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1. Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies

Author

Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Meloni F., Meyer A., Sambataro G., Belliato M., De Langhe E., Cavazzana I., Pipitone N., Triantafyllias K., Mosca M., Barsotti S., Zampogna G., Biglia A., Emmi G., De Visser M., Van Der Kooi A., Parronchi P., Hirschi S., da Silva J. A. P., Scire C. A., Furini F., Giannini M., Martinez Gonzalez O., Damian L., Piette Y., Smith V., Mera-Valera A., Bachiller-Corral J., Cabezas Rodriguez I., Brandy-Garcia A. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., Gonzalez-Gay M. A., Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Cavagna L., Meloni F., Meyer A., Sambataro G., Belliato M., De Langhe E., Cavazzana I., Pipitone N., Triantafyllias K., Mosca M., Barsotti S., Zampogna G., Biglia A., Emmi G., De Visser M., Van Der Kooi A., Parronchi P., Hirschi S., da Silva J. A. P., Scire C. A., Furini F., Giannini M., Martinez Gonzalez O., Damian L., Piette Y., Smith V., Mera-Valera A., Bachiller-Corral J., Cabezas Rodriguez I., Brandy-Garcia A. M., Maurier F., Perrin J., Gonzalez-Moreno J., Drott U., Delbruck C., Schwarting A., Arrigoni E., Sebastiani G. D., Iuliano A., Nannini C., Quartuccio L., Rodriguez Cambron A. B., Blazquez Canamero M. A., Villa Blanco I., Cagnotto G., Pesci A., Luppi F., Dei G., Romero Bueno F. I., Franceschini F., Chiapparoli I., Zanframundo G., Lettieri S., De Stefano L., Cutolo M., Mathieu A., Piga M., Prieto-Gonzalez S., Moraes-Fontes M. F., Fonseca J. E., Jovani V., Riccieri V., Santaniello A., Montfort S., Bilocca D., Erre G. L., Bartoloni E., Gerli R., Monti M. C., Lorenz H. M., Sambataro D., Bellando Randone S., Schneider U., Valenzuela C., Lopez-Mejias R., Cifrian J., Mejia M., Gonzalez Perez M. -I., Wendel S., Fornaro M., De Luca G., Orsolini G., Rossini M., Dieude P., Knitza J., Castaneda S., Voll R. E., Rojas-Serrano J., Valentini A., Vancheri C., Matucci-Cerinic M., Feist E., Codullo V., Iannone F., Distler J. H., Montecucco C., and Gonzalez-Gay M. A.

Abstract

Objective To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods We conducted a multicentre, international, retrospective cohort study. Results 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusion The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Published
2022

2. Intravenous Immunoglobulin is Associated with Lower Rates of Reinfection in Solid Organ Recipients with Infection and Secondary Antibody Deficiency: A Multicenter Randomized Clinical Trial

Author

Carbone, J., primary, Montanchez, J., additional, Cifrian, J., additional, Zatarain, E., additional, Laporta, R., additional, Ussetti, P., additional, Bravo, C., additional, Lopez, S., additional, Salcedo, M., additional, Rodriguez-Ferrero, M., additional, Gonzalez-Costello, J., additional, Sabe, N., additional, Segovia, J., additional, Gomez-Bueno, M., additional, De Pablos, A., additional, Sousa, I., additional, Alarcon, A., additional, Ezzahouri, I., additional, and Sarmiento, E., additional

Published
2022
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4. Preliminary Results of a Randomized Clinical Trial of Intravenous Immunoglobulin in Solid Organ Recipients with Severe Infection and Secondary Antibody Deficiency

Author

Carbone, J., primary, Montanchez, J., additional, Cifrian, J., additional, Laporta, R., additional, Ussetti, P., additional, Zatarain, E., additional, Sousa, I., additional, Muñoz, P., additional, Ezzahouri, I., additional, Salcedo, M., additional, Rodriguez-Ferrero, M., additional, Bravo, C., additional, Segovia, J., additional, Gomez-Bueno, M., additional, Fernandez-Sabe, N., additional, Gonzalez-Costello, J., additional, De Pablos, A., additional, and Sarmiento, E., additional

Published
2020
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5. Perspectives on Scedosporium species and Lomentospora prolificans in lung transplantation: Results of an international practice survey from ESCMID fungal infection study group and study group for infections in compromised hosts, and European Confederation of Medical Mycology

