PMID- 35200123
OWN - NLM
STAT- MEDLINE
DCOM- 20220301
LR  - 20240111
IS  - 0392-856X (Print)
IS  - 0392-856X (Linking)
VI  - 40
IP  - 2
DP  - 2022 Feb
TI  - Clinical spectrum time course in non-Asian patients positive for anti-MDA5 
      antibodies.
PG  - 274-283
LID - 10.55563/clinexprheumatol/di1083 [doi]
AB  - 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.
FAU - Cavagna, Lorenzo
AU  - Cavagna L
AD  - Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, 
      Italy. lorenzo.cavagna@unipv.it.
FAU - Meloni, Federica
AU  - Meloni F
AD  - Transplant Centre Unit, IRCCS San Matteo Foundation and University of Pavia, 
      Italy.
FAU - Meyer, Alain
AU  - Meyer A
AD  - Rheumatology, Centre de Reference des Maladies Auto-Immunes Rares, Service de 
      Physiologie et Explorations Fonctionnelles Musculaires, Hopitaux Universitaires 
      de Strasbourg, France.
FAU - Sambataro, Gianluca
AU  - Sambataro G
AD  - Pulmonology, Azienda Ospedaliera Universitaria Catania, Italy.
FAU - Belliato, Mirko
AU  - Belliato M
AD  - UOC Anestesia e Rianimazione 2 Cardiopolmonare, Foundation IRCCS Policlinico S. 
      Matteo, Pavia, Italy.
FAU - De Langhe, Ellen
AU  - De Langhe E
AD  - Rheumatology, University Hospitals, Leuven, Belgium.
FAU - Cavazzana, Ilaria
AU  - Cavazzana I
AD  - Rheumatology, University and AO Spedali Civili, Brescia, Italy.
FAU - Pipitone, Nicolo
AU  - Pipitone N
AD  - Rheumatology, S. Maria Hospital IRCCS, Reggio Emilia, Italy.
FAU - Triantafyllias, Konstantinos
AU  - Triantafyllias K
AD  - Rheumatology, Centre for Rheumatic Diseases, Bad Kreuznach, Germany.
FAU - Mosca, Marta
AU  - Mosca M
AD  - Rheumatology, Azienda Ospedaliera Universitaria Pisana, Pisa, Italy.
FAU - Barsotti, Simone
AU  - Barsotti S
AD  - Rheumatology, Azienda Ospedaliera Universitaria Pisana, Pisa, Italy.
FAU - Zampogna, Giuseppe
AU  - Zampogna G
AD  - Rheumatology, Azienda Ospedaliera Brunico, Italy.
FAU - Biglia, Alessandro
AU  - Biglia A
AD  - Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, 
      Italy.
FAU - Emmi, Giacomo
AU  - Emmi G
AD  - Internal Interdisciplinary Unit, Lupus Clinic, Careggi University Hospital and 
      Department of Experimental and Clinical Medicine, University of Firenze, Italy.
FAU - De Visser, Marianne
AU  - De Visser M
AD  - Neurology, Amsterdam University Medical Centres, The Netherlands.
FAU - Van Der Kooi, Anneke
AU  - Van Der Kooi A
AD  - Neurology, Amsterdam University Medical Centres, The Netherlands.
FAU - Parronchi, Paola
AU  - Parronchi P
AD  - Department of Experimental and Clinical Medicine, University of Florence, Italy.
FAU - Hirschi, Sandrine
AU  - Hirschi S
AD  - Pneumology, NHC, Strasbourg University Hospital, Strasbourg University, France.
FAU - da Silva, Jose Antonio Pereira
AU  - da Silva JAP
AD  - Rheumatology, Centro Hospitalar e Universitario de Coimbra, Portugal.
FAU - Scire, Carlo Alberto
AU  - Scire CA
AD  - Rheumatology, Azienda Ospedaliero Universitaria S. Anna, Ferrara, Italy.
FAU - Furini, Federica
AU  - Furini F
AD  - Rheumatology, Azienda Ospedaliero Universitaria S. Anna, Ferrara, Italy.
FAU - Giannini, Margherita
AU  - Giannini M
AD  - Rheumatology, Centre de Reference des Maladies Auto-Immunes Rares, Service de 
      Physiologie et Explorations Fonctionnelles Musculaires, Hopitaux Universitaires 
      de Strasbourg, France.
FAU - Martinez Gonzalez, Olga
AU  - Martinez Gonzalez O
AD  - Rheumatology, Complejo Hospitalario de Salamanca, Spain.
