jean-Marc, Emery, Paul, Machold, Klaus, Sarrot-Reynauld, Françoise, Warnatz, Klaus, Adoue, Daniel, Constans, Joël, Tony, Hans-Peter, Del Papa, Nicoletta, Fassas, Athanasios, Himsel, Andrea, Launay, David, Lo Monaco, Andrea, Philippe, Pierre, Quere, Isabelle, Rich, Éric, Westhovens, Rene, Griffiths, Bridget, Saccardi, Riccardo, van den Hoogen, Frank, Fibbe, Willem, Socie, Gérard, Gratwohl, Alois, Tyndall, Alan, Study Group, EBMT/EULAR Scleroderma, Service de Médecine Interne [Saint-Louis], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), CIC - Biotherapie - Saint Louis ((CIC-BT 301)), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], CHU Strasbourg, Département d'Oncologie et Hématologie [Strasbourg], Les Hôpitaux Universitaires de Strasbourg (HUS), Hôpital Pierre Zobda-Quitman [CHU de la Martinique], CHU de la Martinique [Fort de France], Médecine interne et immunologie clinique [Hôpital de la Conception - APHM], Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Hôpital de la Conception [CHU - APHM] (LA CONCEPTION), Clinique de médecine interne, Université Joseph Fourier - Grenoble 1 (UJF)-CHU Grenoble, Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Hôpital Saint-André, Service de médecine interne [Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Clermont-Ferrand, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Caractéristiques féminines des dysfonctions des interfaces cardio-vasculaires (EA 2992), Université Montpellier 1 (UM1)-Université de Montpellier (UM), Hopital Saint-Louis [AP-HP] (AP-HP), Université Paris Diderot - Paris 7 (UPD7)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Service d'hématologie clinique et de thérapie cellulaire [CHU Saint-Antoine], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Hôpital de la Conception [CHU - APHM] (LA CONCEPTION)-Assistance Publique - Hôpitaux de Marseille (APHM)-Aix Marseille Université (AMU), CHU Toulouse [Toulouse], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Université Paris Diderot - Paris 7 (UPD7)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Saint-Antoine [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Université Paris Diderot - Paris 7 (UPD7)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Groupe Hospitalier Saint Louis - Lariboisière - Fernand Widal [Paris], Aix Marseille Université (AMU)-Assistance Publique - Hôpitaux de Marseille (APHM)-Hôpital de la Conception [CHU - APHM] (LA CONCEPTION ), and CHU Saint Louis [APHP]

Subjects
[SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
Abstract

International audience; IMPORTANCE:High-dose immunosuppressive therapy and autologous hematopoietic stem cell transplantation (HSCT) have shown efficacy in systemic sclerosis in phase 1 and small phase 2 trials.OBJECTIVE:To compare efficacy and safety of HSCT vs 12 successive monthly intravenous pulses of cyclophosphamide.DESIGN, SETTING, AND PARTICIPANTS:The Autologous Stem Cell Transplantation International Scleroderma (ASTIS) trial, a phase 3, multicenter, randomized (1:1), open-label, parallel-group, clinical trial conducted in 10 countries at 29 centers with access to a European Group for Blood and Marrow Transplantation-registered transplant facility. From March 2001 to October 2009, 156 patients with early diffuse cutaneous systemic sclerosis were recruited and followed up until October 31, 2013.INTERVENTIONS:HSCT vs intravenous pulse cyclophosphamide.MAIN OUTCOMES AND MEASURES:The primary end point was event-free survival, defined as time from randomization until the occurrence of death or persistent major organ failure.RESULTS:A total of 156 patients were randomly assigned to receive HSCT (n = 79) or cyclophosphamide (n = 77). During a median follow-up of 5.8 years, 53 events occurred: 22 in the HSCT group (19 deaths and 3 irreversible organ failures) and 31 in the control group (23 deaths and 8 irreversible organ failures). During the first year, there were more events in the HSCT group (13 events [16.5%], including 8 treatment-related deaths) than in the control group (8 events [10.4%], with no treatment-related deaths). At 2 years, 14 events (17.7%) had occurred cumulatively in the HSCT group vs 14 events (18.2%) in the control group; at 4 years, 15 events (19%) had occurred cumulatively in the HSCT group vs 20 events (26%) in the control group. Time-varying hazard ratios (modeled with treatment × time interaction) for event-free survival were 0.35 (95% CI, 0.16-0.74) at 2 years and 0.34 (95% CI, 0.16-0.74) at 4 years.CONCLUSIONS AND RELEVANCE:Among patients with early diffuse cutaneous systemic sclerosis, HSCT was associated with increased treatment-related mortality in the first year after treatment. However, HCST conferred a significant long-term event-free survival benefit.TRIAL REGISTRATION:isrctn.org Identifier: ISRCTN54371254.

