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

Objective 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 m...

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Authors: 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; Scire, 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, ABR; Canamero, MAB; Blanco, IV; Cagnotto, G; Pesci, A; Luppi, F; Dei, G; Bueno, FIR; Franceschini, F; Chiapparoli, I; Zanframundo, G; Lettieri, S; De Stefano, L; Cutolo, M; Mathieu, A; Piga, M; Prieto-Gonzalez, 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, MIG; Wendel, S; Fornaro, M; De Luca, G; Orsolini, G; Rossini, M; Dieude, P; Knitza, J; Castaneda, S; Voll, RE; Rojas-Serrano, J; Valentini, A; Vancheri, C; Matucci-Cerinic, M; Feist, E; Codullo, V; Iannone, F; Distler, JH; Montecucco, C; Gonzalez-Gay, MA
Format: Article
Status:Published version
Publication date:2022
Country:España
Institution:Instituto de Investigación Biomédica y Sanitaria de Alicante (ISABIAL)
Repository:r-ISABIAL. Repositorio Institucional de Producción Científica del Instituto de Investigación Biomédica y Sanitaria de Alicante
OAI Identifier:oai:isabial.fundanetsuite.com:p9656
Online Access:https://isabial.portalinvestigacion.com/publicaciones9656
Access Level:Open access
Keyword:melanoma differentiation-associated protein 5 antibody
rapidly progressive interstitial lung diseases
idiopathic inflammatory myopathies
Summary:Objective 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. Conclusion 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.

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