FAU - Damian, Laura
AU  - Damian L
AD  - Rheumatology, Emergency County Teaching Hospital, Cluj-Napoca, Romania.
FAU - Piette, Yves
AU  - Piette Y
AD  - 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.
FAU - Smith, Vanessa
AU  - Smith V
AD  - 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.
FAU - Mera-Valera, Antonio
AU  - Mera-Valera A
AD  - Rheumatology, Hospital Clinico Universitario de Santiago de Compostela, Spain.
FAU - Bachiller-Corral, Javier
AU  - Bachiller-Corral J
AD  - Rheumatology, Hospital Universitario Ramon y Cajal, Spain.
FAU - Cabezas Rodriguez, Ivan
AU  - Cabezas Rodriguez I
AD  - Rheumatology, Hospital Universitario Rio Hortega, Valladolid, Spain.
FAU - Brandy-Garcia, Anahy M
AU  - Brandy-Garcia AM
AD  - Rheumatology, Hospital Universitario San Augustin, Aviles, Spain.
FAU - Maurier, Francois
AU  - Maurier F
AD  - Rheumatology, HPMetz, Hopital Belle-Ile, Metz, France.
FAU - Perrin, Julie
AU  - Perrin J
AD  - Pneumology HPMetz, Hopital Belle-Ile, Metz, France.
FAU - Gonzalez-Moreno, Juan
AU  - Gonzalez-Moreno J
AD  - Rheumatology, Internal Medicine Department, Hospital Universitario Son Llatzer, 
      Palma de Mallorca, Spain.
FAU - Drott, Ulrich
AU  - Drott U
AD  - Rheumatology, Johann Wolfgang Goethe-Universitat, Frankfurt, Germany.
FAU - Delbruck, Christiane
AU  - Delbruck C
AD  - Rheumatology, Johann Wolfgang Goethe-Universitat, Frankfurt, Germany.
FAU - Schwarting, Andreas
AU  - Schwarting A
AD  - Rheumatology, Johannes Gutenberg-University, Mainz, Germany.
FAU - Arrigoni, Eugenio
AU  - Arrigoni E
AD  - Rheumatology, Ospedale Guglielmo da Saliceto, Piacenza, Italy.
FAU - Sebastiani, Gian Domenico
AU  - Sebastiani GD
AD  - Rheumatology, Ospedale San Camillo, Roma, Italy.
FAU - Iuliano, Annamaria
AU  - Iuliano A
AD  - Rheumatology, Ospedale San Camillo, Roma, Italy.
FAU - Nannini, Carlotta
AU  - Nannini C
AD  - Rheumatology, Prato Hospital, Italy.
FAU - Quartuccio, Luca
AU  - Quartuccio L
AD  - Rheumatology Unit, Department of Medicine (DAME), University of Udine, Academic 
      Hospital "Santa Maria della Misericordia", Udine, Italy.
FAU - Rodriguez Cambron, Ana B
AU  - Rodriguez Cambron AB
AD  - Rheumatology, Severo Ochoa Hospital, Madrid, Spain.
FAU - Blazquez Canamero, Maria A
AU  - Blazquez Canamero MA
AD  - Rheumatology, Severo Ochoa Hospital, Madrid, Spain.
FAU - Villa Blanco, Ignacio
AU  - Villa Blanco I
AD  - Rheumatology, Sierralana Hospital, Torrellavega, Spain.
FAU - Cagnotto, Giovanni
AU  - Cagnotto G
AD  - Rheumatology, Skane University Hospital, Sweden.
FAU - Pesci, Alberto
AU  - Pesci A
AD  - Pneumology, University of Milano Bicocca, San Gerardo Hospital, Monza, Italy.
FAU - Luppi, Fabrizio
AU  - Luppi F
AD  - Pneumology, University of Milano Bicocca, San Gerardo Hospital, Monza, Italy.
FAU - Dei, Giulia
AU  - Dei G
AD  - Pneumology, University of Milano Bicocca, San Gerardo Hospital, Monza, Italy.
FAU - Romero Bueno, Fredeswinda Isabel
AU  - Romero Bueno FI
AD  - Rheumatology, University Hospital J. Diaz, Madrid, Spain.
FAU - Franceschini, Franco
AU  - Franceschini F
AD  - Rheumatology, University and AO Spedali Civili, Brescia, Italy.
FAU - Chiapparoli, Ilaria
AU  - Chiapparoli I
AD  - Rheumatology, S. Maria Hospital IRCCS, Reggio Emilia, Italy.
FAU - Zanframundo, Giovanni
AU  - Zanframundo G
AD  - Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, 
      Italy.