Author

Rammaert, B., Puyade, M., Cornely, O. A., Seidel, D., Grossi, P., Husain, S., Picard, C., Lass-Florl, C., Manuel, O., Le Pavec, J., Lortholary, O., Nagel, C., Westall, G., Morrissey, O., Chambers, D., Eschertzhuber, S., Coussement, J., Knoop, C., Vos, R., Dupont, L., Dumonceaux, M., Campos, S. V., Kabbani, D., Cervera, C., Luong, M. -L., Blanchard, E., Senechal, A., Pavec, J. L., Brugiere, O., Boussaud, V., Guillemain, R., Bervar, J. -F., Claustre, J., Haloun, A., Hirschi, S., Reynaud, M., Kneidinger, N., Gottlieb, J., Roilides, E., Zarrinfar, H., Rosso, L., Morlacchi, L. C., Dell'Amore, A., Loy, M., Santos, C. O. D., Rello, J., Monforte, V., Martin-Gomez, M. T., Medrano, F. L., Fernandez-Ruiz, M., Sole, A., Cifrian, J. M., Neofytos, D., Mueller, N., Benden, C., Brill, A. K., Kiyan, E., Gould, K., Gkrania-Klotsas, E., David, M., Weigt, S., Kwak, E. J., Silveira, F., Hadjiliadis, D., Baddley, J., Danziger-Isakov, L., Bhorade, S., Ison, M., Wolfe, C., Aslam, S., Budem, M., Musetti, A., Centre hospitalier universitaire de Poitiers (CHU Poitiers), Université de Poitiers - Faculté de Médecine et de Pharmacie, Université de Poitiers, Pharmacologie des anti-infectieux (PHAR), Université de Poitiers-Institut National de la Santé et de la Recherche Médicale (INSERM), University Hospital of Cologne [Cologne], Cologne Excellence Cluster on Cellular Stress Responses in Aging-Associated Diseases - CECAD [Cologne, Germany] (Institute for Genetics), University of Cologne, German Centre for Infection Research (DZIF), Universitá degli Studi dell’Insubria = University of Insubria [Varese] (Uninsubria), University Health Network, Hôpital Foch [Suresnes], Leopold Franzens Universität Innsbruck - University of Innsbruck, Centre Hospitalier Universitaire Vaudois [Lausanne] (CHUV), Centre Chirurgical Marie Lannelongue (CCML), Hypertension arterielle pulmonaire physiopathologie et innovation thérapeutique, Centre Chirurgical Marie Lannelongue (CCML)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Paris-Sud - Paris 11 - Faculté de médecine (UP11 UFR Médecine), Université Paris-Sud - Paris 11 (UP11), Université Paris-Saclay, Centre d'infectiologie Necker-Pasteur [CHU Necker], Institut Pasteur [Paris] (IP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Mycologie moléculaire - Molecular Mycology, Institut Pasteur [Paris] (IP)-Centre National de la Recherche Scientifique (CNRS), Centre National de Référence Mycologie et Antifongiques-Mycologie Moléculaire (CNRMA), Institut Pasteur [Paris] (IP), The authors thank all the people who helped to collect mycological data and to contact transplant centers: Alejandro Mario Bertolotti (Buenos Aires, Argentina), Claire Heney (Brisbane, Australia), Carlota Montesinos (Brussels, Belgium), Katrien Lagrou (Leuven, Belgium), Youri Gluczynski (Yvoir, Belgium), Sandrine Houze (Paris, France), Eric Dannaoui (Paris, France), Florent Morio (Nantes, France), Murielle Cornet (Grenoble, France), Valérie Bru (Strasbourg, France), Stéphane Ranque (Marseille, France), Emilie Cardot (Suresnes, France), Ludwig Sedlacek (Hannover, Germany), Maurizio Sanguinetti (Roma, Italy), Ana Alastruey (Madrid, Spain), Konrad Muehlethaler (Bern, Switzerland), Kevin Alby (Philadelphia, USA), Malcom Richardson (Manchester, UK), the members of the ESCMID Fungal Infection Study Group (EFISG) and Study Group for Infections in Compromised Hosts (ESGICH) and European Confederation of Medical Mycology (ECMM) who helped to promote the study, Jeffrey Arsham for editing our original English language manuscript., The SCEDO‐LUNG collaborative group collected the data., University of Insubria, Varese, University of Innsbruck, Centre chirurgical Marie Lannelongue, Centre chirurgical Marie Lannelongue-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut Pasteur [Paris], Institut Pasteur [Paris]-Centre National de la Recherche Scientifique (CNRS), and Institut Pasteur [Paris]

Subjects
Internationality, [SDV]Life Sciences [q-bio], invasive fungal disease, lomentosporiosis, scedosporiosis, solid organ transplantation, Ascomycota, Humans, Lung Transplantation, Mycoses, Prospective Studies, Respiratory Tract Infections, Scedosporium, Surveys and Questionnaires, Disease Management, Immunocompromised Host, MESH: Mycoses/epidemiology, [SDV.MHEP.MI]Life Sciences [q-bio]/Human health and pathology/Infectious diseases, MESH: Mycoses/etiology, MESH: Surveys and Questionnaires, [SDV.MP.MYC]Life Sciences [q-bio]/Microbiology and Parasitology/Mycology, MESH: Respiratory Tract Infections/etiology, MESH: Humans, MESH: Respiratory Tract Infections/microbiology, MESH: Scedosporium/isolation & purification, [SDV.SP]Life Sciences [q-bio]/Pharmaceutical sciences, MESH: Lung Transplantation/adverse effects, MESH: Prospective Studies, MESH: Disease Management, MESH: Internationality, MESH: Ascomycota/isolation & purification, MESH: Immunocompromised Host
Abstract

