TY - JOUR
T1 - Clinical Characteristics of Anti-Synthetase Syndrome
T2 - Analysis From the Classification Criteria for Anti-Synthetase Syndrome Project
AU - CLASS project participating investigators
AU - Faghihi-Kashani, Sara
AU - Yoshida, Akira
AU - Bozan, Francisca
AU - Zanframundo, Giovanni
AU - Rozza, Davide
AU - Loganathan, Aravinthan
AU - Dourado, Eduardo
AU - Sambataro, Gianluca
AU - Ventura, Iazsmin Bauer
AU - Bae, Sangmee Sharon
AU - Lim, Darosa
AU - Gallegos, Daphne Rivero
AU - Yamano, Yasuhiko
AU - Selva-O'Callaghan, Albert
AU - Mammen, Andrew L
AU - Scirè, Carlo A
AU - Montecucco, Carlomaurizio
AU - Oddis, Chester V
AU - Fiorentino, David
AU - Bonella, Francesco
AU - Miller, Frederick W
AU - Lundberg, Ingrid E
AU - Schmidt, Jens
AU - Rojas-Serrano, Jorge
AU - Hudson, Marie
AU - Kuwana, Masataka
AU - González-Gay, Miguel Angel
AU - McHugh, Neil
AU - Corte, Tamera J
AU - Doyle, Tracy Jennifer
AU - Werth, Victoria P
AU - Gupta, Latika
AU - Roman, Diana Isabel Perez
AU - Bianchessi, Lorenzo M
AU - Devarasetti, Phani Kumar
AU - Shinjo, Samuel Katsuyuki
AU - Luppi, Fabrizio
AU - Cavazzana, Ilaria
AU - Moghadam-Kia, Siamak
AU - Fornaro, Marco
AU - Volkmann, Elizabeth R
AU - Piga, Matteo
AU - Loarce-Martos, Jesus
AU - De Luca, Giacomo
AU - Knitza, Johannes
AU - Wolff-Cecchi, Veronica
AU - Sebastiani, Marco
AU - Schiffenbauer, Adam
AU - Rider, Lisa G
AU - Gunawardena, Harsha
PY - 2024/10/28
Y1 - 2024/10/28
N2 - Objective: Anti-synthetase syndrome (ASSD) is a rare systemic autoimmune rheumatic disease (SARD) with significant heterogeneity and no shared classification criteria. We aimed to identify clinical and serological features associated with ASSD that may be suitable for inclusion in the data-driven classification criteria for ASSD. Methods: We used a large, international, multicenter “Classification Criteria for Anti-synthetase Syndrome” (CLASS) project database, which includes both patients with ASSD and controls with mimicking conditions, namely, SARDs and/or interstitial lung disease (ILD). The local diagnoses of ASSD and controls were confirmed by project team members. We employed univariable logistic regression and multivariable Ridge regression to evaluate clinical and serological features associated with an ASSD diagnosis in a randomly selected subset of the cohort. Results: Our analysis included 948 patients with ASSD and 1,077 controls. Joint, muscle, lung, skin, and cardiac involvement were more prevalent in patients with ASSD than in controls. Specific variables associated with ASSD included arthritis, diffuse myalgia, muscle weakness, muscle enzyme elevation, ILD, mechanic's hands, secondary pulmonary hypertension due to ILD, Raynaud phenomenon, and unexplained fever. In terms of serological variables, Jo-1 and non–Jo-1 anti-synthetase autoantibodies, antinuclear antibodies with cytoplasmic pattern, and anti-Ro52 autoantibodies were associated with ASSD. In contrast, isolated arthralgia, dysphagia, electromyography/magnetic resonance imaging/muscle biopsy findings suggestive of myopathy, inflammatory rashes, myocarditis, and pulmonary arterial hypertension did not differentiate between patients with ASSD and controls or were inversely associated with ASSD. Conclusion: We identified key clinical and serological variables associated with ASSD, which will help clinicians and offer insights into the development of data-driven classification criteria for ASSD.
AB - Objective: Anti-synthetase syndrome (ASSD) is a rare systemic autoimmune rheumatic disease (SARD) with significant heterogeneity and no shared classification criteria. We aimed to identify clinical and serological features associated with ASSD that may be suitable for inclusion in the data-driven classification criteria for ASSD. Methods: We used a large, international, multicenter “Classification Criteria for Anti-synthetase Syndrome” (CLASS) project database, which includes both patients with ASSD and controls with mimicking conditions, namely, SARDs and/or interstitial lung disease (ILD). The local diagnoses of ASSD and controls were confirmed by project team members. We employed univariable logistic regression and multivariable Ridge regression to evaluate clinical and serological features associated with an ASSD diagnosis in a randomly selected subset of the cohort. Results: Our analysis included 948 patients with ASSD and 1,077 controls. Joint, muscle, lung, skin, and cardiac involvement were more prevalent in patients with ASSD than in controls. Specific variables associated with ASSD included arthritis, diffuse myalgia, muscle weakness, muscle enzyme elevation, ILD, mechanic's hands, secondary pulmonary hypertension due to ILD, Raynaud phenomenon, and unexplained fever. In terms of serological variables, Jo-1 and non–Jo-1 anti-synthetase autoantibodies, antinuclear antibodies with cytoplasmic pattern, and anti-Ro52 autoantibodies were associated with ASSD. In contrast, isolated arthralgia, dysphagia, electromyography/magnetic resonance imaging/muscle biopsy findings suggestive of myopathy, inflammatory rashes, myocarditis, and pulmonary arterial hypertension did not differentiate between patients with ASSD and controls or were inversely associated with ASSD. Conclusion: We identified key clinical and serological variables associated with ASSD, which will help clinicians and offer insights into the development of data-driven classification criteria for ASSD.
UR - http://www.scopus.com/inward/record.url?scp=85212698290&partnerID=8YFLogxK
U2 - 10.1002/art.43038
DO - 10.1002/art.43038
M3 - Article
C2 - 39467037
SN - 2326-5191
JO - Arthritis & Rheumatology
JF - Arthritis & Rheumatology
ER -