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

Autor:	Cavagna L; Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy. lorenzo.cavagna@unipv.it., Meloni F; Transplant Centre Unit, IRCCS San Matteo Foundation and University of Pavia, Italy., Meyer A; Rheumatology, Centre de Référence des Maladies Auto-Immunes Rares, Service de Physiologie et Explorations Fonctionnelles Musculaires, Hôpitaux Universitaires de Strasbourg, France., Sambataro G; Pulmonology, Azienda Ospedaliera Universitaria Catania, Italy., Belliato M; UOC Anestesia e Rianimazione 2 Cardiopolmonare, Foundation IRCCS Policlinico S. Matteo, Pavia, Italy., De Langhe E; Rheumatology, University Hospitals, Leuven, Belgium., Cavazzana I; Rheumatology, University and AO Spedali Civili, Brescia, Italy., Pipitone N; Rheumatology, S. Maria Hospital IRCCS, Reggio Emilia, Italy., Triantafyllias K; Rheumatology, Centre for Rheumatic Diseases, Bad Kreuznach, Germany., Mosca M; Rheumatology, Azienda Ospedaliera Universitaria Pisana, Pisa, Italy., Barsotti S; Rheumatology, Azienda Ospedaliera Universitaria Pisana, Pisa, Italy., Zampogna G; Rheumatology, Azienda Ospedaliera Brunico, Italy., Biglia A; Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy., Emmi G; Internal Interdisciplinary Unit, Lupus Clinic, Careggi University Hospital and Department of Experimental and Clinical Medicine, University of Firenze, Italy., De Visser M; Neurology, Amsterdam University Medical Centres, The Netherlands., Van Der Kooi A; Neurology, Amsterdam University Medical Centres, The Netherlands., Parronchi P; Department of Experimental and Clinical Medicine, University of Florence, Italy., Hirschi S; Pneumology, NHC, Strasbourg University Hospital, Strasbourg University, France., da Silva JAP; Rheumatology, Centro Hospitalar e Universitário de Coimbra, Portugal., Scirè CA; Rheumatology, Azienda Ospedaliero Universitaria S. Anna, Ferrara, Italy., Furini F; Rheumatology, Azienda Ospedaliero Universitaria S. Anna, Ferrara, Italy., Giannini M; Rheumatology, Centre de Référence des Maladies Auto-Immunes Rares, Service de Physiologie et Explorations Fonctionnelles Musculaires, Hôpitaux Universitaires de Strasbourg, France., Martinez Gonzalez O; Rheumatology, Complejo Hospitalario de Salamanca, Spain., Damian L; Rheumatology, Emergency County Teaching Hospital, Cluj-Napoca, Romania., Piette Y; Department of Rheumatology, Ghent University Hospital; Department of Internal Medicine, Ghent University; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium., Smith V; Department of Rheumatology, Ghent University Hospital; Department of Internal Medicine, Ghent University; Unit for Molecular Immunology and Inflammation, VIB Inflammation Research Center (IRC), Ghent, Belgium., Mera-Valera A; Rheumatology, Hospital Clínico Universitario de Santiago de Compostela, Spain., Bachiller-Corral J; Rheumatology, Hospital Universitario Ramon y Cajal, Spain., Cabezas Rodriguez I; Rheumatology, Hospital Universitario Rio Hortega, Valladolid, Spain., Brandy-Garcia AM; Rheumatology, Hospital Universitario San Augustin, Aviles, Spain., Maurier F; Rheumatology, HPMetz, Hopital Belle-Ile, Metz, France., Perrin J; Pneumology HPMetz, Hopital Belle-Ile, Metz, France., Gonzalez-Moreno J; Rheumatology, Internal Medicine Department, Hospital Universitario Son Llàtzer, Palma de Mallorca, Spain., Drott U; Rheumatology, Johann Wolfgang Goethe-Universität, Frankfurt, Germany., Delbruck C; Rheumatology, Johann Wolfgang Goethe-Universität, Frankfurt, Germany., Schwarting A; Rheumatology, Johannes Gutenberg-University, Mainz, Germany., Arrigoni E; Rheumatology, Ospedale Guglielmo da Saliceto, Piacenza, Italy., Sebastiani GD; Rheumatology, Ospedale San Camillo, Roma, Italy., Iuliano A; Rheumatology, Ospedale San Camillo, Roma, Italy., Nannini C; Rheumatology, Prato Hospital, Italy., Quartuccio L; Rheumatology Unit, Department of Medicine (DAME), University of Udine, Academic Hospital 'Santa Maria della Misericordia', Udine, Italy., Rodriguez Cambron AB; Rheumatology, Severo Ochoa Hospital, Madrid, Spain., Blázquez Cañamero MÁ; Rheumatology, Severo Ochoa Hospital, Madrid, Spain., Villa Blanco I; Rheumatology, Sierralana Hospital, Torrellavega, Spain., Cagnotto G; Rheumatology, Skane University Hospital, Sweden., Pesci A; Pneumology, University of Milano Bicocca, San Gerardo Hospital, Monza, Italy., Luppi F; Pneumology, University of Milano Bicocca, San Gerardo Hospital, Monza, Italy., Dei G; Pneumology, University of Milano Bicocca, San Gerardo Hospital, Monza, Italy., Romero Bueno FI; Rheumatology, University Hospital