International audience; Background: Scedosporium species and Lomentospora prolificans (S/L) are the second most common causes of invasive mold infections following Aspergillus in lung transplant recipients.Methods: We assessed the current practices on management of S/L colonization/infection of the lower respiratory tract before and after lung transplantation in a large number of lung transplant centers through an international practice survey from October 2016 to March 2017.Results: A total of 51 respondents from 45 lung transplant centers (17 countries, 4 continents) answered the survey (response rate 58%). S/L colonization was estimated to be detected in candidates by 48% of centers. Only 18% of the centers used a specific medium to detect S/L colonization. Scedosporium spp. colonization was a contraindication to transplantation in 10% of centers whereas L prolificans was a contraindication in 31%; 22% of centers declared having had 1-5 recipients infected with S/L in the past 5 years.Conclusions: This survey gives an overview of the current practices regarding S/L colonization and infection in lung transplant centers worldwide and underscores the need of S/L culture procedure standardization before implementing prospective studies.

Published
2019

6. 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

8. 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

9. Defining Severe Secondary Antibody Deficiency in Heart and Lung Transplantation

Author

Carbone, J., primary, Sousa, I., additional, Cifrian, J., additional, Gomez-Sanchez, M., additional, Laporta, R., additional, Crespo-Leiro, M., additional, Bravo, C., additional, Almenar, L., additional, Sole, A., additional, Rabago, G., additional, Segovia, J., additional, de Pablos, A., additional, Lopez, J., additional, Mirabet, S., additional, Navarro, J., additional, and Sarmiento, E., additional

Published
2018
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13. Prophylaxis with nebulized liposomal amphotericin B for Aspergillus infection in lung transplant patients does not cause changes in the lipid content of pulmonary surfactant

Author

Monforte V, López-Sánchez A, Zurbano F, Ussetti P, Solé A, Casals C, Cifrian J, de Pablos A, Bravo C, and Román A

Abstract

Prophylaxis with inhaled liposomal amphotericin B has proven to be safe and effective for preventing infection due to Aspergillus spp in lung transplant recipients. However, the liposome contains a large quantity of phospholipids, and inhalation of these substances could potentially change the composition of pulmonary surfactant. The aim of this study was to determine the lipid composition of pulmonary surfactant in patients receiving inhaled liposomal amphotericin B prophylaxis.

