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1. Evaluación protocolizada odontológica en el paciente con síndrome de Sjögren primario. Proyecto EPOX-SSp: metodología y objetivos

Author

Hernández Vallejo, Gonzalo, Ángeles Blázquez, M., Bohórquez, Cristina, Bonilla, Gema, Cobo, Tatiana, García Vadillo, Jesús, González Martín, Jorge Juan, Illera, Oscar, Lojo, Leticia, López Longo, Francisco Javier, Melchor, Sheila, Teresa Navío, María, Nuño, Laura, Carmen Ortega, María, Peiteado, Diana, Recuerdo, Sheila, Richi, Patricia, Rodríguez, Ana, Steiner, Martina, Valero, Marta, Fernández Castro, Mónica, López-Pintor, Rosa María, Serrano, Julia, Ramírez, Lucía, Sanz, Mariano, Andreu, Jose Luis, and Muñoz Fernández, Santiago

Published
2021
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2. Recomendaciones de la Sociedad Española de Reumatología sobre síndrome antifosfolípido primario. Parte II: síndrome antifosfolípido obstétrico y situaciones especiales

Author

Cáliz Cáliz, Rafael, Díaz del Campo Fontecha, Petra, Galindo Izquierdo, María, López Longo, Francisco Javier, Martínez Zamora, María Ángeles, Santamaria Ortiz, Amparo, Amengual Pliego, Olga, Cuadrado Lozano, María José, Delgado Beltrán, María Paloma, Ortells, Loreto Carmona, Pérez, Evelin Cecilia Cervantes, Rego, Gisela Díaz-Cordovés, Corral, Sandra Garrote, Varela, Clara Fuego, López, María Martín, Nishishinya, Betina, Navarro, Marta Novella, Testa, Claudia Pereda, Pérez, Hiurma Sánchez, Silva-Fernández, Lucia, and Taboada, Víctor Manuel Martínez

Published
2020
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3. Recomendaciones de la Sociedad Española de Reumatología sobre síndrome antifosfolípido primario. Parte I: Diagnóstico, evaluación y tratamiento

Author

Cáliz Cáliz, Rafael, Díaz del Campo Fontecha, Petra, Galindo Izquierdo, María, López Longo, Francisco Javier, Martínez Zamora, María Ángeles, Santamaría Ortiz, Amparo, Amengual Pliego, Olga, Cuadrado Lozano, María José, Delgado Beltrán, María Paloma, Carmona Ortells, Loreto, Cervantes Pérez, Evelin Cecilia, Díaz-Cordovés Rego, Gisela, Garrote Corral, Sandra, Fuego Varela, Clara, Martín López, María, Nishishinya, Betina, Novella Navarro, Marta, Pereda Testa, Claudia, Sánchez Pérez, Hiurma, Silva-Fernández, Lucia, and Martínez Taboada, Víctor Manuel

Published
2020
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4. Pure Membranous Lupus Nephritis: Description of a Cohort of 150 Patients and Review of the Literature

Author

Silva-Fernández, Lucía, Otón, Teresa, Askanase, Anca, Carreira, Patricia, López-Longo, Francisco Javier, Olivé, Alejandro, Rúa-Figueroa, Íñigo, Narváez, Javier, Ruiz-Lucea, Esther, Andrés, Mariano, Calvo, Enrique, Toyos, Francisco, Alegre-Sancho, Juan José, Tomero, Eva, Montilla, Carlos, Zea, Antonio, Uriarte, Esther, Calvo-Alén, Jaime, Marras, Carlos, Martínez-Taboada, Víctor M., Belmonte-López, María Ángeles, Rosas, José, Raya, Enrique, Bonilla, Gema, Freire, Mercedes, Pego-Reigosa, José María, Millán, Isabel, Hughes-Morley, Adwoa, and Andreu, José Luis

Published
2019
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5. Registro de pacientes con miopatía inflamatoria de la Sociedad Madrileña de Reumatología: análisis descriptivo

Author

Nuño, Laura, Joven, Beatriz, Carreira, Patricia, Maldonado, Valentina, Larena, Carmen, Llorente, Irene, Tomero, Eva, Barbadillo, María Carmen, García-de la Peña, Paloma, Ruiz, Lucía, López-Robledillo, Juan Carlos, Moruno, Henry, Pérez, Ana, Cobo-Ibáñez, Tatiana, Almodóvar, Raquel, Lojo, Leticia, Monteagudo, Indalecio, García-De Yébenes, María Jesús, and López-Longo, Francisco Javier

Published
2017
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6. Recomendaciones sobre actuaciones a seguir durante la edad fértil, el embarazo, posparto y lactancia en pacientes con enfermedades reumáticas inflamatorias y autoinmunes

Author

Martínez López, Juan Antonio, García Vivar, M. Luz, Cáliz, Rafael, Freire, Mercedes, Galindo, María, Hernández, Maria Victoria, López Longo, Francisco Javier, Martínez Taboada, Víctor, Pego Reigosa, Jose María, Rubio, Esteban, Trujillo, Elisa, and Vela-Casasempere, Paloma

Published
2017
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7. Immunochip Analysis Identifies Multiple Susceptibility Loci for Systemic Sclerosis

