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Start Over Author "Mera-Valera, A" Topic dermatomyositis
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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 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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2. 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

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