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1. 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

2. Video-kapillarmikroskopische Veränderungen bei Patienten mit Antisynthetase-Syndrom. Ergebnisse einer internationalen, multizentrischen Studie des American and European Network of Antisynthetase Syndrome (AENEAS)

Author

Triantafyllias, Konstantinos, Sebastiani, Marco, Manfredi, Andreina, Codullo, Veronica, Schwarting, Andreas, Drott, Ulrich, Delbrück, Christiane, Weinmann-Menke, Julia, Bauhammer, Jutta, Miehle, Nikolaus, Bonella, Francesco, Bergner, Raoul, Specker, Christof, Lütgendorf, Freya, and Cavagna, Lorenzo

Subjects
ddc: 610, 610 Medical sciences, Medicine
Abstract

Einleitung: Das Antisynthetase-Syndrom (ASSD) ist eine Autoimmunerkrankung, die durch die klinische Trias Arthritis, Myositis und interstitielle Lungenerkrankung (ILD) charakterisiert wird. Trotz der Tatsache, dass ein Raynaud-Phänomen ebenso häufig vorhanden ist, sind kapillarmikroskopische[zum vollständigen Text gelangen Sie über die oben angegebene URL], 46. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh), 32. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh), Wissenschaftliche Herbsttagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR)

Published
2019

3. 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, Silva, José António Pereira da, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo Alberto, Govoni, Marcello, Furini, Federica, López-Longo, Francisco Javier, Martínez-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, 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, Delbrück, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Berzolari, Francesca Gigli, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Gomez, Nair Perez, 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, Jörg Hans Wilhelm, 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, López-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O’Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, González-Gay, Miguel Ángel, Cavagna, Lorenzo, Trallero-Araguás, Ernesto, Meloni, Federica, Cavazzana, Ilaria, Rojas-Serrano, Jorge, Feist, Eugen, Zanframundo, Giovanni, Morandi, Valentina, Meyer, Alain, Silva, José António Pereira da, Matos Costa, Carlo Jorge, Molberg, Oyvind, Andersson, Helena, Codullo, Veronica, Mosca, Marta, Barsotti, Simone, Neri, Rossella, Scirè, Carlo Alberto, Govoni, Marcello, Furini, Federica, López-Longo, Francisco Javier, Martínez-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, 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, Delbrück, Christiane, Bauhammer, Jutta, Cagnotto, Giovanni, Vancheri, Carlo, Sambataro, Gianluca, De Langhe, Ellen, Sainaghi, Pier Paolo, Monti, Cristina, Berzolari, Francesca Gigli, Romano, Mariaeva, Bonella, Francesco, Specker, Christof, Schwarting, Andreas, Villa Blanco, Ignacio, Selmi, Carlo, Ceribelli, Angela, Nuno, Laura, Mera-Varela, Antonio, Gomez, Nair Perez, 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, Jörg Hans Wilhelm, 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, López-Mejias, Raquel, Matucci-Cerinic, Marco, Selva-O’Callaghan, Albert, Castañeda, Santos, Montecucco, Carlomaurizio, and González-Gay, Miguel Ángel

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

5. Suchtverhalten als Problemfeld in der Transition

Author

Braner, Axel, Delbrück, Christiane, and Burkhardt, Harald Louis

Subjects
Suchtverhalten, ddc: 610, Transition, Adoleszenz, 610 Medical sciences, Medicine
Abstract

Einleitung: Der Betreuungswechsel vom Kinderrheumatologen zum internistischen Rheumatologen, die so genannte Transition, stellt eine extrem vulnerable Phase für die Betreuung der adoleszenten Patientendar. Wesentliche Änderungen ergeben sich u.a. in der Betreuungsstruktur wie die Übernahme[zum vollständigen Text gelangen Sie über die oben angegebene URL], 44. Kongress der Deutschen Gesellschaft für Rheumatologie (DGRh); 30. Jahrestagung der Deutschen Gesellschaft für Orthopädische Rheumatologie (DGORh); 26. Jahrestagung der Gesellschaft für Kinder- und Jugendrheumatologie (GKJR)

Published
2016
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6. Nailfold Capillaroscopy Characteristics of Antisynthetase Syndrome and Possible Clinical Associations: Results of a Multicenter International Study.

Author

Sebastiani M, Triantafyllias K, Manfredi A, González-Gay MA, Palmou-Fontana N, Cassone G, Drott U, Delbrück C, Rojas-Serrano J, Bertolazzi C, Nuño L, Giannini M, Iannone F, Vicente EF, Castañeda S, Selva-O'Callaghan A, Trallero Araguas E, Emmi G, Iuliano A, Bauhammer J, Miehle N, Parisi S, Cavagna L, Codullo V, Montecucco C, Lopez-Longo FJ, Martínez-Barrio J, Nieto-González JC, Vichi S, Confalonieri M, Tomietto P, Bergner R, Sulli A, Bonella F, Furini F, Scirè CA, Bortoluzzi A, Specker C, Barsotti S, Neri R, Mosca M, Caproni M, Weinmann-Menke J, Schwarting A, Smith V, and Cutolo M

Subjects
Adult, Aged, Amino Acyl-tRNA Synthetases immunology, Antibodies, Antinuclear blood, Capillaries diagnostic imaging, Female, Follow-Up Studies, Humans, Male, Middle Aged, Nails blood supply, Raynaud Disease diagnostic imaging, Raynaud Disease immunology, Retrospective Studies, Microscopic Angioscopy methods, Myositis diagnostic imaging, Myositis immunology
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
Full Text
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