Published
2013

20. Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies

Author

Lorenzo, Cavagna, Federica, Meloni, Alain, Meyer, Gianluca, Sambataro, Mirko, Belliato, Ellen De Langhe, Cavazzana, Ilaria, Nicolò, Pipitone, Konstantinos, Triantafyllias, Marta, Mosca, Simone, Barsotti, Giuseppe, Zampogna, Alessandro, Biglia, Giacomo, Emmi, Marianne De Visser, Anneke Van Der Kooi, Paola, Parronchi, Sandrine, Hirschi, Jose Antonio Pereira da Silva, Carlo Alberto Scirè, Federica, Furini, Margherita, Giannini, Olga Martinez Gonzalez, Laura, Damian, Yves, Piette, Vanessa, Smith, Antonio, Mera-Valera, Javier, Bachiller-Corral, Ivan Cabezas Rodriguez, Anahy, M Brandy-Garcia, François, Maurier, Julie, Perrin, Juan, Gonzalez-Moreno, Ulrich, Drott, Christiane, Delbruck, Andreas, Schwarting, Eugenio, Arrigoni, Gian Domenico Sebastiani, Annamaria, Iuliano, Carlotta, Nannini, Luca, Quartuccio, Ana, B Rodriguez Cambron, Maria, Á Blázquez Cañamero, Ignacio Villa Blanco, Giovanni, Cagnotto, Alberto, Pesci, Francesco, Luppi, Giulia, Dei, Fredeswinda Isabel Romero Bueno, Franceschini, Franco, Ilaria, Chiapparoli, Giovanni, Zanframundo, Sara, Lettieri, Ludovico De Stefano, Maurizio, Cutolo, Alessandro, Mathieu, Matteo, Piga, Sergio, Prieto-González, Maria Francisca Moraes-Fontes, Joao Eurico Fonseca, Vega, Jovani, Valeria, Riccieri, Alessandro, Santaniello, Stephen, Montfort, David, Bilocca, Gian Luca Erre, Elena, Bartoloni, Roberto, Gerli, M Cristina Monti, Hanns, M Lorenz, Domenico, Sambataro, Silvia Bellando Randone, Udo, Schneider, Claudia, Valenzuela, Raquel, Lopez-Mejias, Jose, Cifrian, Mayra, Mejia, Monserrat-Ixchel Gonzalez Perez, Sarah, Wendel, Marco, Fornaro, Giacomo De Luca, Giovanni, Orsolini, Maurizio, Rossini, Philippe, Dieude, Johannes, Knitza, Santos, Castañeda, Reinhard, E Voll, Jorge, Rojas-Serrano, Adele, Valentini, Carlo, Vancheri, Marco, Matucci-Cerinic, Eugen, Feist, Veronica, Codullo, Florenzo, Iannone, Jorg, H Distler, Carlomaurizio, Montecucco, Miguel, A Gonzalez-Gay, AENEAS collaborative group, Neurology, ANS - Neuroinfection & -inflammation, AII - Inflammatory diseases, EURO-NMD, Cavagna, L, Meloni, F, Meyer, A, Sambataro, G, Belliato, M, De Langhe, E, Cavazzana, I, Pipitone, N, Triantafyllias, K, Mosca, M, Barsotti, S, Zampogna, G, Biglia, A, Emmi, G, De Visser, M, Van Der Kooi, A, Parronchi, P, Hirschi, S, da Silva, J, Scire, C, Furini, F, Giannini, M, Martinez Gonzalez, O, Damian, L, Piette, Y, Smith, V, Mera-Valera, A, Bachiller-Corral, J, Cabezas Rodriguez, I, Brandy-Garcia, A, Maurier, F, Perrin, J, Gonzalez-Moreno, J, Drott, U, Delbruck, C, Schwarting, A, Arrigoni, E, Sebastiani, G, Iuliano, A, Nannini, C, Quartuccio, L, Rodriguez Cambron, A, Blazquez Canamero, M, Villa Blanco, I, Cagnotto, G, Pesci, A, Luppi, F, Dei, G, Romero Bueno, F, Franceschini, F, Chiapparoli, I, Zanframundo, G, Lettieri, S, De Stefano, L, Cutolo, M, Mathieu, A, Piga, M, Prieto-Gonzalez, S, Moraes-Fontes, M, Fonseca, J, Jovani, V, Riccieri, V, Santaniello, A, Montfort, S, Bilocca, D, Erre, G, Bartoloni, E, Gerli, R, Monti, M, Lorenz, H, Sambataro, D, Bellando Randone, S, Schneider, U, Valenzuela, C, Lopez-Mejias, R, Cifrian, J, Mejia, M, Gonzalez Perez, M, Wendel, S, Fornaro, M, De Luca, G, Orsolini, G, Rossini, M, Dieude, P, Knitza, J, Castaneda, S, Voll, R, Rojas-Serrano, J, Valentini, A, Vancheri, C, Matucci-Cerinic, M, Feist, E, Codullo, V, Iannone, F, Distler, J, Montecucco, C, Gonzalez-Gay, M, Repositório da Universidade de Lisboa, Cavagna, Lorenzo, Meloni, Federica, Meyer, Alain, Sambataro, Gianluca, Belliato, Mirko, De Langhe, Ellen, Cavazzana, Ilaria, Pipitone, Nicolò, Triantafyllias, Konstantino, Mosca, Marta, Barsotti, Simone, Zampogna, Giuseppe, Biglia, Alessandro, Emmi, Giacomo, De Visser, Marianne, Van Der Kooi, Anneke, Parronchi, Paola, Hirschi, Sandrine, da Silva, Jose Antonio Pereira, Scirè, Carlo Alberto, Furini, Federica, Giannini, Margherita, Martinez Gonzalez, Olga, Damian, Laura, Piette, Yve, Smith, Vanessa, Mera-Valera, Antonio, Bachiller-Corral, Javier, Cabezas Rodriguez, Ivan, Brandy-Garcia, Anahy M, Maurier, Françoi, Perrin, Julie, Gonzalez-Moreno, Juan, Drott, Ulrich, Delbruck, Christiane, Schwarting, Andrea, Arrigoni, Eugenio, Sebastiani, Gian Domenico, Iuliano, Annamaria, Nannini, Carlotta, Quartuccio, Luca, Rodriguez Cambron, Ana B, Blázquez Cañamero, Maria Á, Villa Blanco, Ignacio, Cagnotto, Giovanni, Pesci, Alberto, Luppi, Francesco, Dei, Giulia, Romero Bueno, Fredeswinda Isabel, Franceschini, Franco, Chiapparoli, Ilaria, Zanframundo, Giovanni, Lettieri, Sara, De Stefano, Ludovico, Cutolo, Maurizio, Mathieu, Alessandro, Piga, Matteo, Prieto-González, Sergio, Moraes-Fontes, Maria Francisca, Fonseca, Joao Eurico, Jovani, Vega, Riccieri, Valeria, Santaniello, Alessandro, Montfort, Stephen, Bilocca, David, Erre, Gian Luca, Bartoloni, Elena, Gerli, Roberto, Monti, M Cristina, Lorenz, Hanns M, Sambataro, Domenico, Bellando Randone, Silvia, Schneider, Udo, Valenzuela, Claudia, Lopez-Mejias, Raquel, Cifrian, Jose, Mejia, Mayra, Gonzalez Perez, Monserrat-Ixchel, Wendel, Sarah, Fornaro, Marco, De Luca, Giacomo, Orsolini, Giovanni, Rossini, Maurizio, Dieude, Philippe, Knitza, Johanne, Castañeda, Santo, Voll, Reinhard E, Rojas-Serrano, Jorge, Valentini, Adele, Vancheri, Carlo, Matucci-Cerinic, Marco, Feist, Eugen, Codullo, Veronica, Iannone, Florenzo, Distler, Jorg H, Montecucco, Carlomaurizio, and Gonzalez-Gay, Miguel A