Author

Ortego-Centeno, Norberto, Ríos, Raquel, Callejas, José Luis, Navarrete, Nuria, García Portales, Rosa, Camps, María Teresa, Fernández-Nebro, Antonio, González-Escribano, María F., Sánchez-Román, Julio, García-Hernández, Francisco José, Castillo, María Jesús, Aguirre, María Ángeles, Gómez-Gracia, Inmaculada, Fernández-Gutiérrez, Benjamín, Rodríguez-Rodríguez, Luis, Vicente, Esther, Andreu, José Luis, Fernández de Castro, Mónica, García de la Peña, Paloma, López-Longo, Francisco Javier, Martínez, Lina, Fonollosa, Vicente, Espinosa, Gerard, Tolosa, Carlos, Pros, Anna, Rodríguez Carballeira, Mónica, Narváez, Francisco Javier, Rubio Rivas, Manel, Ortiz Santamaría, Vera, Díaz, Bernardino, Trapiella, Luis, Freire, María del Carmen, Sousa, Adrián, Egurbide, María Victoria, Fanlo Mateo, Patricia, Sáez-Comet, Luis, Díaz, Federico, Hernández, Vanesa, Beltrán, Emma, Román-Ivorra, José Andrés, Grau, Elena, Alegre Sancho, Juan José, Blanco García, Francisco J., Oreiro, Natividad, Fernández Sueiro, Luis, Mayes, Maureen D., Bossini-Castillo, Lara, Gorlova, Olga, Martin, José Ezequiel, Zhou, Xiaodong, Chen, Wei V., Assassi, Shervin, Ying, Jun, Tan, Filemon K., Arnett, Frank C., Reveille, John D., Guerra, Sandra, Teruel, María, Carmona, Francisco David, Gregersen, Peter K., Lee, Annette T., López-Isac, Elena, Ochoa, Eguzkine, Carreira, Patricia, Simeón, Carmen Pilar, Castellví, Iván, González-Gay, Miguel Ángel, Zhernakova, Alexandra, Padyukov, Leonid, Alarcón-Riquelme, Marta, Wijmenga, Cisca, Brown, Matthew, Beretta, Lorenzo, Riemekasten, Gabriela, Witte, Torsten, Hunzelmann, Nicolas, Kreuter, Alexander, Distler, Jörg H.W., Voskuyl, Alexandre E., Schuerwegh, Annemie J., Hesselstrand, Roger, Nordin, Annika, Airó, Paolo, Lunardi, Claudio, Shiels, Paul, van Laar, Jacob M., Herrick, Ariane, Worthington, Jane, Denton, Christopher, Wigley, Fredrick M., Hummers, Laura K., Varga, John, Hinchcliff, Monique E., Baron, Murray, Hudson, Marie, Pope, Janet E., Furst, Daniel E., Khanna, Dinesh, Phillips, Kristin, Schiopu, Elena, Segal, Barbara M., Molitor, Jerry A., Silver, Richard M., Steen, Virginia D., Simms, Robert W., Lafyatis, Robert A., Fessler, Barri J., Frech, Tracy M., AlKassab, Firas, Docherty, Peter, Kaminska, Elzbieta, Khalidi, Nader, Jones, Henry Niall, Markland, Janet, Robinson, David, Broen, Jasper, Radstake, Timothy R.D.J., Fonseca, Carmen, Koeleman, Bobby P., and Martin, Javier

Published
2014
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11. Tocilizumab in systemic sclerosis:a randomised, double-blind, placebo-controlled, phase 3 trial

Author

Khanna, Dinesh, Lin, Celia J.F., Furst, Daniel E., Goldin, Jonathan, Kim, Grace, Kuwana, Masataka, Allanore, Yannick, Matucci-Cerinic, Marco, Distler, Oliver, Shima, Yoshihito, van Laar, Jacob M., Spotswood, Helen, Wagner, Bridget, Siegel, Jeffrey, Jahreis, Angelika, Denton, Christopher P., Lucero, Eleonora, Pons-Estel, Bernardo, Rivero, Mariano, Tate, Guillermo, Smith, Vanessa, De Langhe, Ellen, Rashkov, Rasho, Batalov, Anastas, Goranov, Ivan, Stoilov, Rumen, Dunne, James, Johnson, Sindhu R., Pope, Janet E., Martinović Kaliterna, Dušanka, Mogensen, Mette, Olesen, Anne Braae, Henes, Joerg Christoph, Müller-Ladner, Ulf, Riemekasten, Gabriela, Skapenko, Alla, Vlachoyiannopoulos, Panayiotis, Kiss, Emese, Minier, Tünde, Beretta, Lorenzo, Gremese, Elisa, Valentini, Gabriele, Asano, Yoshihide, Atsumi, Tatsuya, Ihn, Hironobu, Ishii, Tomonori, Ishikawa, Osamu, Takahashi, Hiroki, Takehara, Kazuhiko, Tanaka, Yoshiya, Yamasaki, Yoshioki, Bukauskiene, Loreta, Butrimiene, Irena, Medrano Ramirez, Gabriel, Ramos-Remus, Cesar, Sofia Rodriguez Reyna, Tatiana, de Vries-Bouwstra, Jeska, Batko, Bogdan, Jeka, Slawomir, Kucharz, Eugeniusz, Majdan, Maria, Olesinska, Marzena, Smolenska, Zaneta, Alves, Jose, Santos, Maria, Mihai, Carmen Marina, Rednic, Simona, Castellvi Barranco, Ivan, Lopez Longo, Francisco Javier, Simeon Aznar, Carmen, Carreira, Patricia, Walker, Ulrich A., Derrett-Smith, Emma, Griffiths, Bridget, McKay, Neil, Aelion, Jacob, Borofsky, Michael, Fleischmann, Roy, Forstot, Joseph Z., Kafaja, Suzanne, Khan, M. Faisal, Kohen, Michael D., Martin, Richard W., Mendoza-Ballesteros, Fabian, Nami, Alireza, Pang, Shirley, Rios, Grissel, Simms, Robert, Sullivan, Keith Michael, Steen, Virginia D., Khanna, Dinesh, Lin, Celia J.F., Furst, Daniel E., Goldin, Jonathan, Kim, Grace, Kuwana, Masataka, Allanore, Yannick, Matucci-Cerinic, Marco, Distler, Oliver, Shima, Yoshihito, van Laar, Jacob M., Spotswood, Helen, Wagner, Bridget, Siegel, Jeffrey, Jahreis, Angelika, Denton, Christopher P., Lucero, Eleonora, Pons-Estel, Bernardo, Rivero, Mariano, Tate, Guillermo, Smith, Vanessa, De Langhe, Ellen, Rashkov, Rasho, Batalov, Anastas, Goranov, Ivan, Stoilov, Rumen, Dunne, James, Johnson, Sindhu R., Pope, Janet E., Martinović Kaliterna, Dušanka, Mogensen, Mette, Olesen, Anne Braae, Henes, Joerg Christoph, Müller-Ladner, Ulf, Riemekasten, Gabriela, Skapenko, Alla, Vlachoyiannopoulos, Panayiotis, Kiss, Emese, Minier, Tünde, Beretta, Lorenzo, Gremese, Elisa, Valentini, Gabriele, Asano, Yoshihide, Atsumi, Tatsuya, Ihn, Hironobu, Ishii, Tomonori, Ishikawa, Osamu, Takahashi, Hiroki, Takehara, Kazuhiko, Tanaka, Yoshiya, Yamasaki, Yoshioki, Bukauskiene, Loreta, Butrimiene, Irena, Medrano Ramirez, Gabriel, Ramos-Remus, Cesar, Sofia Rodriguez Reyna, Tatiana, de Vries-Bouwstra, Jeska, Batko, Bogdan, Jeka, Slawomir, Kucharz, Eugeniusz, Majdan, Maria, Olesinska, Marzena, Smolenska, Zaneta, Alves, Jose, Santos, Maria, Mihai, Carmen Marina, Rednic, Simona, Castellvi Barranco, Ivan, Lopez Longo, Francisco Javier, Simeon Aznar, Carmen, Carreira, Patricia, Walker, Ulrich A., Derrett-Smith, Emma, Griffiths, Bridget, McKay, Neil, Aelion, Jacob, Borofsky, Michael, Fleischmann, Roy, Forstot, Joseph Z., Kafaja, Suzanne, Khan, M. Faisal, Kohen, Michael D., Martin, Richard W., Mendoza-Ballesteros, Fabian, Nami, Alireza, Pang, Shirley, Rios, Grissel, Simms, Robert, Sullivan, Keith Michael, and Steen, Virginia D.