FAU - Lettieri, Sara
AU  - Lettieri S
AD  - Pneumology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, Italy.
FAU - De Stefano, Ludovico
AU  - De Stefano L
AD  - Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, 
      Italy.
FAU - Cutolo, Maurizio
AU  - Cutolo M
AD  - Rheumatology, University of Genova, DIMI, IRCCS San Martino, Genova, Italy.
FAU - Mathieu, Alessandro
AU  - Mathieu A
AD  - Rheumatology, University Clinic and Azienda Ospedaliera Universitaria of 
      Cagliari, Italy.
FAU - Piga, Matteo
AU  - Piga M
AD  - Rheumatology, University Clinic and Azienda Ospedaliera Universitaria of 
      Cagliari, Italy.
FAU - Prieto-Gonzalez, Sergio
AU  - Prieto-Gonzalez S
AD  - Internal Medicine, Hospital Clinic, Barcelona, Spain.
FAU - Moraes-Fontes, Maria Francisca
AU  - Moraes-Fontes MF
AD  - Rheumatology, University Hospital Lisboa Norte, Portugal.
FAU - Fonseca, Joao Eurico
AU  - Fonseca JE
AD  - Servico Reumatologia, Centro Hospitalar Lisboa Norte and Instituto de Medicina 
      Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Academico de 
      Medicina de Lisboa, Portugal.
FAU - Jovani, Vega
AU  - Jovani V
AD  - Rheumatology, University Hospital, Alicante, Spain.
FAU - Riccieri, Valeria
AU  - Riccieri V
AD  - Rheumatology, University La Sapienza and Policlinico Umberto I, Rome, Italy.
FAU - Santaniello, Alessandro
AU  - Santaniello A
AD  - Scleroderma Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, 
      Milano, Italy.
FAU - Montfort, Stephen
AU  - Montfort S
AD  - Pneumology, Mater Dei Hospital, Msida, Malta.
FAU - Bilocca, David
AU  - Bilocca D
AD  - Pneumology, Mater Dei Hospital, Msida, Malta.
FAU - Erre, Gian Luca
AU  - Erre GL
AD  - Rheumatology, Azienda Ospedaliero-Universitaria di Sassari, Italy.
FAU - Bartoloni, Elena
AU  - Bartoloni E
AD  - Rheumatology, University of Perugia, Italy.
FAU - Gerli, Roberto
AU  - Gerli R
AD  - Rheumatology, University of Perugia, Italy.
FAU - Monti, M Cristina
AU  - Monti MC
AD  - Biostatistics Unit, Department of Public Health, University of Pavia, Italy.
FAU - Lorenz, Hanns M
AU  - Lorenz HM
AD  - Rheumatology, University of Heidelberg, Germany.
FAU - Sambataro, Domenico
AU  - Sambataro D
AD  - Artroreuma srl, Outpatient of Rheumatology accredited with the Italian National 
      Health System, Mascalucia, Catania, Italy.
FAU - Bellando Randone, Silvia
AU  - Bellando Randone S
AD  - Rheumatology, Azienda Ospedaliera Universitaria Careggi, Firenze, Italy.
FAU - Schneider, Udo
AU  - Schneider U
AD  - Rheumatology, Charite - Universitatsmedizin Berlin, Germany.
FAU - Valenzuela, Claudia
AU  - Valenzuela C
AD  - Pneumology, Hospital Universitario de la Princesa, IIS-Princesa, Catedra 
      UAM-Roche (EPID Future), Universidad Autonoma, Madrid, Spain.
FAU - Lopez-Mejias, Raquel
AU  - Lopez-Mejias R
AD  - Rheumatology, Hospital Universitario Marques de Valdecilla, IDIVAL, University of 
      Cantabria, Santander, Spain.
FAU - Cifrian, Jose
AU  - Cifrian J
AD  - Pneumology, Hospital Universitario Marques de Valdecilla, IDIVAL, University of 
      Cantabria, Santander, Spain.
FAU - Mejia, Mayra
AU  - Mejia M
AD  - Pneumology, Interstitial Lung Disease and Rheumatology Units, Instituto Nacional 
      de Enfermedades Respiratorias, Ismael Cosio Villegas, Mexico City, Mexico.
FAU - Gonzalez Perez, Monserrat-Ixchel
AU  - Gonzalez Perez MI
AD  - Pneumology, Interstitial Lung Disease and Rheumatology Units, Instituto Nacional 
      de Enfermedades Respiratorias, Ismael Cosio Villegas, Mexico City, Mexico.