Subjects
Lung Diseases, Interferon-Induced Helicase, IFIH1, rapidly progressive interstitial lung diseases, idiopathic inflammatory myopathies, idiopathic inflammatory myopathie, Immunology, Middle Aged, Prognosis, Dermatomyositis, rapidly progressive interstitial lung disease, Rheumatology, melanoma differentiation-associated protein 5 antibody, rapidly progressive interstitial lung diseases, idiopathic inflammatory myopathies, Humans, Immunology and Allergy, Female, Lung Diseases, Interstitial, Interferon-Induced Helicase, Interstitial, melanoma differentiation-associated protein 5 antibody, Autoantibodies, Retrospective Studies, IFIH1
Abstract

© Copyright Clinical and Experimental Rheumatology 2022., Objectives: To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies. Methods: We conducted a multicentre, international, retrospective cohort study. Results: 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD. Conclusions: The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Published
2022

21. 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

22. 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

23. Agreement between local and central anti-synthetase antibodies detection: results from the Classification Criteria of Anti-Synthetase Syndrome project biobank.

Author

Loganathan A, Zanframundo G, Yoshida A, Faghihi-Kashani S, Bauer Ventura I, Dourado E, Bozan F, Sambataro G, Yamano Y, Bae SS, Lim D, Ceribelli A, Isailovic N, Selmi C, Fertig N, Bravi E, Kaneko Y, Saraiva AP, Jovani V, Bachiller-Corral J, Cifrian J, Mera-Varela A, Moghadam-Kia S, Wolff V, Campagne J, Meyer A, Giannini M, Triantafyllias K, Knitza J, Gupta L, Molad Y, Iannone F, Cavazzana I, Piga M, De Luca G, Tansley S, Bozzalla-Cassione E, Bonella F, Corte TJ, Doyle TJ, Fiorentino D, Gonzalez-Gay MA, Hudson M, Kuwana M, Lundberg IE, Mammen AL, McHugh NJ, Miller FW, Montecucco C, Oddis CV, Rojas-Serrano J, Schmidt J, Scirè CA, Selva-O'Callaghan A, Werth VP, Alpini C, Bozzini S, Cavagna L, and Aggarwal R

Subjects
Humans, Ligases, Reproducibility of Results, Biological Specimen Banks, Autoantibodies, Amino Acyl-tRNA Synthetases, Myositis diagnosis
Abstract

Objectives: The CLASS (Classification Criteria of Anti-Synthetase Syndrome) project is a large international multicentre study that aims to create the first data-driven anti-synthetase syndrome (ASSD) classification criteria. Identifying anti-aminoacyl tRNA synthetase antibodies (anti-ARS) is crucial for diagnosis, and several commercial immunoassays are now available for this purpose. However, using these assays risks yielding false-positive or false-negative results, potentially leading to misdiagnosis. The established reference standard for detecting anti-ARS is immunoprecipitation (IP), typically employed in research rather than routine autoantibody testing. We gathered samples from participating centers and results from local anti-ARS testing. As an "ad-interim" study within the CLASS project, we aimed to assess how local immunoassays perform in real-world settings compared to our central definition of anti-ARS positivity., Methods: We collected 787 serum samples from participating centres for the CLASS project and their local anti-ARS test results. These samples underwent initial central testing using RNA-IP. Following this, the specificity of ARS was reconfirmed centrally through ELISA, line-blot assay (LIA), and, in cases of conflicting results, protein-IP. The sensitivity, specificity, positive likelihood ratio and positive and negative predictive values were evaluated. We also calculated the inter-rater agreement between central and local results using a weighted κ co-efficient., Results: Our analysis demonstrates that local, real-world detection of anti-Jo1 is reliable with high sensitivity and specificity with a very good level of agreement with our central definition of anti-Jo1 antibody positivity. However, the agreement between local immunoassay and central determination of anti-non-Jo1 antibodies varied, especially among results obtained using local LIA, ELISA and "other" methods., Conclusions: Our study evaluates the performance of real-world identification of anti-synthetase antibodies in a large cohort of multi-national patients with ASSD and controls. Our analysis reinforces the reliability of real-world anti-Jo1 detection methods. In contrast, challenges persist for anti-non-Jo1 identification, particularly anti-PL7 and rarer antibodies such as anti-OJ/KS. Clinicians should exercise caution when interpreting anti-synthetase antibodies, especially when commercial immunoassays test positive for non-anti-Jo1 antibodies.