Published
2014

95. EULAR Sjögren's syndrome disease activity index (ESSDAI) : a user guide

Author

Seror, Raphaèle, Bowman, Simon J, Brito-Zeron, Pilar, Theander, Elke, Bootsma, Hendrika, Tzioufas, Athanasios, Gottenberg, Jacques-Eric, Ramos-Casals, Manel, Dörner, Thomas, Ravaud, Philippe, Vitali, Claudio, Mariette, Xavier, Asmussen, Karsten, Jacobsen, Soren, Bartoloni, Elena, Gerli, Roberto, Bijlsma, Johannes Wj, Kruize, Aike A, Bombardieri, Stefano, Bookman, Arthur, Kallenberg, Cees, Meiners, Petra, Brun, Johan G, Jonsson, Roland, Caporali, Roberto, Carsons, Steven, De Vita, Salvatore, Del Papa, Nicoletta, Devauchelle, Valerie, Saraux, Alain, Fauchais, Anne-Laure, Sibilia, Jean, Hachulla, Eric, Illei, Gabor, Isenberg, David, Jones, Adrian, Manoussakis, Menelaos, Mandl, Thomas, Jacobsson, Lennart, Demoulins, Frederic, Montecucco, Carlomaurizio, Ng, Wan-Fai, Nishiyama, Sumusu, Omdal, Roald, Parke, Ann, Praprotnik, Sonja, Tomsic, Matjia, Price, Elizabeth, Scofield, Hal, L Sivils, Kathy, Smolen, Josef, Laqué, Roser Solans, Steinfeld, Serge, Sutcliffe, Nurhan, Sumida, Takayuki, Valesini, Guido, Valim, Valeria, Vivino, Frederick B, Vollenweider, Cristina, Seror, Raphaèle, Bowman, Simon J, Brito-Zeron, Pilar, Theander, Elke, Bootsma, Hendrika, Tzioufas, Athanasios, Gottenberg, Jacques-Eric, Ramos-Casals, Manel, Dörner, Thomas, Ravaud, Philippe, Vitali, Claudio, Mariette, Xavier, Asmussen, Karsten, Jacobsen, Soren, Bartoloni, Elena, Gerli, Roberto, Bijlsma, Johannes Wj, Kruize, Aike A, Bombardieri, Stefano, Bookman, Arthur, Kallenberg, Cees, Meiners, Petra, Brun, Johan G, Jonsson, Roland, Caporali, Roberto, Carsons, Steven, De Vita, Salvatore, Del Papa, Nicoletta, Devauchelle, Valerie, Saraux, Alain, Fauchais, Anne-Laure, Sibilia, Jean, Hachulla, Eric, Illei, Gabor, Isenberg, David, Jones, Adrian, Manoussakis, Menelaos, Mandl, Thomas, Jacobsson, Lennart, Demoulins, Frederic, Montecucco, Carlomaurizio, Ng, Wan-Fai, Nishiyama, Sumusu, Omdal, Roald, Parke, Ann, Praprotnik, Sonja, Tomsic, Matjia, Price, Elizabeth, Scofield, Hal, L Sivils, Kathy, Smolen, Josef, Laqué, Roser Solans, Steinfeld, Serge, Sutcliffe, Nurhan, Sumida, Takayuki, Valesini, Guido, Valim, Valeria, Vivino, Frederick B, and Vollenweider, Cristina

