TY - JOUR
T1 - Clinical spectrum time course in non-Asian patients positive for anti-MDA5 antibodies
AU - AENEAS collaborative group
AU - Cavagna, Lorenzo
AU - Meloni, Federica
AU - Meyer, Alain
AU - Sambataro, Gianluca
AU - Belliato, Mirko
AU - de Langhe, Ellen
AU - Cavazzana, Ilaria
AU - Pipitone, Nicolò
AU - Triantafyllias, Konstantinos
AU - Mosca, Marta
AU - Barsotti, Simone
AU - Zampogna, Giuseppe
AU - Biglia, Alessandro
AU - Emmi, Giacomo
AU - de Visser, Marianne
AU - van der Kooi, Anneke
AU - Parronchi, Paola
AU - Hirschi, Sandrine
AU - da Silva, Jose Antonio Pereira
AU - Scirè, Carlo Alberto
AU - Furini, Federica
AU - Giannini, Margherita
AU - Martinez Gonzalez, Olga
AU - Damian, Laura
AU - Piette, Yves
AU - Smith, Vanessa
AU - Mera-Valera, Antonio
AU - Bachiller-Corral, Javier
AU - Cabezas Rodriguez, Ivan
AU - Brandy-Garcia, Anahy M.
AU - Maurier, François
AU - Perrin, Julie
AU - Gonzalez-Moreno, Juan
AU - Drott, Ulrich
AU - Delbruck, Christiane
AU - Schwarting, Andreas
AU - Arrigoni, Eugenio
AU - Sebastiani, Gian Domenico
AU - Iuliano, Annamaria
AU - Nannini, Carlotta
AU - Quartuccio, Luca
AU - Rodriguez Cambron, Ana B.
AU - Blázquez Cañamero, Maria Á
AU - Villa Blanco, Ignacio
AU - Cagnotto, Giovanni
AU - Pesci, Alberto
AU - Luppi, Francesco
AU - Dei, Giulia
AU - Romero Bueno, Fredeswinda Isabel
AU - Franceschini, Franco
N1 - Funding Information: this research was partially funded by FOREUM - Foundation for Research in Rheumatology Publisher Copyright: © Clinical and ExpErimEntal rhEumatology 2022.
PY - 2022/2/1
Y1 - 2022/2/1
N2 - 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.
AB - 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.
KW - idiopathic inflammatory myopathies
KW - melanoma differentiation-associated protein 5 antibody
KW - rapidly progressive interstitial lung diseases
UR - http://www.scopus.com/inward/record.url?scp=85125551103&partnerID=8YFLogxK
U2 - https://doi.org/10.55563/CLINEXPRHEUMATOL/DI1083
DO - https://doi.org/10.55563/CLINEXPRHEUMATOL/DI1083
M3 - Article
C2 - 35200123
SN - 0392-856X
VL - 40
SP - 274
EP - 283
JO - Clinical and experimental rheumatology
JF - Clinical and experimental rheumatology
IS - 2
ER -