Published
2024
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24. Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies.

Author

Cavagna L, Meloni F, Meyer A, Sambataro G, Belliato M, De Langhe E, Cavazzana I, Pipitone N, Triantafyllias K, Mosca M, Barsotti S, Zampogna G, Biglia A, Emmi G, De Visser M, Van Der Kooi A, Parronchi P, Hirschi S, da Silva JAP, Scirè CA, Furini F, Giannini M, Martinez Gonzalez O, Damian L, Piette Y, Smith V, Mera-Valera A, Bachiller-Corral J, Cabezas Rodriguez I, Brandy-Garcia AM, Maurier F, Perrin J, Gonzalez-Moreno J, Drott U, Delbruck C, Schwarting A, Arrigoni E, Sebastiani GD, Iuliano A, Nannini C, Quartuccio L, Rodriguez Cambron AB, Blázquez Cañamero MÁ, Villa Blanco I, Cagnotto G, Pesci A, Luppi F, Dei G, Romero Bueno FI, Franceschini F, Chiapparoli I, Zanframundo G, Lettieri S, De Stefano L, Cutolo M, Mathieu A, Piga M, Prieto-González S, Moraes-Fontes MF, Fonseca JE, Jovani V, Riccieri V, Santaniello A, Montfort S, Bilocca D, Erre GL, Bartoloni E, Gerli R, Monti MC, Lorenz HM, Sambataro D, Bellando Randone S, Schneider U, Valenzuela C, Lopez-Mejias R, Cifrian J, Mejia M, Gonzalez Perez MI, Wendel S, Fornaro M, De Luca G, Orsolini G, Rossini M, Dieude P, Knitza J, Castañeda S, Voll RE, Rojas-Serrano J, Valentini A, Vancheri C, Matucci-Cerinic M, Feist E, Codullo V, Iannone F, Distler JH, Montecucco C, and Gonzalez-Gay MA

Subjects
Autoantibodies, Female, Humans, Interferon-Induced Helicase, IFIH1, Middle Aged, Prognosis, Retrospective Studies, Dermatomyositis complications, Lung Diseases, Interstitial drug therapy
Abstract

Objectives: To define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies., Methods: We conducted a multicentre, international, retrospective cohort study., Results: 149 anti-MDA5 positive patients (median onset age 53 years, median disease duration 18 months), mainly females (100, 67%), were included. Dermatomyositis (64, 43%) and amyopathic dermatomyositis (47, 31%), were the main diagnosis; 15 patients (10%) were classified as interstitial pneumonia with autoimmune features (IPAF) and 7 (5%) as rheumatoid arthritis. The main clinical findings observed were myositis (84, 56%), interstitial lung disease (ILD) (108, 78%), skin lesions (111, 74%), and arthritis (76, 51%). The onset of these manifestations was not concomitant in 74 cases (50%). Of note, 32 (21.5%) patients were admitted to the intensive care unit for rapidly progressive-ILD, which occurred in median 2 months from lung involvement detection, in the majority of cases (28, 19%) despite previous immunosuppressive treatment. One-third of patients (47, 32% each) was ANA and anti-ENA antibodies negative and a similar percentage was anti-Ro52 kDa antibodies positive. Non-specific interstitial pneumonia (65, 60%), organising pneumonia (23, 21%), and usual interstitial pneumonia-like pattern (14, 13%) were the main ILD patterns observed. Twenty-six patients died (17%), 19 (13%) had a rapidly progressive-ILD., Conclusions: The clinical spectrum of the anti-MDA5 antibodies-related disease is heterogeneous. Rapidly-progressive ILD deeply impacts the prognosis also in non-Asian patients, occurring early during the disease course. Anti-MDA5 antibody positivity should be considered even when baseline autoimmune screening is negative, anti-Ro52 kDa antibodies are positive, and radiology findings show a NSIP pattern.

Published
2022
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25. Myeloid-Derived Suppressor Cells Are Increased in Lung Transplant Recipients and Regulated by Immunosuppressive Therapy.