Abstract

Background: A phase 2 trial of tocilizumab showed preliminary evidence of efficacy in systemic sclerosis. We assessed skin fibrosis and systemic sclerosis-associated interstitial lung disease (SSc-ILD) in a phase 3 trial to investigate the safety and efficacy of tocilizumab, an anti-interleukin-6 receptor antibody, in the treatment of systemic sclerosis. Methods: In this multicentre, randomised, double-blind, placebo-controlled, phase 3 trial, participants were recruited from 75 sites in 20 countries across Europe, North America, Latin America, and Japan. Adults with diffuse cutaneous systemic sclerosis for 60 months or less and a modified Rodnan skin score (mRSS) of 10–35 at screening were randomly assigned (1:1) with a voice-web-response system to receive subcutaneous tocilizumab 162 mg or placebo weekly for 48 weeks, stratified by IL-6 levels; participants and investigators were masked to treatment group. The primary endpoint was the difference in change from baseline to week 48 in mRSS. Percentage of predicted forced vital capacity (FVC% predicted) at week 48, time to treatment failure, and patient-reported and physician-reported outcomes were secondary endpoints. This trial is registered with ClinicalTrials.gov (number NCT02453256) and is closed to accrual. Findings: Between Nov 20, 2015, and Feb 14, 2017, 210 individuals were randomly assigned to receive tocilizumab (n=104) or placebo (n=106). In the intention-to-treat population, least squares mean [LSM] change from baseline to week 48 in mRSS was −6·14 for tocilizumab and −4·41 for placebo (adjusted difference −1·73 [95% CI −3·78 to 0·32]; p=0·10). The shift in distribution of change from baseline in FVC% predicted at week 48 favoured tocilizumab (van Elteren nominal p=0·002 vs placebo), with a difference in LSM of 4·2 (95% CI 2·0–6·4; nominal p=0·0002), as did time to treatment failure (hazard ratio 0·63 [95% CI 0·37–1·06]; nominal p=0·08). Change in LSM from baseline to week 48 in Health Assessment Ques

Published
2020

12. Timing of onset affects arthritis presentation pattern in antisyntethase syndrome

Author

González Gay, Miguel A., Montecucco, Carlomaurizio, Selva O Callaghan, Albert, Trallero Araguas, Ernesto, Molberg, Ovynd, Andersson, Helena, Rojas Serrano, Jorge, Perez Roman, Diana Isabel, Bauhammer, Jutta, Fiehn, Christoph, Neri, Rossella, Barsotti, Simone, Lorenz, Hannes M., Andrea Doria, anna ghirardello, Iannone, Florenzo, Giannini, Margherita, Franceschini, Franco, Cavazzana, Ilaria, Triantafyllias, Konstantinos, Benucci, Maurizio, Infantino, Maria, Manfredi, Mariangela, Conti, Fabrizio, Schwarting, Andreas, Sebastiani, Giandomenico, Iuliano, Annamaria, Emmi, Giacomo, Silvestri, Elena, Govoni, Marcello, Scirè, Carlo Alberto, Furini, Federica, Lopez Longo, Francisco Javier, Martínez Barrio, Julia, Sebastiani, Marco, Manfredi, Andreina, Bachiller Corral, Javier, Sifuentes Giraldo, Walter Alberto, Cimmino, Marco A., Cosso, Claudio, Belotti Masserini, Alessandro, Cagnotto, Giovanni, Codullo, Veronica, Romano, Mariaeva, Paolazzi, Giuseppe, Pellerito, Raffaele, Saketkoo, Lesley Ann, Ortego Centeno, Norberto, Quartuccio, Luca, Batticciotto, Alberto, Bartoloni Bocci, Elena, Gerli, Roberto, Specker, Christof, Bravi, Elena, Selmi, Carlo, Parisi, Simone, Salaffi, Fausto, Meloni, Federica, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Confalonieri, Marco, Tomietto, Paola, Nuno, Laura, Bonella, Francesco, Pipitone, Nicolò, Mera Valera, Antonio, Perez Gomez, Nair, Gerzeli, Simone, Lopez Mejias, Raquel, Matos Costa, Carlo Jorge, Pereira Da Silva, Jose Antonio, Cifrian, José, Alpini, Claudia, Olivieri, Ignazio, Blázquez Cañamero, María Ángeles, Rodriguez Cambrón, Ana Belén, Castañeda, Santos, Cavagna, Lorenzo, González-Gay, M, Montecucco, C, Selva-O'Callaghan, A, Trallero-Araguas, E, Molberg, O, Andersson, H, Rojas-Serrano, J, Perez-Roman, D, Bauhammer, J, Fiehn, C, Neri, R, Barsotti, S, Lorenz, H, Doria, A, Ghirardello, A, Iannone, F, Giannini, M, Franceschini, F, Cavazzana, I, Triantafyllias, K, Benucci, M, Infantino, M, Manfredi, M, Conti, F, Schwarting, A, Sebastiani, G, Iuliano, A, Emmi, G, Silvestri, E, Govoni, M, Scirè, C, Furini, F, Lopez-Longo, F, Martínez-Barrio, J, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Cimmino, M, Cosso, C, Belotti Masserini, A, Cagnotto, G, Codullo, V, Romano, M, Paolazzi, G, Pellerito, R, Saketkoo, L, Ortego-Centeno, N, Quartuccio, L, Batticciotto, A, Bartoloni Bocci, E, Gerli, R, Specker, C, Bravi, E, Selmi, C, Parisi, S, Salaffi, F, Meloni, F, Marchioni, E, Pesci, A, Dei, G, Confalonieri, M, Tomietto, P, Nuno, L, Bonella, F, Pipitone, N, Mera-Valera, A, Perez-Gomez, N, Gerzeli, S, Lopez-Mejias, R, Matos-Costa, C, Pereira da Silva, J, Cifrian, J, Alpini, C, Olivieri, I, Blázquez Cañamero, M, Rodriguez Cambrón, A, Castañeda, S, Cavagna, L, González-Gay, Miguel A, Montecucco, Carlomaurizio, Selva-O'Callaghan, Albert, Trallero-Araguas, Ernesto, Molberg, Ovynd, Andersson, Helena, Rojas-Serrano, Jorge, Perez-Roman, Diana Isabel, Bauhammer, Jutta, Fiehn, Christoph, Neri, Rossella, Barsotti, Simone, Lorenz, Hannes M, Doria, Andrea, Ghirardello, Anna, Iannone, Florenzo, Giannini, Margherita, Franceschini, Franco, Cavazzana, Ilaria, Triantafyllias, Konstantino, Benucci, Maurizio, Infantino, Maria, Manfredi, Mariangela, Conti, Fabrizio, Schwarting, Andrea, Sebastiani, Giandomenico, Iuliano, Annamaria, Emmi, Giacomo, Silvestri, Elena, Govoni, Marcello, Scirè, Carlo Alberto, Furini, Federica, Lopez-Longo, Francisco Javier, Martínez-Barrio, Julia, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Cimmino, Marco A, Cosso, Claudio, Belotti Masserini, Alessandro, Cagnotto, Giovanni, Codullo, Veronica, Romano, Mariaeva, Paolazzi, Giuseppe, Pellerito, Raffaele, Saketkoo, Lesley Ann, Ortego-Centeno, Norberto, Quartuccio, Luca, Batticciotto, Alberto, Bartoloni Bocci, Elena, Gerli, Roberto, Specker, Christof, Bravi, Elena, Selmi, Carlo, Parisi, Simone, Salaffi, Fausto, Meloni, Federica, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Confalonieri, Marco, Tomietto, Paola, Nuno, Laura, Bonella, Francesco, Pipitone, Nicolò, Mera-Valera, Antonio, Perez-Gomez, Nair, Gerzeli, Simone, Lopez-Mejias, Raquel, Matos-Costa, Carlo Jorge, Pereira da Silva, Jose Antonio, Cifrian, José, Alpini, Claudia, Olivieri, Ignazio, Blázquez Cañamero, María Ángele, Rodriguez Cambrón, Ana Belén, Castañeda, Santo, and Cavagna, Lorenzo

