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

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 M, 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 J, Montecucco C, Gonzalez-Gay MA (2022)

Publication Type: Journal article

Publication year: 2022

Journal

Book Volume: 40

Pages Range: 274-283

Journal Issue: 2

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.

Authors with CRIS profile

Involved external institutions

Università degli Studi di Firenze / University of Florence Italy (IT) Hôpital Belle-Isle France (FR) University of Milano-Bicocca / Università degli Studi di Milano-Bicocca, UNIMIB Italy (IT) Hospital Universitario Rio Hortega Spain (ES) Policlinico San Matteo Pavia Fondazione IRCCS Italy (IT) Instituto Nacional de Enfermedades Respiratorias Mexico (MX) Universitätsklinikum Freiburg Germany (DE) University of Verona / Università degli Studi di Verona Italy (IT) Charité - Universitätsmedizin Berlin Germany (DE) Marqués de Valdecilla University Hospital / Hospital Universitario de Marqués de Valdecilla (HUMV) Spain (ES) Hospital Universitario de La Princesa Spain (ES) University of Bari Aldo Moro / Università degli Studi di Bari Aldo Moro Italy (IT) Mater Dei Hospital Malta (MT) Hospital General Universitario de Alicante Spain (ES) Ruprecht-Karls-Universität Heidelberg Germany (DE) Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia Italy (IT) University Hospital Leuven (UZ) / Universitaire ziekenhuizen Leuven Belgium (BE) Università degli Studi di Pavia Italy (IT) University of Udine / Università degli Studi di Udine Italy (IT) Hospital Universitario Severo Ochoa Spain (ES) Azienda Ospedaliera Universitaria di Ferrara Arcispedale Sant'Anna Italy (IT) Università degli studi "La Sapienza" Italy (IT) Azienda Ospedaliero Universitaria di Sassari Italy (IT) Università degli Studi di Catania Italy (IT) Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico Italy (IT) Azienda ospedaliera San Camillo-Forlanini Italy (IT) Hospital Universitario Ramón y Cajal Spain (ES) Hospital Universitario San Agustin Spain (ES) Goethe-Universität Frankfurt am Main Germany (DE) Hospital Clínic de Barcelona Spain (ES) Cluj County Emergency Clinical Hospital Romania (RO) Complejo Hospitalario de Salamanca / Complejo Asistencial Universitario de Salamanca Spain (ES) University of Lisbon / Universidade de Lisboa (ULisboa) Portugal (PT) Azienda Ospedaliero-Universitaria Pisana Italy (IT) University Hospital Ghent Belgium (BE) Università degli Studi di Perugia Italy (IT) Rheumazentrum Rheinland-Pfalz Germany (DE) Azienda Ospedaliera Universitaria di Cagliari Italy (IT) Sierrallana Hospital Spain (ES) University of Amsterdam Netherlands (NL) Skåne University Hospital / Skånes universitetssjukhus Sweden (SE) Hôpitaux universitaires de Strasbourg (HUS) / University Hospital Strasbourg France (FR) Complejo Hospitalario Universitario de Santiago de Compostela Spain (ES) Centro Hospitalar Universitário Lisboa Norte (CHULN) Portugal (PT) Centro Hospitalar e Universitário de Coimbra (CHUC) Portugal (PT) Ospedale Policlinico San Martino Italy (IT) Ospedale Guglielmo da Saliceto Italy (IT) Careggi University Hospital / Azienda Ospedaliero Universitaria Careggi Italy (IT) Hospital Universitario Fundación Jiménez Díaz Spain (ES) Università Vita-Salute San Raffaele (UniSR) Italy (IT) Arcispedale Santa Maria Nuova Italy (IT) Hôpital Bichat-Claude-Bernard (CHU, APHP) France (FR) Johannes Gutenberg-Universität Mainz (JGU) Germany (DE) Hospital Universitari Són Llàtzer Spain (ES)

How to cite

APA:

Cavagna, L., Meloni, F., Meyer, A., Sambataro, G., Belliato, M., De Langhe, E.,... Gonzalez-Gay, M.A. (2022). Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies. Clinical and Experimental Rheumatology, 40(2), 274-283.

MLA:

Cavagna, Lorenzo, et al. "Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies." Clinical and Experimental Rheumatology 40.2 (2022): 274-283.

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