J. Dìaz, Madrid, Spain., Franceschini F; Rheumatology, University and AO Spedali Civili, Brescia, Italy., Chiapparoli I; Rheumatology, S. Maria Hospital IRCCS, Reggio Emilia, Italy., Zanframundo G; Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy., Lettieri S; Pneumology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy., De Stefano L; Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy., Cutolo M; Rheumatology, University of Genova, DIMI, IRCCS San Martino, Genova, Italy., Mathieu A; Rheumatology, University Clinic and Azienda Ospedaliera Universitaria of Cagliari, Italy., Piga M; Rheumatology, University Clinic and Azienda Ospedaliera Universitaria of Cagliari, Italy., Prieto-González S; Internal Medicine, Hospital Clínic, Barcelona, Spain., Moraes-Fontes MF; Rheumatology, University Hospital Lisboa Norte, Portugal., Fonseca JE; Serviço Reumatologia, Centro Hospitalar Lisboa Norte and Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa, Portugal., Jovani V; Rheumatology, University Hospital, Alicante, Spain., Riccieri V; Rheumatology, University La Sapienza and Policlinico Umberto I, Rome, Italy., Santaniello A; Scleroderma Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy., Montfort S; Pneumology, Mater Dei Hospital, Msida, Malta., Bilocca D; Pneumology, Mater Dei Hospital, Msida, Malta., Erre GL; Rheumatology, Azienda Ospedaliero-Universitaria di Sassari, Italy., Bartoloni E; Rheumatology, University of Perugia, Italy., Gerli R; Rheumatology, University of Perugia, Italy., Monti MC; Biostatistics Unit, Department of Public Health, University of Pavia, Italy., Lorenz HM; Rheumatology, University of Heidelberg, Germany., Sambataro D; Artroreuma srl, Outpatient of Rheumatology accredited with the Italian National Health System, Mascalucia, Catania, Italy., Bellando Randone S; Rheumatology, Azienda Ospedaliera Universitaria Careggi, Firenze, Italy., Schneider U; Rheumatology, Charité - Universitätsmedizin Berlin, Germany., Valenzuela C; Pneumology, Hospital Universitario de la Princesa, IIS-Princesa, Cátedra UAM-Roche (EPID Future), Universidad Autonoma, Madrid, Spain., Lopez-Mejias R; Rheumatology, Hospital Universitario Marques de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain., Cifrian J; Pneumology, Hospital Universitario Marques de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain., Mejia M; Pneumology, Interstitial Lung Disease and Rheumatology Units, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, México City, Mexico., Gonzalez Perez MI; Pneumology, Interstitial Lung Disease and Rheumatology Units, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, México City, Mexico., Wendel S; Rheumatology, Medical Center, University of Freiburg, Faculty of Medicine, Freiburg, Germany., Fornaro M; Rheumatology, University of Bari, Italy., De Luca G; Rheumatology, Università Vita-Salute, San Raffaele, Milano, Italy., Orsolini G; Rheumatology, University of Verona, Italy., Rossini M; Rheumatology, University of Verona, Italy., Dieude P; Rheumatology, Hôpital Bichat-Claude Bernard, Université Paris Diderot, Paris, France., Knitza J; Rheumatology, University of Erlangen, Germany., Castañeda S; Rheumatology, Hospital Universitario de la Princesa, IIS-Princesa, Cátedra UAM-Roche (EPID Future), Universidad Autonoma, Madrid, Spain., Voll RE; Rheumatology, Medical Center, University of Freiburg, Faculty of Medicine, Freiburg, Germany., Rojas-Serrano J; Rheumatology, Interstitial Lung Disease and Rheumatology Units, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, México City, México., Valentini A; Radiology, IRCCS Policlinico S. Matteo Foundation, Pavia, Italy., Vancheri C; Pulmonology, Azienda Ospedaliera Universitaria Catania, Italy., Matucci-Cerinic M; Rheumatology, Azienda Ospedaliera Universitaria Careggi, Firenze, Italy., Feist E; Rheumatology, Charité - Universitätsmedizin Berlin, Germany., Codullo V; Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy., Iannone F; Rheumatology, University of Bari, Italy., Distler JH; Rheumatology, University of Erlangen, Germany., Montecucco C; Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy., Gonzalez-Gay MA; Rheumatology, Hospital Universitario Marques de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.
Jazyk:	angličtina
Zdroj:	Clinical and experimental rheumatology [Clin Exp Rheumatol] 2022 Feb; Vol. 40 (2), pp. 274-283. Date of Electronic Publication: 2022 Feb 16.
DOI:	10.55563/clinexprheumatol/di1083
Abstrakt:	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.
Databáze:	MEDLINE
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