Subjects
Adult, Male, Time Factors, phenotype, autoantibodies, prevalence, Medizin, Antisynthetase syndrome, Arthritis pattern, Timing of onset, Arthritis, Autoantibodies, Biomarkers, Europe, Female, Humans, Mexico, Middle Aged, Myositis, Phenotype, Prevalence, Prognosis, Retrospective Studies, Risk Factors, NO, antisynthetase syndrome, arthritis, pulmonary disease, male, middle aged, risk factors, humans, adult, biomarkers, timefFactors, arthriti, retrospective studies, female, arthritis, antisyntethase syndrome, prognosis, myositis
Abstract

Objective To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD). Methods The patients were selected from a retrospective large international cohort of ASSD patients regularly followed-up in centres referring to AENEAS collaborative group. Patients were eligible if they had an antisynthetase antibody testing positive in at least two determinations along with arthritis occurring either at ASSD onset (Group 1) or during the course of the disease (Group 2). Results 445 (70%; 334 females, 110 males, 1 transsexual) out of the 636 ASSD we collected had arthritis, in the majority of cases (367, 83%) from disease onset (Group 1). Patients belonging to Group 1 with respect to Group 2 had an arthritis more commonly polyarticular and symmetrical (p=0.015), IgM-Rheumatoid factor positive (p=0.035), erosions at hands and feet plain x-rays (p=0.036) and more commonly satisfying the 1987 revised classification criteria for rheumatoid arthritis (RA) (p=0.004). Features such as Raynaud's phenomenon, mechanic's hands and fever (e.g. accompanying findings) were more frequently reported in Group 2 (p=0.005). Conclusion In ASSD, the timing of appearance with respect to disease onset influences arthritis characteristics. In particular, RA features are more common when arthritis occurs from ASSD onset, suggesting an overlap between RA and ASSD in these patients. When arthritis appears during the follow-up, it is very close to a connective tissue disease-related arthritis. Also, the different prevalence of accompanying features between these two groups is in line with this possibility

Published
2018

14. Nailfold capillaroscopy characteristics of antisynthetase syndrome and possible clinical associations : Results of a multicenter international study

