Timing of onset affects arthritis presentation pattern in antisyntethase syndrome

79To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD).nonenoneGonzález-Gay, Miguel A; Montecucco, Carlomaurizio; Selva-O'Callaghan, Albert; Trallero-Araguas, Ernesto; Molberg, Ovynd; Andersson, Helena; Rojas-Serrano, Jorge; Perez-Roman, Diana Isabel; Bauhammer, Jutta; Fiehn, Christoph; Neri, Rossella; Barsotti, Simone; Lorenz, Hannes M; Doria, Andrea; Ghirardello, Anna; Iannone, Florenzo; Giannini, Margherita; Franceschini, Franco; Cavazzana, Ilaria; Triantafyllias, Konstantinos; Benucci, Maurizio; Infantino, Maria; Manfredi, Mariangela; Conti, Fabrizio; Schwarting, Andreas; Sebastiani, Giandomenico; Iuliano, Annamaria; Emmi, Giacomo; Silvestri, Elena; Govoni, Marcello; Scirè, Carlo Alberto; Furini, Federica; Lopez-Longo, Francisco Javier; Martínez-Barrio, Julia; Sebastiani, Marco; Manfredi, Andreina; Bachiller-Corral, Javier; Sifuentes Giraldo, Walter Alberto; Cimmino, Marco A; Cosso, Claudio; Belotti Masserini, Alessandro; Cagnotto, Giovanni; Codullo, Veronica; Romano, Mariaeva; Paolazzi, Giuseppe; Pellerito, Raffaele; Saketkoo, Lesley Ann; Ortego-Centeno, Norberto; Quartuccio, Luca; Batticciotto, Alberto; Bartoloni Bocci, Elena; Gerli, Roberto; Specker, Christof; Bravi, Elena; Selmi, Carlo; Parisi, Simone; Salaffi, Fausto; Meloni, Federica; Marchioni, Enrico; Pesci, Alberto; Dei, Giulia; Confalonieri, Marco; Tomietto, Paola; Nuno, Laura; Bonella, Francesco; Pipitone, Nicolò; Mera-Valera, Antonio; Perez-Gomez, Nair; Gerzeli, Simone; Lopez-Mejias, Raquel; Matos-Costa, Carlo Jorge; Pereira da Silva, Jose Antonio; Cifrian, José; Alpini, Claudia; Olivieri, Ignazio; Blázquez Cañamero, María Ángeles; Rodriguez Cambrón, Ana Belén; Castañeda, Santos; Cavagna, LorenzoGonzález-Gay, Miguel A; Montecucco, Carlomaurizio; Selva-O'Callaghan, Albert; Trallero-Araguas, Ernesto; Molberg, Ovynd; Andersson, Helena; Rojas-Serrano, Jorge; Perez-Roman, Diana Isabel; Bauhammer, Jutta; Fiehn, Christoph; Neri, Rossella; Barsotti, Simone; Lorenz, Hannes M; Doria, Andrea; Ghirardello, Anna; Iannone, Florenzo; Giannini, Margherita; Franceschini, Franco; Cavazzana, Ilaria; Triantafyllias, Konstantinos; Benucci, Maurizio; Infantino, Maria; Manfredi, Mariangela; Conti, Fabrizio; Schwarting, Andreas; Sebastiani, Giandomenico; Iuliano, Annamaria; Emmi, Giacomo; Silvestri, Elena; Govoni, Marcello; Scirè, Carlo Alberto; Furini, Federica; Lopez-Longo, Francisco Javier; Martínez-Barrio, Julia; Sebastiani, Marco; Manfredi, Andreina; Bachiller-Corral, Javier; Sifuentes Giraldo, Walter Alberto; Cimmino, Marco A; Cosso, Claudio; Belotti Masserini, Alessandro; Cagnotto, Giovanni; Codullo, Veronica; Romano, Mariaeva; Paolazzi, Giuseppe; Pellerito, Raffaele; Saketkoo, Lesley Ann; Ortego-Centeno, Norberto; Quartuccio, Luca; Batticciotto, Alberto; Bartoloni Bocci, Elena; Gerli, Roberto; Specker, Christof; Bravi, Elena; Selmi, Carlo; Parisi, Simone; Salaffi, Fausto; Meloni, Federica; Marchioni, Enrico; Pesci, Alberto; Dei, Giulia; Confalonieri, Marco; Tomietto, Paola; Nuno, Laura; Bonella, Francesco; Pipitone, Nicolò; Mera-Valera, Antonio; Perez-Gomez, Nair; Gerzeli, Simone; Lopez-Mejias, Raquel; Matos-Costa, Carlo Jorge; Pereira da Silva, Jose Antonio; Cifrian, José; Alpini, Claudia; Olivieri, Ignazio; Blázquez Cañamero, María Ángeles; Rodriguez Cambrón, Ana Belén; Castañeda, Santos; Cavagna, Lorenz

Timing of onset affects arthritis presentation pattern in antisynthetase syndrome

