An herbal formula attenuates collagen-induced arthritis via inhibition of JAK2-STAT3 signaling and regulation of Th17 cells in mice.
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Cavagna Lorenzo, Meloni Federica, Meyer Alain, Sambataro Gianluca, Belliato Mirko, De Langhe Ellen, Cavazzana Ilaria, Pipitone Nicolò, Triantafyllias Konstantinos, Mosca Marta, Barsotti Simone, Zampogna Giuseppe, Biglia Alessandro, Emmi Giacomo, De Visser Marianne, Van Der Kooi Anneke, Parronchi Paola, Hirschi Sandrine, da Silva Jose Antonio Pereira, Scirè Carlo Alberto, Furini Federica, Giannini Margherita, Martinez Gonzalez Olga, Damian Laura, Piette Yves, Smith Vanessa, Mera-Valera Antonio, Bachiller-Corral Javier, Cabezas Rodriguez Ivan, Brandy-Garcia Anahy M, Maurier François, Perrin Julie, Gonzalez-Moreno Juan, Drott Ulrich, Delbruck Christiane, Schwarting Andreas, Arrigoni Eugenio, Sebastiani Gian Domenico, Iuliano Annamaria, Nannini Carlotta, Quartuccio Luca, Rodriguez Cambron Ana B, Blázquez Cañamero Maria Á, Villa Blanco Ignacio, Cagnotto Giovanni, Pesci Alberto, Luppi Fabrizio, Dei Giulia, Romero Bueno Fredeswinda Isabel, Franceschini Franco, Chiapparoli Ilaria, Zanframundo Giovanni, Lettieri Sara, De Stefano Ludovico, Cutolo Maurizio, Mathieu Alessandro, Piga Matteo, Prieto-González Sergio, Moraes-Fontes Maria Francisca, Fonseca Joao Eurico, Jovani Vega, Riccieri Valeria, Santaniello Alessandro, Montfort Stephen, Bilocca David, Erre Gian Luca, Bartoloni Elena, Gerli Roberto, Monti M Cristina, Lorenz Hanns M, Sambataro Domenico, Bellando Randone Silvia, Schneider Udo, Valenzuela Claudia, Lopez-Mejias Raquel, Cifrian Jose, Mejia Mayra, Gonzalez Perez Monserrat-Ixchel, Wendel Sarah, Fornaro Marco, De Luca Giacomo, Orsolini Giovanni, Rossini Maurizio, Dieude Philippe, Knitza Johannes, Castañeda Santos, Voll Reinhard E, Rojas-Serrano Jorge, Valentini Adele, Vancheri Carlo, Matucci-Cerinic Marco, Feist Eugen, Codullo Veronica, Iannone Florenzo, Distler Jorg H, Montecucco Carlomaurizio, Gonzalez-Gay Miguel A, AENEAS collaborative group
Аннотация
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.