Author

Sebastiani, Marco, Triantafyllias, Konstantinos, Manfredi, Andreina, González-Gay, Miguel Angel, Palmou-Fontana, Natalia, Cassone, Giulia, Drott, Ulrich, Delbrück, Christiane, Rojas-Serrano, Jorge, Bertolazzi, Chiara, Nuño, Laura, Giannini, Margherita, Iannone, Florenzo, Vicente, Esther F., Castañeda, Santos, Selva-O'Callaghan, Albert, Araguas, Ernesto Trallero, Emmi, Giacomo, Iuliano, Annamaria, Bauhammer, Jutta, Miehle, Nikolaus, Parisi, Simone, Cavagna, Lorenzo, Codullo, Veronica, Montecucco, Carlomaurizio, Lopez-Longo, Francisco Javier, Martínez-Barrio, Julia, Nieto-González, Juan Carlos, Vichi, Silvia, Confalonieri, Marco, Tomietto, Paola, Bergner, Raoul, Sulli, Alberto, Bonella, Francesco, Furini, Federica, Scirè, Carlo Alberto, Bortoluzzi, Alessandra, Specker, Christof, Barsotti, Simone, Neri, Rossella, Mosca, Marta, Caproni, Marzia, Weinmann-Menke, Julia, Schwarting, Andreas, Smith, Vanessa, Cutolo, Maurizio, Selva-O’Callaghan, Albert, Sebastiani, M, Triantafyllias, K, Manfredi, A, Gonzalez-Gay, M, Palmou-Fontana, N, Cassone, G, Drott, U, Delbruck, C, Rojas-Serrano, J, Bertolazzi, C, Nuno, L, Giannini, M, Iannone, F, Vicente, E, Castaneda, S, Selva-O'Callaghan, A, Araguas, E, Emmi, G, Iuliano, A, Bauhammer, J, Miehle, N, Parisi, S, Cavagna, L, Codullo, V, Montecucco, C, Lopez-Longo, F, Martinez-Barrio, J, Nieto-Gonzalez, J, Vichi, S, Confalonieri, M, Tomietto, P, Bergner, R, Sulli, A, Bonella, F, Furini, F, Scire, C, Bortoluzzi, A, Specker, C, Barsotti, S, Neri, R, Mosca, M, Caproni, M, Weinmann-Menke, J, Schwarting, A, Smith, V, Cutolo, M, Sebastiani, Marco, Triantafyllias, Konstantino, Manfredi, Andreina, Angel González-Gay, Miguel, Palmou-Fontana, Natalia, Cassone, Giulia, Drott, Ulrich, Delbrück, Christiane, Rojas-Serrano, Jorge, Bertolazzi, Chiara, Nuño, Laura, Giannini, Margherita, Iannone, Florenzo, Vicente, Esther F., Castañeda, Santo, Selva-O’Callaghan, Albert, Trallero Araguas, Ernesto, Emmi, Giacomo, Iuliano, Annamaria, Bauhammer, Jutta, Miehle, Nikolau, Parisi, Simone, Cavagna, Lorenzo, Codullo, Veronica, Montecucco, Carlomaurizio, Javier Lopez-Longo, Francisco, Martínez-Barrio, Julia, Carlos Nieto-González, Juan, Vichi, Silvia, Confalonieri, Marco, Tomietto, Paola, Bergner, Raoul, Sulli, Alberto, Bonella, Francesco, Furini, Federica, Alberto Scirè, Carlo, Bortoluzzi, Alessandra, Specker, Christof, Barsotti, Simone, Neri, Rossella, Mosca, Marta, Caproni, Marzia, Weinmann-Menke, Julia, Schwarting, Andrea, Smith, Vanessa, and Cutolo, Maurizio

Subjects
Male, Medizin, Antisynthetase syndrome, ANTISYNTHETASE ANTIBODIES, Disease, Serology, Microscopic Angioscopy, 0302 clinical medicine, Interquartile range, Capillaroscopy, Anti-synthetase syndrome, Immunology and Allergy, 030212 general & internal medicine, Raynaud phenomenon, Prospective cohort study, Myositis, Nailfold Capillaroscopy, Middle Aged, ANTISYNTHETASE SYNDROME, NAILFOLD VIDEOCAPILLAROSCOPY, RAYNAUD PHENOMENON, SYSTEMIC SCLEROSIS PATTERN, Antibodies, Antinuclear, Female, Adult, medicine.medical_specialty, Nailfold capillaroscopy, anti-synthetase syndrome, myositis, Immunology, Systemic sclerosis pattern, NO, Amino Acyl-tRNA Synthetases, 03 medical and health sciences, Rheumatology, Internal medicine, medicine, Humans, Aged, Retrospective Studies, 030203 arthritis & rheumatology, Antisynthetase antibodie, business.industry, Nailfold videocapillaroscopy, Raynaud Disease, Antisynthetase antibodies, Antisynthetase syndrome, Nailfold videocapillaroscopy, Raynaud phenomenon, Systemic sclerosis pattern, medicine.disease, Capillaries, Nails, business, Follow-Up Studies
Abstract

Objective.To describe nailfold videocapillaroscopy (NVC) features of patients with antisynthetase syndrome (AS) and to investigate possible correlations with clinical and serological features of the disease.Methods.We retrospectively analyzed NVC images of 190 patients with AS [females/males 3.63, mean age 49.7 ± 12.8 yrs, median disease duration 53.7 mos (interquartile range 82), 133 anti-Jo1 and 57 non–anti-Jo1-positive patients]. For each patient, we examined number of capillaries, giant capillaries, microhemorrhages, avascular areas, ramified capillaries, and the presence of systemic sclerosis (SSc)-like pattern. Finally, we correlated NVC features with clinical and serological findings of patients with AS. Concomitantly, a historical cohort of 75 patients with antinuclear antibody–negative primary Raynaud phenomenon (RP) and longterm followup was used as a control group (female/male ratio 4.13/1, mean age 53.9 ± 17.6 yrs) for NVC measures.Results.NVC abnormalities were observed in 62.1% of AS patients compared with 29.3% of primary RP group (p < 0.001). An SSc-like pattern was detected in 67 patients (35.3%) and it was associated with anti-Jo1 antibodies (p = 0.002) and also with a longer disease duration (p = 0.004). Interestingly, there was no significant correlation between the presence of SSc-like pattern and RP, and only 47% of patients with SSc-like pattern had RP.Conclusion.NVC abnormalities are commonly observed in AS, independently from the occurrence of RP. The presence of an SSc-like pattern could allow identification of a more defined AS subtype, and prospective studies could confirm the association with clinical and serological features of AS.

Published
2019

15. 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

16. 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

17. Pure Membranous Lupus Nephritis: Description of a Cohort of 150 Patients and Review of the Literature

Author

Silva-Fernández, Lucía, Otón, Teresa, Askanase, Anca, Carreira, Patricia, Lopez-Longo, Francisco Javier, Olive, Alejandro, Rua-Figuero, Inigo, Ruiz-Lucea, Esther, Andres, Mariano, Calvo, Enrique, Toyos, Francisco, Alegre-Sancho, Juan Jose, Tomero, Eva, Montilla, Carlos, Zea, Antonio, Uriarte, Esther, Calvo-Alen, Jaime, Marras, Carlos, Martinez-Taboada, Victor M., Belmonte-Lopez, Maria Angeles, Rosas, Jose, Raya, Enrique, Bonilla, Gema, Freire, Mercedes, Pego-Reigosa, Jose Maria, Millan, Isabel, and Parker, Adwoa