Objective To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD). Methods The patients were selected from a retrospective large international cohort of ASSD patients regularly followed-up in centres referring to AENEAS collaborative group. Patients were eligible if they had an antisynthetase antibody testing positive in at least two determinations along with arthritis occurring either at ASSD onset (Group 1) or during the course of the disease (Group 2). Results 445 (70%; 334 females, 110 males, 1 transsexual) out of the 636 ASSD we collected had arthritis, in the majority of cases (367, 83%) from disease onset (Group 1). Patients belonging to Group 1 with respect to Group 2 had an arthritis more commonly polyarticular and symmetrical (p=0.015), IgM-Rheumatoid factor positive (p=0.035), erosions at hands and feet plain x-rays (p=0.036) and more commonly satisfying the 1987 revised classification criteria for rheumatoid arthritis (RA) (p=0.004). Features such as Raynaud's phenomenon, mechanic's hands and fever (e.g. accompanying findings) were more frequently reported in Group 2 (p=0.005). Conclusion In ASSD, the timing of appearance with respect to disease onset influences arthritis characteristics. In particular, RA features are more common when arthritis occurs from ASSD onset, suggesting an overlap between RA and ASSD in these patients. When arthritis appears during the follow-up, it is very close to a connective tissue disease-related arthritis. Also, the different prevalence of accompanying features between these two groups is in line with this possibility

Timing of onset affects arthritis presentation pattern in antisyntethase syndrome

OBJECTIVES: To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD). METHODS: The patients were selected from a retrospective large international cohort of ASSD patients regularly followed-up in centres referring to AENEAS collaborative group. Patients were eligible if they had an antisynthetase antibody testing positive in at least two determinations along with arthritis occurring either at ASSD onset (Group 1) or during the course of the disease (Group 2). RESULTS: 445 (70%; 334 females, 110 males, 1 transsexual) out of the 636 ASSD we collected had arthritis, in the majority of cases (367, 83%) from disease onset (Group 1). Patients belonging to Group 1 with respect to Group 2 had an arthritis more commonly polyarticular and symmetrical (p=0.015), IgM-Rheumatoid factor positive (p=0.035), erosions at hands and feet plain x-rays (p=0.036) and more commonly satisfying the 1987 revised classification criteria for rheumatoid arthritis (RA) (p=0.004). Features such as Raynaud's phenomenon, mechanic's hands and fever (e.g. accompanying findings) were more frequently reported in Group 2 (p=0.005). CONCLUSIONS: In ASSD, the timing of appearance with respect to disease onset influences arthritis characteristics. In particular, RA features are more common when arthritis occurs from ASSD onset, suggesting an overlap between RA and ASSD in these patients. When arthritis appears during the follow-up, it is very close to a connective tissue disease-related arthritis. Also, the different prevalence of accompanying features between these two groups is in line with this possibility

Timing of onset affects arthritis presentation pattern in antisyntethase syndrome

445 (70%; 334 females, 110 males, 1 transsexual) out of the 636 ASSD we collected had arthritis, in the majority of cases (367, 83%) from disease onset (Group 1). Patients belonging to Group 1 with respect to Group 2 had an arthritis more commonly polyarticular and symmetrical (p=0.015), IgM-Rheumatoid factor positive (p=0.035), erosions at hands and feet plain x-rays (p=0.036) and more commonly satisfying the 1987 revised classification criteria for rheumatoid arthritis (RA) (p=0.004). Features such as Raynaud's phenomenon, mechanic's hands and fever (e.g. accompanying findings) were more frequently reported in Group 2 (p=0.005). In ASSD, the timing of appearance with respect to disease onset influences arthritis characteristics. In particular, RA features are more common when arthritis occurs from ASSD onset, suggesting an overlap between RA and ASSD in these patients. When arthritis appears during the follow-up, it is very close to a connective tissue disease-related arthritis. Also, the different prevalence of accompanying features between these two groups is in line with this possibility

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

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

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

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

Timing of onset affects arthritis presentation pattern in antisynthetase syndrome

Abstract OBJECTIVES: To evaluate if the timing of appearance with respect to disease onset may influence the arthritis presentation pattern in antisynthetase syndrome (ASSD). METHODS: The patients were selected from a retrospective large international cohort of ASSD patients regularly followed-up in centres referring to AENEAS collaborative group. Patients were eligible if they had an antisynthetase antibody testing positive in at least two determinations along with arthritis occurring either at ASSD onset (Group 1) or during the course of the disease (Group 2). RESULTS: 445 (70%; 334 females, 110 males, 1 transsexual) out of the 636 ASSD we collected had arthritis, in the majority of cases (367, 83%) from disease onset (Group 1). Patients belonging to Group 1 with respect to Group 2 had an arthritis more commonly polyarticular and symmetrical (p=0.015), IgM-Rheumatoid factor positive (p=0.035), erosions at hands and feet plain x-rays (p=0.036) and more commonly satisfying the 1987 revised classification criteria for rheumatoid arthritis (RA) (p=0.004). Features such as Raynaud's phenomenon, mechanic's hands and fever (e.g. accompanying findings) were more frequently reported in Group 2 (p=0.005). CONCLUSIONS: In ASSD, the timing of appearance with respect to disease onset influences arthritis characteristics. In particular, RA features are more common when arthritis occurs from ASSD onset, suggesting an overlap between RA and ASSD in these patients. When arthritis appears during the follow-up, it is very close to a connective tissue disease-related arthritis. Also, the different prevalence of accompanying features between these two groups is in line with this possibility