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 JA, Scirè CA, Furini F, Giannini M, Gonzalez OM, Damian L, Piette Y, Smith V, Mera-Varela A, Bachiller-Corral J, Rodriguez IC, 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, Cambron AB, Cañamero MÁ, Blanco IV, Cagnotto G, Pesci A, Luppi F, Dei G, 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, Montefort S, Bilocca D, Erre GL, Bartoloni E, Gerli R, Monti MC, Lorenz HM, Sambataro D, Randone SB, Schneider U, Valenzuela C, Lopez-Mejias R, Cifrian J, Mejia M, 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

DOI: 10.55563/clinexprheumatol/di1083

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

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

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. https://doi.org/10.55563/clinexprheumatol/di1083

MLA:

Cavagna, L., 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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