Author

Iglesias-Escudero M, Segundo DS, Merino-Fernandez D, Mora-Cuesta VM, Lamadrid P, Alonso-Peña M, Raso S, Iturbe D, Fernandez-Rozas S, Cifrian J, and López-Hoyos M

Subjects
Humans, Immune Tolerance, Immunosuppression Therapy, Immunosuppressive Agents pharmacology, Immunosuppressive Agents therapeutic use, Lung, Transplant Recipients, Myeloid-Derived Suppressor Cells
Abstract

Lung transplantation remains as a primary treatment for end-stage lung diseases. Although remarkable improvement has been achieved due to the immunosuppressive protocols, long-term survival for lung transplant recipients (LTR) is still limited. In the last few decades, an increasing interest has grown in the study of dysregulation of immune mechanisms underlying allograft failure. In this regard, myeloid-derived suppressor cells (MDSCs) could play an important role in the promotion of graft tolerance due to their immune regulatory function. Here, we describe for the first time circulating subsets MDSCs from LTR at several time points and we evaluate the relationship of MDSCs with sort-term lung transplant outcomes. Although no effect of MDSCs subsets on short-term clinical events was observed, our results determine that Mo-MDSCs frequencies are increased after acute cellular rejection (ACR), suggesting a possible role for Mo-MDSCs in the development of chronic lung allograft dysfunction (CLAD). Therefore, whether MDSCs subsets play a role as biomarkers of chronic rejection remains unknown and requires further investigations. Also, the effects of the different immunosuppressive treatments on these subpopulations remain under research and further studies are needed to establish to what extend MDSCs immune modulation could be responsible for allograft acceptance., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Iglesias-Escudero, Segundo, Merino-Fernandez, Mora-Cuesta, Lamadrid, Alonso-Peña, Raso, Iturbe, Fernandez-Rozas, Cifrian and López-Hoyos.)

Published
2022
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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. Monitoring of early humoral immunity to identify lung recipients at risk for development of serious infections: A multicenter prospective study.

Author

Sarmiento E, Cifrian J, Calahorra L, Bravo C, Lopez S, Laporta R, Ussetti P, Sole A, Morales C, de Pablos A, Jaramillo M, Ezzahouri I, García S, Navarro J, Lopez-Hoyos M, and Carbone J

Subjects
Adult, Agammaglobulinemia diagnosis, Agammaglobulinemia immunology, Aged, Antibody Formation immunology, B-Cell Activating Factor blood, Bacterial Infections diagnosis, Bacterial Infections immunology, Biomarkers blood, Cross Infection diagnosis, Cytomegalovirus Infections diagnosis, Cytomegalovirus Infections immunology, Early Diagnosis, Female, Humans, Male, Middle Aged, Mycoses diagnosis, Mycoses immunology, Opportunistic Infections diagnosis, Prospective Studies, Risk Factors, Cross Infection immunology, Immunity, Humoral immunology, Lung Transplantation, Monitoring, Physiologic, Opportunistic Infections immunology
Abstract

Background: Infection is still a leading cause of death during the first year after lung transplantation. We performed a multicenter study among teaching hospitals to assess monitoring of early humoral immunity as a means of identifying lung recipients at risk of serious infections., Methods: We prospectively analyzed 82 adult lung recipients at 5 centers in Spain. Data were collected before transplantation and at 7 and 30 days after transplantation. Biomarkers included IgG, IgM, IgA, complement factors C3 and C4, titers of antibodies to pneumococcal polysaccharide antigens (IgG, IgA, IgM) and antibodies to cytomegalovirus (IgG), and serum B-cell activating factor (BAFF) levels. The clinical follow-up period lasted 6 months. Clinical outcomes were bacterial infections requiring intravenous anti-microbial agents, cytomegalovirus (CMV) disease, and fungal infections requiring therapy., Results: We found that 33 patients (40.2%) developed at least 1 serious bacterial infection, 8 patients (9.8%) had CMV disease, and 10 patients (12.2%) had fungal infections. Lower IgM antibody levels against pneumococcal polysaccharide antigens at Day 7 (defined as <5 mg/dl) were a risk factor for serious bacterial infection (adjusted odds ratio [OR] 3.96; 95% confidence interval [CI] 1.39 to 11.26; p = 0.0099). At Day 7 after transplantation, IgG hypogammaglobulinemia (defined as IgG <600 mg/dl) was associated with a higher risk of CMV disease (after adjustment for CMV mismatch: OR 8.15; 95% CI 1.27 to 52.55; p = 0.028) and fungal infection (adjusted OR 8.03, 95% CI 1.51 to 42.72; p = 0.015). Higher BAFF levels before transplantation were associated with a higher rate of development of serious bacterial infection and acute cellular rejection., Conclusion: Early monitoring of specific humoral immunity parameters proved useful for the identification of lung recipients who are at risk of serious infections., (Copyright © 2018 International Society for the Heart and Lung Transplantation. Published by Elsevier Inc. All rights reserved.)

