Clinical Characteristics of Anti-synthetase Syndrome: Analysis From the Classification Criteria for Anti-Synthetase Syndrome Project

Faghihi-Kashani S, Yoshida A, Bozan F, Zanframundo G, Rozza D, Loganathan A, Dourado E, Sambataro G, Bauer-Ventura I, Bae SS, Lim D, Rivero-Gallegos D, Yamano Y, Selva-O'Callaghan A, Mammen AL, Scirè CA, Montecucco C, Oddis CV, Fiorentino D, Bonella F, Miller FW, Lundberg IE, Schmidt J, Rojas-Serrano J, Hudson M, Kuwana M, González-Gay MA, McHugh N, Corte TJ, Doyle TJ, Werth VP, Gupta L, Perez Roman DI, Bianchessi LM, Devarasetti PK, Shinjo SK, Luppi F, Cavazzana I, Moghadam-Kia S, Fornaro M, Volkmann ER, Piga M, Loarce-Martos J, De Luca G, Knitza J, Wolff-Cecchi V, Sebastiani M, Schiffenbauer A, Rider LG, Campanilho-Marques R, Marts L, Bravi E, Gunawardena H, Aggarwal R, Cavagna L, Anuja A, Sambataro D, Colaci M, Vancheri C, Mejia M, Rivera Matias PA, Bottazzi F, Alpini C, Rajasekhar L, Agarwal V, De Souza FHC, Miossi R, Pozzi MR, Faverio P, Franco G, Dei G, Franceschini F, Lacomis D, Iannone F, Girolamo F, Trallero-Araguás E, Gil-Vila A, Villar A, Charles-Schoeman C, Bukiri H, Murru C, Cauli A, Bachiller-Corral J, Dagna L, Campochiaro C, Distler J, Schett G, Florenzano M, Reyes F, Amato A, Dellaripa P, Saavedra S, Vergara K, Manfredi A, Vacchi C, Melo T, Gandiga P, Amratia D, Arrigoni E, Kaneko Y, Triantafyllias K, Mandel A, Castañeda S, Vicente-Rabaneda EF, Landon-Cardinal O, Alberti ML, Caro F, Greco Merino MG, Rua-Figueroa I, Fiehn C, Bauhammer J, Molad Y, Klein O, Govoni M, Nakashima R, Alpsoy E, Meyer A, Giannini M, Chinoy H, Lilleker J, Gallay L, Cottin V, Dieudè P, Campagne J, Saraiva AP, Conticini E, Sebastiani G, Nuno L, Scarpato S, Schiopu E, Sainaghi PP, Jovani V, Barsotti S, Umezawa N, Needham M, Cagnotto G, Danieli MG, Tomietto P, Gomez NP, Limonta M, Barausse G, Kondo Y, Ghetie D, Tamirou F, Basharat P, Tomaras S, Voll RE, Andersson H, Barrio JM, Schneider M, Parronchi P, Notarnicola A, Cifrian J (2024)

Publication Type: Journal article

Publication year: 2024

Journal

DOI: 10.1002/art.43038

Abstract

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.

Involved external institutions

Policlinico San Matteo Pavia Fondazione IRCCS Italy (IT) Italian Society of Rheumatology / Società Italiana di Reumatologia (SIR) Italy (IT) University of Bath United Kingdom (GB) University of Lisbon / Universidade de Lisboa (ULisboa) Portugal (PT) Università degli Studi di Catania Italy (IT) University of Chicago United States (USA) (US) University of California Los Angeles (UCLA) United States (USA) (US) Veterans Health Administration (VHA) United States (USA) (US) Instituto Nacional de Enfermedades Respiratorias Mexico (MX) Tosei General Hospital / 公立陶生病院 Japan (JP) Vall d'Hebron University Hospital / Hospital Universitari Vall d'Hebron Spain (ES) Johns Hopkins Hospital United States (USA) (US) University of Milano-Bicocca / Università degli Studi di Milano-Bicocca, UNIMIB Italy (IT) University of Pittsburgh United States (USA) (US) Stanford University United States (USA) (US) Ruhrlandklinik, Westdeutsches Lungenzentrum am Universitätsklinikum Essen gGmbH Germany (DE) National Institute of Environmental Health Sciences (NIEHS) United States (USA) (US) Hospital Clínico de la Universidad de Chile (HCUCH) Chile (CL) Karolinska Institute Sweden (SE) Universitätsklinikum Göttingen Germany (DE) McGill University Canada (CA) Universidad de Cantabria (UC) Spain (ES) University of Sydney (USYD) Australia (AU) Brigham and Women's Hospital (BWH) United States (USA) (US) University of Manchester United Kingdom (GB) Università degli Studi di Pavia Italy (IT) University of São Paulo / Universidade de São Paulo (USP) Brazil (BR) Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia Italy (IT) University of Bari Aldo Moro / Università degli Studi di Bari Aldo Moro Italy (IT) Azienda Ospedaliera Universitaria di Cagliari Italy (IT) Hospital Universitario Ramón y Cajal Spain (ES) Ospedale San Raffaele (früher: Centro San Raffaele del Monte Tabor Foundation) Italy (IT) Universitätsklinikum Gießen und Marburg (UKGM) Germany (DE) Hospital del Tórax / Instituto Nacional del Tórax Chile (CL) University of Parma / Università degli Studi di Parma Italy (IT) Emory University United States (USA) (US) Ospedale Guglielmo da Saliceto Italy (IT) North Bristol NHS Trust United Kingdom (GB)

How to cite

APA:

Faghihi-Kashani, S., Yoshida, A., Bozan, F., Zanframundo, G., Rozza, D., Loganathan, A.,... Cifrian, J. (2024). Clinical Characteristics of Anti-synthetase Syndrome: Analysis From the Classification Criteria for Anti-Synthetase Syndrome Project. Arthritis and Rheumatology. https://doi.org/10.1002/art.43038

MLA:

Faghihi-Kashani, Sara, et al. "Clinical Characteristics of Anti-synthetase Syndrome: Analysis From the Classification Criteria for Anti-Synthetase Syndrome Project." Arthritis and Rheumatology (2024).

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