FAU - Wendel, Sarah
AU  - Wendel S
AD  - Rheumatology, Medical Center, University of Freiburg, Faculty of Medicine, 
      Freiburg, Germany.
FAU - Fornaro, Marco
AU  - Fornaro M
AD  - Rheumatology, University of Bari, Italy.
FAU - De Luca, Giacomo
AU  - De Luca G
AD  - Rheumatology, Universita Vita-Salute, San Raffaele, Milano, Italy.
FAU - Orsolini, Giovanni
AU  - Orsolini G
AD  - Rheumatology, University of Verona, Italy.
FAU - Rossini, Maurizio
AU  - Rossini M
AD  - Rheumatology, University of Verona, Italy.
FAU - Dieude, Philippe
AU  - Dieude P
AD  - Rheumatology, Hopital Bichat-Claude Bernard, Universite Paris Diderot, Paris, 
      France.
FAU - Knitza, Johannes
AU  - Knitza J
AD  - Rheumatology, University of Erlangen, Germany.
FAU - Castaneda, Santos
AU  - Castaneda S
AD  - Rheumatology, Hospital Universitario de la Princesa, IIS-Princesa, Catedra 
      UAM-Roche (EPID Future), Universidad Autonoma, Madrid, Spain.
FAU - Voll, Reinhard E
AU  - Voll RE
AD  - Rheumatology, Medical Center, University of Freiburg, Faculty of Medicine, 
      Freiburg, Germany.
FAU - Rojas-Serrano, Jorge
AU  - Rojas-Serrano J
AD  - Rheumatology, Interstitial Lung Disease and Rheumatology Units, Instituto 
      Nacional de Enfermedades Respiratorias, Ismael Cosio Villegas, Mexico City, 
      Mexico.
FAU - Valentini, Adele
AU  - Valentini A
AD  - Radiology, IRCCS Policlinico S. Matteo Foundation, Pavia, Italy.
FAU - Vancheri, Carlo
AU  - Vancheri C
AD  - Pulmonology, Azienda Ospedaliera Universitaria Catania, Italy.
FAU - Matucci-Cerinic, Marco
AU  - Matucci-Cerinic M
AD  - Rheumatology, Azienda Ospedaliera Universitaria Careggi, Firenze, Italy.
FAU - Feist, Eugen
AU  - Feist E
AD  - Rheumatology, Charite - Universitatsmedizin Berlin, Germany.
FAU - Codullo, Veronica
AU  - Codullo V
AD  - Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, 
      Italy.
FAU - Iannone, Florenzo
AU  - Iannone F
AD  - Rheumatology, University of Bari, Italy.
FAU - Distler, Jorg H
AU  - Distler JH
AD  - Rheumatology, University of Erlangen, Germany.
FAU - Montecucco, Carlomaurizio
AU  - Montecucco C
AD  - Rheumatology, University and IRCCS Policlinico S. Matteo Foundation, Pavia, 
      Italy.
FAU - Gonzalez-Gay, Miguel A
AU  - Gonzalez-Gay MA
AD  - Rheumatology, Hospital Universitario Marques de Valdecilla, IDIVAL, University of 
      Cantabria, Santander, Spain.
CN  - AENEAS collaborative group
LA  - eng
PT  - Journal Article
PT  - Multicenter Study
DEP - 20220216
PL  - Italy
TA  - Clin Exp Rheumatol
JT  - Clinical and experimental rheumatology
JID - 8308521
RN  - 0 (Autoantibodies)
RN  - EC 3.6.4.13 (Interferon-Induced Helicase, IFIH1)
SB  - IM
MH  - Autoantibodies
MH  - *Dermatomyositis/complications
MH  - Female
MH  - Humans
MH  - Interferon-Induced Helicase, IFIH1
MH  - *Lung Diseases, Interstitial/drug therapy
MH  - Middle Aged
MH  - Prognosis
MH  - Retrospective Studies
EDAT- 2022/02/25 06:00
MHDA- 2022/03/03 06:00
CRDT- 2022/02/24 12:19
PHST- 2021/08/21 00:00 [received]
PHST- 2021/10/13 00:00 [accepted]
PHST- 2022/02/25 06:00 [pubmed]
PHST- 2022/03/03 06:00 [medline]
PHST- 2022/02/24 12:19 [entrez]
AID - 17797 [pii]
AID - 10.55563/clinexprheumatol/di1083 [doi]
PST - ppublish
SO  - Clin Exp Rheumatol. 2022 Feb;40(2):274-283. doi: 
      10.55563/clinexprheumatol/di1083. Epub 2022 Feb 16.