Published
2018
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28. 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

29. Recommendations on the use of everolimus in lung transplantation.

Author

de Pablo A, Santos F, Solé A, Borro JM, Cifrian JM, Laporta R, Monforte V, Román A, de la Torre M, Ussetti P, and Zurbano F

Subjects
Antineoplastic Agents, Everolimus, Humans, Immunosuppressive Agents therapeutic use, Sirolimus therapeutic use, Graft Rejection prevention & control, Immunosuppression Therapy methods, Lung Transplantation, Sirolimus analogs & derivatives
Abstract

The antiproliferative effect of everolimus provides a therapeutic option in the immunosuppression therapy of lung transplantation, by reducing both the risk of acute rejection and the process of progressive fibrosis that determines chronic graft rejection. However, few data on the use of everolimus in lung transplantation have been published to date, and the specific indications of the drug, along with the most adequate time for its introduction or dosing, have not been defined yet. The aim of this article is to propose recommendations for the use of everolimus in lung transplant recipients, including indications, dosing schedules and the use of concomitant immunosuppression. This consensus document has been developed by experts of all the Spanish lung transplant groups from the review of the existing literature and the clinical experience., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Published
2013
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30. Polymorphisms in the serotonin transporter protein (SERT) gene in patients with pulmonary arterial hypertension.

Author

Baloira A, Núñez M, Cifrian J, Vilariño C, Ojeda M, and Valverde D

Subjects
Adult, Aged, Alleles, Connective Tissue Diseases complications, Connective Tissue Diseases genetics, Female, Gene Frequency, Genotype, HIV Infections complications, HIV Infections genetics, Heart Defects, Congenital complications, Heart Defects, Congenital genetics, Humans, Hypertension, Portal complications, Hypertension, Portal genetics, Hypertension, Pulmonary epidemiology, Hypertension, Pulmonary etiology, Male, Middle Aged, Polymerase Chain Reaction, Promoter Regions, Genetic genetics, Pulmonary Disease, Chronic Obstructive complications, Pulmonary Disease, Chronic Obstructive genetics, Pulmonary Embolism complications, Pulmonary Embolism genetics, Sampling Studies, Serotonin physiology, Spain epidemiology, Hypertension, Pulmonary genetics, Polymorphism, Genetic, Serotonin Plasma Membrane Transport Proteins genetics
Abstract

Unlabelled: Serotonin is a potent vasoconstrictor and pulmonary vascular growth factor whose concentration is increased in patients with pulmonary arterial hypertension (PAH). Its functions are mediated in part by the serotonin transporter protein (SERT) whose gene can have two allelic forms, both long (L) and short (S). The first was associated with greater function., Objectives: To determine whether the prevalence of the L allelic form of SERT is higher in patients with PAH than in the general population. To observe whether there are any clinical differences in patients with PAH based on the SERT allele., Methods: We included patients diagnosed with PAH with catheterization based on the established criteria. Peripheral blood samples were taken and the DNA was extracted from the peripheral leukocytes. We amplified the promoter region of SERT by polymerase chain reaction and separated the products by electrophoresis. The patient samples were compared with samples from 50 healthy controls and among the most common types of PAH (idiopathic, thromboembolic and associated with connective tissue disorders). Several clinical variables were assessed according to the SERT gene alleles., Results: The study included 50 patients, and adequate samples were obtained in 49 (30 women). Mean age at diagnosis was 56 ± 16 years. No differences were seen in the distribution of alleles between patients and controls (P = .54). There were no differences among the three most common types of PAH (P = .3). The most frequent allelic form was LS (54% patients, 56% controls). There were no differences in either age of diagnosis or response to treatment according to the SERT alleles. There was a trend toward higher mean pulmonary pressure levels in the LL forms (49 ± 5 mm Hg vs 42 ± 9 mm Hg, P = .07)., Conclusions: The distribution of SERT gene alleles does not appear to be different in patients with PAH than in the normal population. Different types of PAH have a similar distribution of alleles. The LL forms do not appear to confer either clinical differences or differences in response to treatment., (Copyright © 2011 SEPAR. Published by Elsevier Espana. All rights reserved.)

Published
2012
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31. Guidelines for the selection of lung transplantation candidates.

Author

Román A, Ussetti P, Solé A, Zurbano F, Borro JM, Vaquero JM, de Pablo A, Morales P, Blanco M, Bravo C, Cifrian J, de la Torre M, Gámez P, Laporta R, Monforte V, Mons R, Salvatierra A, Santos F, Solé J, and Varela A

Subjects
Humans, Lung Transplantation, Patient Selection
Abstract

The present guidelines have been prepared with the consensus of at least one representative of each of the hospitals with lung transplantation programs in Spain. In addition, prior to their publication, these guidelines have been reviewed by a group of prominent reviewers who are recognized for their professional experience in the field of lung transplantation. Within the following pages, the reader will find the selection criteria for lung transplantation candidates, when and how to remit a patient to a transplantation center and, lastly, when to add the patient to the waiting list. A level of evidence has been identified for the most relevant questions. Our intention is for this document to be a practical guide for pulmonologists who do not directly participate in lung transplantations but who should consider this treatment for their patients. Finally, these guidelines also propose an information form in order to compile in an organized manner the patient data of the potential candidate for lung transplantation, which are relevant in order to be able to make the best decisions possible., (Copyright © 2011 SEPAR. Published by Elsevier Espana. All rights reserved.)

Published
2011
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