ObjectiveTo define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies.MethodsWe conducted a multicentre, international, retrospective cohort study.Results149 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.ConclusionThe 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.

Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies / Cavagna, Lorenzo; Meloni, Federica; Meyer, Alain; Sambataro, Gianluca; Belliato, Mirko; De Langhe, Ellen; Cavazzana, Ilaria; Pipitone, Nicolò; Triantafyllias, Konstantinos; 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, Yves; Smith, Vanessa; Mera-Valera, Antonio; Bachiller-Corral, Javier; Cabezas Rodriguez, Ivan; Brandy-Garcia, Anahy M; Maurier, François; Perrin, Julie; Gonzalez-Moreno, Juan; Drott, Ulrich; Delbruck, Christiane; Schwarting, Andreas; 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, Johannes; Castañeda, Santos; Voll, Reinhard E; Rojas-Serrano, Jorge; Valentini, Adele; Vancheri, Carlo; Matucci-Cerinic, Marco; Feist, Eugen; Codullo, Veronica; Iannone, Florenzo; Distler, Jorg H; Montecucco, Carlomaurizio; Gonzalez-Gay, Miguel A. - In: CLINICAL AND EXPERIMENTAL RHEUMATOLOGY. - ISSN 0392-856X. - 40:2(2022), pp. 274-283-283. [10.55563/clinexprheumatol/di1083]

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

Meloni, Federica;Erre, Gian Luca;Montecucco, Carlomaurizio;
2022-01-01

Abstract

ObjectiveTo define the clinical spectrum time-course and prognosis of non-Asian patients positive for anti-MDA5 antibodies.MethodsWe conducted a multicentre, international, retrospective cohort study.Results149 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.ConclusionThe 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.
2022
Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies / Cavagna, Lorenzo; Meloni, Federica; Meyer, Alain; Sambataro, Gianluca; Belliato, Mirko; De Langhe, Ellen; Cavazzana, Ilaria; Pipitone, Nicolò; Triantafyllias, Konstantinos; 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, Yves; Smith, Vanessa; Mera-Valera, Antonio; Bachiller-Corral, Javier; Cabezas Rodriguez, Ivan; Brandy-Garcia, Anahy M; Maurier, François; Perrin, Julie; Gonzalez-Moreno, Juan; Drott, Ulrich; Delbruck, Christiane; Schwarting, Andreas; 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, Johannes; Castañeda, Santos; Voll, Reinhard E; Rojas-Serrano, Jorge; Valentini, Adele; Vancheri, Carlo; Matucci-Cerinic, Marco; Feist, Eugen; Codullo, Veronica; Iannone, Florenzo; Distler, Jorg H; Montecucco, Carlomaurizio; Gonzalez-Gay, Miguel A. - In: CLINICAL AND EXPERIMENTAL RHEUMATOLOGY. - ISSN 0392-856X. - 40:2(2022), pp. 274-283-283. [10.55563/clinexprheumatol/di1083]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11388/285485
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