Published
2015

96. EULAR Sjögren's syndrome disease activity index (ESSDAI): a user guide

Author

MS Reumatologie/Immunologie/Infectie, Regenerative Medicine and Stem Cells, Infection & Immunity, Seror, Raphaèle, Bowman, Simon J, Brito-Zeron, Pilar, Theander, Elke, Bootsma, Hendrika, Tzioufas, Athanasios, Gottenberg, Jacques-Eric, Ramos-Casals, Manel, Dörner, Thomas, Ravaud, Philippe, Vitali, Claudio, Mariette, Xavier, Asmussen, Karsten, Jacobsen, Soren, Bartoloni, Elena, Gerli, Roberto, Bijlsma, Johannes Wj, Kruize, Aike A, Bombardieri, Stefano, Bookman, Arthur, Kallenberg, Cees, Meiners, Petra, Brun, Johan G, Jonsson, Roland, Caporali, Roberto, Carsons, Steven, De Vita, Salvatore, Del Papa, Nicoletta, Devauchelle, Valerie, Saraux, Alain, Fauchais, Anne-Laure, Sibilia, Jean, Hachulla, Eric, Illei, Gabor, Isenberg, David, Jones, Adrian, Manoussakis, Menelaos, Mandl, Thomas, Jacobsson, Lennart, Demoulins, Frederic, Montecucco, Carlomaurizio, Ng, Wan-Fai, Nishiyama, Sumusu, Omdal, Roald, Parke, Ann, Praprotnik, Sonja, Tomsic, Matjia, Price, Elizabeth, Scofield, Hal, L Sivils, Kathy, Smolen, Josef, Laqué, Roser Solans, Steinfeld, Serge, Sutcliffe, Nurhan, Sumida, Takayuki, Valesini, Guido, Valim, Valeria, Vivino, Frederick B, Vollenweider, Cristina, MS Reumatologie/Immunologie/Infectie, Regenerative Medicine and Stem Cells, Infection & Immunity, Seror, Raphaèle, Bowman, Simon J, Brito-Zeron, Pilar, Theander, Elke, Bootsma, Hendrika, Tzioufas, Athanasios, Gottenberg, Jacques-Eric, Ramos-Casals, Manel, Dörner, Thomas, Ravaud, Philippe, Vitali, Claudio, Mariette, Xavier, Asmussen, Karsten, Jacobsen, Soren, Bartoloni, Elena, Gerli, Roberto, Bijlsma, Johannes Wj, Kruize, Aike A, Bombardieri, Stefano, Bookman, Arthur, Kallenberg, Cees, Meiners, Petra, Brun, Johan G, Jonsson, Roland, Caporali, Roberto, Carsons, Steven, De Vita, Salvatore, Del Papa, Nicoletta, Devauchelle, Valerie, Saraux, Alain, Fauchais, Anne-Laure, Sibilia, Jean, Hachulla, Eric, Illei, Gabor, Isenberg, David, Jones, Adrian, Manoussakis, Menelaos, Mandl, Thomas, Jacobsson, Lennart, Demoulins, Frederic, Montecucco, Carlomaurizio, Ng, Wan-Fai, Nishiyama, Sumusu, Omdal, Roald, Parke, Ann, Praprotnik, Sonja, Tomsic, Matjia, Price, Elizabeth, Scofield, Hal, L Sivils, Kathy, Smolen, Josef, Laqué, Roser Solans, Steinfeld, Serge, Sutcliffe, Nurhan, Sumida, Takayuki, Valesini, Guido, Valim, Valeria, Vivino, Frederick B, and Vollenweider, Cristina