Abstract

Objectives The course and long-term outcome of pure membranous lupus nephritis (MLN) are little understood. The aims of this study are to evaluate the clinical features, course, outcome and prognostic indicators in pure MLN and to determine the impact of ethnicity and the type of health insurance on the course and prognosis of pure MLN. Methods We conducted a retrospective review of medical records of 150 patients with pure MLN from Spain and the USA. Results Mean age was 34.2±12.5 and 80% were women. Sixty-eight percent of patients had nephrotic syndrome at diagnosis. The average serum creatinine was 0.98±0.78mg/dl. Six percent of patients died and 5.3% developed end-stage renal disease (ESRD). ESRD was predicted by male sex, hypertension, dyslipidemia, high basal 24h-proteinuria, high basal serum creatinine and a low basal creatinine clearance. Age, cardiac insufficiency, peripheral artheriopathy, hemodialysis and not having received mycophenolate mofetil or antimalarials for MLN predicted death. Conclusions Pure MLN frequently presents with nephrotic syndrome, high proteinuria and normal serum creatinine. Its prognosis is favourable in maintaining renal function although proteinuria usually persists over time. Baseline cardiovascular disease and not having a health insurance are related with poor prognosis.

Published
2017

18. Timing of onset affects arthritis presentation pattern in antisyntethase syndrome

Author

González-Gay, M, Montecucco, C, Selva-O'Callaghan, A, Trallero-Araguas, E, Molberg, O, Andersson, H, Rojas-Serrano, J, Perez-Roman, D, Bauhammer, J, Fiehn, C, Neri, R, Barsotti, S, Lorenz, H, Doria, A, Ghirardello, A, Iannone, F, Giannini, M, Franceschini, F, Cavazzana, I, Triantafyllias, K, Benucci, M, Infantino, M, Manfredi, M, Conti, F, Schwarting, A, Sebastiani, G, Iuliano, A, Emmi, G, Silvestri, E, Govoni, M, Scirè, C, Furini, F, Lopez-Longo, F, Martínez-Barrio, J, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Cimmino, M, Cosso, C, Belotti Masserini, A, Cagnotto, G, Codullo, V, Romano, M, Paolazzi, G, Pellerito, R, Saketkoo, L, Ortego-Centeno, N, Quartuccio, L, Batticciotto, A, Bartoloni Bocci, E, Gerli, R, Specker, C, Bravi, E, Selmi, C, Parisi, S, Salaffi, F, Meloni, F, Marchioni, E, Pesci, A, Dei, G, Confalonieri, M, Tomietto, P, Nuno, L, Bonella, F, Pipitone, N, Mera-Valera, A, Perez-Gomez, N, Gerzeli, S, Lopez-Mejias, R, Matos-Costa, C, Pereira da Silva, J, Cifrian, J, Alpini, C, Olivieri, I, Blázquez Cañamero, M, Rodriguez Cambrón, A, Castañeda, S, Cavagna, L, González-Gay, Miguel A, Montecucco, Carlomaurizio, Selva-O'Callaghan, Albert, Trallero-Araguas, Ernesto, Molberg, Ovynd, Andersson, Helena, Rojas-Serrano, Jorge, Perez-Roman, Diana Isabel, Bauhammer, Jutta, Fiehn, Christoph, Neri, Rossella, Barsotti, Simone, Lorenz, Hannes M, Doria, Andrea, Ghirardello, Anna, Iannone, Florenzo, Giannini, Margherita, Franceschini, Franco, Cavazzana, Ilaria, Triantafyllias, Konstantinos, Benucci, Maurizio, Infantino, Maria, Manfredi, Mariangela, Conti, Fabrizio, Schwarting, Andreas, Sebastiani, Giandomenico, Iuliano, Annamaria, Emmi, Giacomo, Silvestri, Elena, Govoni, Marcello, Scirè, Carlo Alberto, Furini, Federica, Lopez-Longo, Francisco Javier, Martínez-Barrio, Julia, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Cimmino, Marco A, Cosso, Claudio, Belotti Masserini, Alessandro, Cagnotto, Giovanni, Codullo, Veronica, Romano, Mariaeva, Paolazzi, Giuseppe, Pellerito, Raffaele, Saketkoo, Lesley Ann, Ortego-Centeno, Norberto, Quartuccio, Luca, Batticciotto, Alberto, Bartoloni Bocci, Elena, Gerli, Roberto, Specker, Christof, Bravi, Elena, Selmi, Carlo, Parisi, Simone, Salaffi, Fausto, Meloni, Federica, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Confalonieri, Marco, Tomietto, Paola, Nuno, Laura, Bonella, Francesco, Pipitone, Nicolò, Mera-Valera, Antonio, Perez-Gomez, Nair, Gerzeli, Simone, Lopez-Mejias, Raquel, Matos-Costa, Carlo Jorge, Pereira da Silva, Jose Antonio, Cifrian, José, Alpini, Claudia, Olivieri, Ignazio, Blázquez Cañamero, María Ángeles, Rodriguez Cambrón, Ana Belén, Castañeda, Santos, Cavagna, Lorenzo, González-Gay, M, Montecucco, C, Selva-O'Callaghan, A, Trallero-Araguas, E, Molberg, O, Andersson, H, Rojas-Serrano, J, Perez-Roman, D, Bauhammer, J, Fiehn, C, Neri, R, Barsotti, S, Lorenz, H, Doria, A, Ghirardello, A, Iannone, F, Giannini, M, Franceschini, F, Cavazzana, I, Triantafyllias, K, Benucci, M, Infantino, M, Manfredi, M, Conti, F, Schwarting, A, Sebastiani, G, Iuliano, A, Emmi, G, Silvestri, E, Govoni, M, Scirè, C, Furini, F, Lopez-Longo, F, Martínez-Barrio, J, Sebastiani, M, Manfredi, A, Bachiller-Corral, J, Sifuentes Giraldo, W, Cimmino, M, Cosso, C, Belotti Masserini, A, Cagnotto, G, Codullo, V, Romano, M, Paolazzi, G, Pellerito, R, Saketkoo, L, Ortego-Centeno, N, Quartuccio, L, Batticciotto, A, Bartoloni Bocci, E, Gerli, R, Specker, C, Bravi, E, Selmi, C, Parisi, S, Salaffi, F, Meloni, F, Marchioni, E, Pesci, A, Dei, G, Confalonieri, M, Tomietto, P, Nuno, L, Bonella, F, Pipitone, N, Mera-Valera, A, Perez-Gomez, N, Gerzeli, S, Lopez-Mejias, R, Matos-Costa, C, Pereira da Silva, J, Cifrian, J, Alpini, C, Olivieri, I, Blázquez Cañamero, M, Rodriguez Cambrón, A, Castañeda, S, Cavagna, L, González-Gay, Miguel A, Montecucco, Carlomaurizio, Selva-O'Callaghan, Albert, Trallero-Araguas, Ernesto, Molberg, Ovynd, Andersson, Helena, Rojas-Serrano, Jorge, Perez-Roman, Diana Isabel, Bauhammer, Jutta, Fiehn, Christoph, Neri, Rossella, Barsotti, Simone, Lorenz, Hannes M, Doria, Andrea, Ghirardello, Anna, Iannone, Florenzo, Giannini, Margherita, Franceschini, Franco, Cavazzana, Ilaria, Triantafyllias, Konstantinos, Benucci, Maurizio, Infantino, Maria, Manfredi, Mariangela, Conti, Fabrizio, Schwarting, Andreas, Sebastiani, Giandomenico, Iuliano, Annamaria, Emmi, Giacomo, Silvestri, Elena, Govoni, Marcello, Scirè, Carlo Alberto, Furini, Federica, Lopez-Longo, Francisco Javier, Martínez-Barrio, Julia, Sebastiani, Marco, Manfredi, Andreina, Bachiller-Corral, Javier, Sifuentes Giraldo, Walter Alberto, Cimmino, Marco A, Cosso, Claudio, Belotti Masserini, Alessandro, Cagnotto, Giovanni, Codullo, Veronica, Romano, Mariaeva, Paolazzi, Giuseppe, Pellerito, Raffaele, Saketkoo, Lesley Ann, Ortego-Centeno, Norberto, Quartuccio, Luca, Batticciotto, Alberto, Bartoloni Bocci, Elena, Gerli, Roberto, Specker, Christof, Bravi, Elena, Selmi, Carlo, Parisi, Simone, Salaffi, Fausto, Meloni, Federica, Marchioni, Enrico, Pesci, Alberto, Dei, Giulia, Confalonieri, Marco, Tomietto, Paola, Nuno, Laura, Bonella, Francesco, Pipitone, Nicolò, Mera-Valera, Antonio, Perez-Gomez, Nair, Gerzeli, Simone, Lopez-Mejias, Raquel, Matos-Costa, Carlo Jorge, Pereira da Silva, Jose Antonio, Cifrian, José, Alpini, Claudia, Olivieri, Ignazio, Blázquez Cañamero, María Ángeles, Rodriguez Cambrón, Ana Belén, Castañeda, Santos, and Cavagna, Lorenzo