Published
2015

97. EULAR Sjögren's syndrome disease activity index (ESSDAI):a user guide

Author

Seror, Raphaèle, Bowman, Simon J, Brito-Zeron, Pilar, Theander, Elke, Bootsma, Hendrika, Tzioufas, Athanasios, Gottenberg, Jacques-Eric, Ramos-Casals, Manel, Dörner, Thomas, Ravaud, Philippe, Vitali, Claudio, Mariette, Xavier, Asmussen, Karsten, Jacobsen, Soren, Bartoloni, Elena, Gerli, Roberto, Bijlsma, Johannes Wj, Kruize, Aike A, Bombardieri, Stefano, Bookman, Arthur, Kallenberg, Cees, Meiners, Petra, Brun, Johan G, Jonsson, Roland, Caporali, Roberto, Carsons, Steven, De Vita, Salvatore, Del Papa, Nicoletta, Devauchelle, Valerie, Saraux, Alain, Fauchais, Anne-Laure, Sibilia, Jean, Hachulla, Eric, Illei, Gabor, Isenberg, David, Jones, Adrian, Manoussakis, Menelaos, Mandl, Thomas, Jacobsson, Lennart, Demoulins, Frederic, Montecucco, Carlomaurizio, Ng, Wan-Fai, Nishiyama, Sumusu, Omdal, Roald, Parke, Ann, Praprotnik, Sonja, Tomsic, Matjia, Price, Elizabeth, Scofield, Hal, L Sivils, Kathy, Smolen, Josef, Laqué, Roser Solans, Steinfeld, Serge, Sutcliffe, Nurhan, Sumida, Takayuki, Valesini, Guido, Valim, Valeria, Vivino, Frederick B, Vollenweider, Cristina, Seror, Raphaèle, Bowman, Simon J, Brito-Zeron, Pilar, Theander, Elke, Bootsma, Hendrika, Tzioufas, Athanasios, Gottenberg, Jacques-Eric, Ramos-Casals, Manel, Dörner, Thomas, Ravaud, Philippe, Vitali, Claudio, Mariette, Xavier, Asmussen, Karsten, Jacobsen, Soren, Bartoloni, Elena, Gerli, Roberto, Bijlsma, Johannes Wj, Kruize, Aike A, Bombardieri, Stefano, Bookman, Arthur, Kallenberg, Cees, Meiners, Petra, Brun, Johan G, Jonsson, Roland, Caporali, Roberto, Carsons, Steven, De Vita, Salvatore, Del Papa, Nicoletta, Devauchelle, Valerie, Saraux, Alain, Fauchais, Anne-Laure, Sibilia, Jean, Hachulla, Eric, Illei, Gabor, Isenberg, David, Jones, Adrian, Manoussakis, Menelaos, Mandl, Thomas, Jacobsson, Lennart, Demoulins, Frederic, Montecucco, Carlomaurizio, Ng, Wan-Fai, Nishiyama, Sumusu, Omdal, Roald, Parke, Ann, Praprotnik, Sonja, Tomsic, Matjia, Price, Elizabeth, Scofield, Hal, L Sivils, Kathy, Smolen, Josef, Laqué, Roser Solans, Steinfeld, Serge, Sutcliffe, Nurhan, Sumida, Takayuki, Valesini, Guido, Valim, Valeria, Vivino, Frederick B, and Vollenweider, Cristina

Abstract

The EULAR Sjögren's syndrome (SS) disease activity index (ESSDAI) is a systemic disease activity index that was designed to measure disease activity in patients with primary SS. With the growing use of the ESSDAI, some domains appear to be more challenging to rate than others. The ESSDAI is now in use as a gold standard to measure disease activity in clinical studies, and as an outcome measure, even a primary outcome measure, in current randomised clinical trials. Therefore, ensuring an accurate and reproducible rating of each domain, by providing a more detailed definition of each domain, has emerged as an urgent need. The purpose of the present article is to provide a user guide for the ESSDAI. This guide provides definitions and precisions on the rating of each domain. It also includes some minor improvement of the score to integrate advance in knowledge of disease manifestations. This user guide may help clinicians to use the ESSDAI, and increase the reliability of rating and consequently of the ability to detect true changes over time. This better appraisal of ESSDAI items, along with the recent definition of disease activity levels and minimal clinically important change, will improve the assessment of patients with primary SS and facilitate the demonstration of effectiveness of treatment for patients with primary SS.

Published
2015

99. Reply to J. Magalon et al.

Author

Del Papa, Nicoletta, primary, Di Luca, Gabriele, additional, Sambataro, Domenico, additional, Zaccara, Eleonora, additional, Maglione, Wanda, additional, Gabrielli, Armando, additional, Fraticelli, Paolo, additional, Moroncini, Gianluca, additional, Beretta, Lorenzo, additional, Santaniello, Alessandro, additional, Sambataro, Gianluca, additional, Ferraresi, Roberto, additional, and Vitali, Claudio, additional

Published
2015
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100. Regional Implantation of Autologous Adipose Tissue-Derived Cells Induces a Prompt Healing of Long-Lasting Indolent Digital Ulcers in Patients with Systemic Sclerosis

Author

Del Papa, Nicoletta, primary, Di Luca, Gabriele, additional, Sambataro, Domenico, additional, Zaccara, Eleonora, additional, Maglione, Wanda, additional, Gabrielli, Armando, additional, Fraticelli, Paolo, additional, Moroncini, Gianluca, additional, Beretta, Lorenzo, additional, Santaniello, Alessandro, additional, Sambataro, Gianluca, additional, Ferraresi, Roberto, additional, and Vitali, Claudio, additional

Published
2015
Full Text
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