Abstract

Objective To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD). Methods The patients were selected from a retrospective large international cohort of ASSD patients regularly followed-up in centres referring to AENEAS collaborative group. Patients were eligible if they had an antisynthetase antibody testing positive in at least two determinations along with arthritis occurring either at ASSD onset (Group 1) or during the course of the disease (Group 2). Results 445 (70%; 334 females, 110 males, 1 transsexual) out of the 636 ASSD we collected had arthritis, in the majority of cases (367, 83%) from disease onset (Group 1). Patients belonging to Group 1 with respect to Group 2 had an arthritis more commonly polyarticular and symmetrical (p=0.015), IgM-Rheumatoid factor positive (p=0.035), erosions at hands and feet plain x-rays (p=0.036) and more commonly satisfying the 1987 revised classification criteria for rheumatoid arthritis (RA) (p=0.004). Features such as Raynaud's phenomenon, mechanic's hands and fever (e.g. accompanying findings) were more frequently reported in Group 2 (p=0.005). Conclusion In ASSD, the timing of appearance with respect to disease onset influences arthritis characteristics. In particular, RA features are more common when arthritis occurs from ASSD onset, suggesting an overlap between RA and ASSD in these patients. When arthritis appears during the follow-up, it is very close to a connective tissue disease-related arthritis. Also, the different prevalence of accompanying features between these two groups is in line with this possibility

Published
2018

19. Primary Respiratory Disease in Patients with Systemic Lupus Erythematosus: Data from the Spanish Rheumatology Society Lupus Registry (RELESSER) Cohort

Author

Narvaez, Javier, Borrell, Helena, Sanchez-Alonso, Fernando, Rua-Figueroa, Iigo, Lopez Longo, Francisco Javier, Galindo, Maria, Calvo-Alen, Jaime, Luis Andreu, Jose, Mariano Andrés, Jose Alegre, Juan, Blanco, Ricardo, Cobo-Ibanez, Tatiana, Bonilla, Gema, Boteanu, Alina, Diez Alvarez, Elvira, Fernandez-Nebro, Antonio, Freire, Mercedes, Gantes, Marian, Garcia La Pena, Paloma, Garcia-Vicuna, Rosario, Hernandez Beirain, Jose, Loreto Horcada, Maria, Ibanez, Jesus, Juan, Antonio, Lozano-Rivas, Nuria, Marenco La Fuente, Jose Luis, Melero Gonzalez, Rafael-Benito, Alberto Montilla-Morales, Carlos, Moreno, Mireia, Olive, Alejandro, Oton Sanchez, Maria Teresa, Pecondon-Espanol, Angela, Ruiz Lucea, Esther, Sanchez Atrio, Ana, Santos-Soler, Gregorio, Toyos, Francisco, Uriarte Isacelaya, Esther, Vazquez Rodriguez, Tomas Ramon, Nolla, Joan Miquel, and Pego-Reigosa, J. M.

Published
2017

25. Genome-wide Association Study Meta-analysis Identifies Five New Loci For Systemic Lupus Erythematosus

Author

Julià, Antonio, Lopez-Longo, Francisco Javier, Pérez Venegas, José J., Bonàs Guarch, Sílvia, Olivé, Àlex, Andreu, José Luís, Aguirre-Zamorano, Maria Ángeles, Vela, Paloma, Nolla Solé, Joan Miquel, Marenco de la Fuente, José Luís, Zea, Antonio, Pego-Reigosa, José María, Freire, Mercedes, Diez, Elvira, Rodriguez-Almaraz, Esther, Carreira, Patricia, Blanco, Ricardo, Martínez Taboada, Víctor, Lopez-Lasanta, María, López Corbeto, Mireia, Mercader, Josep M., Torrents, David, Absher, Devin, Marsal, Sara, and Fernandez-Nebro, Antonio

Subjects
Genòmica, Lupus erythematosus, Malalties autoimmunitàries, Lupus eritematós, Autoimmune diseases, Genomics
Abstract

Background: Systemic lupus erythematosus (SLE) is a common systemic autoimmune disease with a complex genetic inheritance. Genome-wide association studies (GWAS) have significantly increased the number of significant loci associated with SLE risk. To date, however, established loci account for less than 30% of the disease heritability and additional risk variants have yet to be identified. Here we performed a GWAS followed by a meta-analysis to identify new genome-wide significant loci for SLE. Methods: We genotyped a cohort of 907 patients with SLE (cases) and 1524 healthy controls from Spain and performed imputation using the 1000 Genomes reference data. We tested for association using logistic regression with correction for the principal components of variation. Meta-analysis of the association results was subsequently performed on 7,110,321 variants using genetic data from a large cohort of 4036 patients with SLE and 6959 controls of Northern European ancestry. Genetic association was also tested at the pathway level after removing the effect of known risk loci using PASCAL software. Results: We identified five new loci associated with SLE at the genome-wide level of significance (p < 5 x 10(-8)): GRB2, SMYD3, ST8SIA4, LAT2 and ARHGAP27. Pathway analysis revealed several biological processes significantly associated with SLE risk: B cell receptor signaling (p = 5.28 x 10(-6)), CTLA4 co-stimulation during T cell activation (p = 3.06 x 10(-5)), interleukin-4 signaling (p = 3.97 x 10(-5)) and cell surface interactions at the vascular wall (p = 4.63 x 10(-5)). Conclusions: Our results identify five novel loci for SLE susceptibility, and biologic pathways associated via multiple low-effect-size loci.

26. 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 JA, Matos Costa CJ, Molberg O, Andersson H, Codullo V, Mosca M, Barsotti S, Neri R, Scirè C, Govoni M, Furini F, Lopez-Longo FJ, Martinez-Barrio J, Schneider U, Lorenz HM, Doria A, Ghirardello A, Ortego-Centeno N, Confalonieri M, Tomietto P, Pipitone N, Rodriguez Cambron AB, Blázquez Cañamero MÁ, Voll RE, 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 PP, 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 MA, Riccieri V, Conti F, Sebastiani GD, Iuliano A, Emmi G, Cammelli D, Sebastiani M, Manfredi A, Bachiller-Corral J, Sifuentes Giraldo WA, Paolazzi G, Saketkoo LA, Giorgi R, Salaffi F, Cifrian J, Caporali R, Locatelli F, Marchioni E, Pesci A, Dei G, Pozzi MR, 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 MI, Mejia M, Vicente E, Triantafyllias K, Lopez-Mejias R, Matucci-Cerinic M, Selva-O'Callaghan A, Castañeda S, Montecucco C, and Gonzalez-Gay MA

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
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27. Timing of onset affects arthritis presentation pattern in antisyntethase syndrome.

Author

González-Gay MA, Montecucco C, Selva-O'Callaghan A, Trallero-Araguas E, Molberg O, Andersson H, Rojas-Serrano J, Perez-Roman DI, Bauhammer J, Fiehn C, Neri R, Barsotti S, Lorenz HM, Doria A, Ghirardello A, Iannone F, Giannini M, Franceschini F, Cavazzana I, Triantafyllias K, Benucci M, Infantino M, Manfredi M, Conti F, Schwarting A, Sebastiani G, Iuliano A, Emmi G, Silvestri E, Govoni M, Scirè CA, Furini F, Lopez-Longo FJ, Martínez-Barrio J, Sebastiani M, Manfredi A, Bachiller-Corral J, Sifuentes Giraldo WA, Cimmino MA, Cosso C, Belotti Masserini A, Cagnotto G, Codullo V, Romano M, Paolazzi G, Pellerito R, Saketkoo LA, Ortego-Centeno N, Quartuccio L, Batticciotto A, Bartoloni Bocci E, Gerli R, Specker C, Bravi E, Selmi C, Parisi S, Salaffi F, Meloni F, Marchioni E, Pesci A, Dei G, Confalonieri M, Tomietto P, Nuno L, Bonella F, Pipitone N, Mera-Valera A, Perez-Gomez N, Gerzeli S, Lopez-Mejias R, Matos-Costa CJ, Pereira da Silva JA, Cifrian J, Alpini C, Olivieri I, Blázquez Cañamero MÁ, Rodriguez Cambrón AB, Castañeda S, and Cavagna L

Subjects
Adult, Arthritis diagnosis, Arthritis immunology, Autoantibodies blood, Biomarkers blood, Europe epidemiology, Female, Humans, Male, Mexico epidemiology, Middle Aged, Myositis diagnosis, Myositis immunology, Phenotype, Prevalence, Prognosis, Retrospective Studies, Risk Factors, Time Factors, Arthritis epidemiology, Myositis epidemiology
Abstract

Objectives: To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD)., Methods: The patients were selected from a retrospective large international cohort of ASSD patients regularly followed-up in centres referring to AENEAS collaborative group. Patients were eligible if they had an antisynthetase antibody testing positive in at least two determinations along with arthritis occurring either at ASSD onset (Group 1) or during the course of the disease (Group 2)., Results: 445 (70%; 334 females, 110 males, 1 transsexual) out of the 636 ASSD we collected had arthritis, in the majority of cases (367, 83%) from disease onset (Group 1). Patients belonging to Group 1 with respect to Group 2 had an arthritis more commonly polyarticular and symmetrical (p=0.015), IgM-Rheumatoid factor positive (p=0.035), erosions at hands and feet plain x-rays (p=0.036) and more commonly satisfying the 1987 revised classification criteria for rheumatoid arthritis (RA) (p=0.004). Features such as Raynaud's phenomenon, mechanic's hands and fever (e.g. accompanying findings) were more frequently reported in Group 2 (p=0.005)., Conclusions: In ASSD, the timing of appearance with respect to disease onset influences arthritis characteristics. In particular, RA features are more common when arthritis occurs from ASSD onset, suggesting an overlap between RA and ASSD in these patients. When arthritis appears during the follow-up, it is very close to a connective tissue disease-related arthritis. Also, the different prevalence of accompanying features between these two groups is in line with this possibility.

Published
2018

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