Citations 2
Consensus nomenclature and abbreviation for anti-synthetase syndrome: an IMACS project
Aggarwal, Anushka , Chandra, Tanya , Keret, Shiri , Pauling, John D , Shamim, Ejaz A. , Miller, Fredrick W., Mammen, Andrew L. , Sambataro, Gianluca , Castañeda, Santos , Dimachkie, Mazen M. , Katsumata, Yasuhiro , Mozaffar, Tahseen , Gunawardena, Harsha , Moghadam‐Kia, Siamak , Ladha, Parth, Mittal, Srijan, Haldule, Saloni , Edpuganti, Namratha , Sridhar, Shreya , Savaliya, Rutvik, Batra, Vanshita, Liewluck, T , Fernandez, Anthony P , Bartoloni, Elena , Shinjo, Samuel Katsuyuki , Katsumata, Yasuhiro , Fujimoto, Manabu , Lilleker, James B , Mozaffar, Tahseen , Rojas Serrano, Jorge , Edpuganti, Namratha , Aggarwal, Rohit , Pauling, John D , Dimachkie, Mazen M , Bonella, Francesco , Liewluck, Teerin , Shinjo, Samuel Katsuyuki , Werth, Victoria P. , Campanilho‐Marques, Raquel , Selva-O’Callaghan, Albert , Mann, H. , Cavagna, Lorenzo , Oddis, Chester V. , Mittal, Srijan, Aggarwal, Rohit
Rheumatology | April 25, 2025 | Volume: 64 | Issue: 9 | Pages: 4930-4936
Abstract Objectives The lack of uniform terminology and abbreviations for anti-synthetase syndrome has led to significant challenges in research and clinical practice. This study aimed to establish an international consensus on a standardized nomenclature and abbreviation among a diverse group of global myositis experts and patient representatives. Methods This qualitative project was approved by the International Myositis Assessment and Clinical Studies Group (IMACS). Based on the outcomes of a literature review and preliminary survey, list of terms for nomenclature and abbreviations was finalized. Subsequently, two rounds of the Delphi survey were conducted among myositis experts and patient participants to ascertain current practices and preferences. Using Nominal Group Technique (NGT), the project steering committee developed a consensus nomenclature and abbreviation. Results A total of 241 and 132 myositis experts from six continents, 35 countries and 9 specialities participated in round 1 and round 2 of the Delphi survey, respectively. The results indicated a preference for the terms anti-synthetase syndrome, antisynthetase syndrome and anti-tRNA synthetase syndrome. The abbreviations ASS, ASyS and ASSD were most used, though ASS received notable disapproval. The steering committee, including twenty-five myositis experts and three patient representatives from diverse backgrounds and geographical locations, reached a consensus on ‘anti-synthetase syndrome’ as the preferred nomenclature and ASyS as the preferred abbreviation, with 100% and 93.1% votes, respectively. Conclusion The terms anti-synthetase syndrome and ASyS should be adopted as the standard nomenclature and abbreviation in both research and clinical practice. This standardization is expected to increase consistency within the literature.
Comparison of clinical features between patients with anti-synthetase syndrome and dermatomyositis: results from the MYONET registry
Hum, Ryan Malcolm , Lamb, Janine A. , Oldroyd, Alexander , Wang, Guochun , Wedderburn, Lucy R, Diederichsen, Louise Pyndt , Schmidt, Jens , Danieli, Maria Giovanna , Oakley, Paula, Griger, Zoltán , Phuong, Thuy Nguyen Thi , Kodishala, Chanakya , Mercado, Mónica Vázquez-Del , Andersson, Helena , Paepe, Boél De , Maurer, Britta , McCann, Liza , Pipitone, Nicolò , McHugh, Neil , New, Robert P, Ollier, William , Krogh, Niels Steen , Lundberg, Ingrid E. , Chinoy, Hector , Registry, MYONET, D’hose, Sophie, Lu, Xin , Tian, Xiaolan , Pleštilová, Lenka, Barochová, Tereza, Kubínová, Kateřina , Gelardi, Chiara, Paladini, Alberto , Piga, Mario Andrea , Jara, Luis J. , Cruz‐Reyes, Claudia , Andrade-Ortega, Lilia, Medrano-Ramírez, Gabriel , Salazar-Páramo, Mario , Chavarría-Ávila, Efrain , Aguilar-Vázquez, Andrea, Anda, Jesús-Aureliano Robles-De , Molberg, Øyvind , Dastmalchi, Maryam, Notarnicola, Antonella , Gheorghe, Karina, Liden, Maria, Hanna, Balsam , Jalal, Awat, Hellström, Helena, Martineus, Jehns Christian, New, Paul, Platt, Hazel, Ahmed, Yasmeen , Armstrong, Raymond, Bernstein, Robert, Black, Carol M., Butler, Robin, Carty, John , Chattopadhyay, Chandra, Chelliah, E G, Clarke, Fiona, Dawes, P. T., Devlin, Joseph, Edwards, Christopher, Emery, Paul , Fordham, J. L. A., Fraser, Alexander, Gaston, Hill, Gordon, Patrick , Griffiths, Bridget , Gunawardena, Harsha , Hall, Frances, Hanna, Michael G. , Harrison, Beverley, Hay, Elaine M. , Hilton‐Jones, David, Horden, Lesley, Jones, Adrian, Kamath, Sanjeet, Kennedy, Thomas J. , Klimiuk, P S, Knights, Sally, Lambert, John, Laxminarayan, Ramasharan, Lecky, Bryan, Marks, Jeffrey M., Martin, Michael F., McGonagle, Dennis , McHugh, Neil, McKenna, Francis, McLaren, John, McMahon, Michael , McRorie, Euan , Merry, P, Miller, James , Nicholls, Anne, Ong, Voon , Over, K. E., Packham, John, Pipitone, Nicolo, Plant, Michael, Pountain, G., Pullar, T, Roberts, Mark, Scott, David A. , Shadforth, M F, Sheeran, Thomas, Srinivasan, Arul, Swinson, David, Teh, Lee‐Suan , Webley, M, Winer, Jonathan M., Wedderburn, Lucy R. , Lilleker, James B , Lamb, Janine A , Oldroyd, Alexander G S , Wedderburn, Lucy R , Griger, Zoltan , Nguyen Thi Phuong, Thuy , Kodishala, Chanakya , Vazquez-Del Mercado, Monica , De Paepe, Boel , McHugh, Neil , Vencovsky, Jiri , Lundberg, Ingrid E , Chinoy, Hector , McCann, Liza J , Bleecker, Jan De , Vencovský, Jiří , Registry, MYONET, Mann, H. , Kryštůfková, Olga , Klein, Martin , Saavedra, Miguel A , Lastra, Olga Lidia Vera , Satoh, Minoru , Petri, Marcelo H. , Rönnelid, Johan , Lan, Nguyễn Thị Ngọc , Padyukov, Leonid , Rothwell, Simon W. , Bernstein, Robert, Bowman, Simon , Bruce, Ian N , Carty, John, Denton, Christopher P. , Edwards, Christopher, Fordham, John, Isaacs, John Dudley , Isenberg, David , Kitas, George D. , Lanyon, Peter , Luqmani, Raashid Ahmed , Machado, Pedro , Marks, Jeffrey, Martin, Michael, McHugh, Neil, Miles, Sarah, Nixon, Jennifer, Ong, Voon, Packham, John, Pipitone, Nicolo, Sanders, Paul W. , Williams, Brian, Winer, Jonathan
Rheumatology | September 11, 2023 | Volume: 63 | Issue: 8 | Pages: 2093-2100
Abstract Objectives To compare clinical characteristics, including the frequency of cutaneous, extramuscular manifestations and malignancy, between adults with anti-synthetase syndrome (ASyS) and DM. Methods Using data regarding adults from the MYONET registry, a cohort of DM patients with anti-Mi2/-TIF1γ/-NXP2/-SAE/-MDA5 autoantibodies, and a cohort of ASyS patients with anti-tRNA synthetase autoantibodies (anti-Jo1/-PL7/-PL12/-OJ/-EJ/-Zo/-KS) were identified. Patients with DM sine dermatitis or with discordant dual autoantibody specificities were excluded. Sub-cohorts of patients with ASyS with or without skin involvement were defined based on presence of DM-type rashes (heliotrope rash, Gottron’s papules/sign, violaceous rash, shawl sign, V-sign, erythroderma, and/or periorbital rash). Results In total 1054 patients were included (DM, n = 405; ASyS, n = 649). In the ASyS cohort, 31% (n = 203) had DM-type skin involvement (ASyS-DMskin). A higher frequency of extramuscular manifestations, including Mechanic’s hands, Raynaud’s phenomenon, arthritis, interstitial lung disease and cardiac involvement differentiated ASyS-DMskin from DM (all P < 0.001), whereas higher frequency of any of four DM-type rashes—heliotrope rash (n = 248, 61% vs n = 90, 44%), violaceous rash (n = 166, 41% vs n = 57, 9%), V-sign (n = 124, 31% vs n = 28, 4%), and shawl sign (n = 133, 33% vs n = 18, 3%)—differentiated DM from ASyS-DMskin (all P < 0.005). Cancer-associated myositis (CAM) was more frequent in DM (n = 67, 17%) compared with ASyS (n = 21, 3%) and ASyS-DMskin (n = 7, 3%) cohorts (both P < 0.001). Conclusion DM-type rashes are frequent in patients with ASyS; however, distinct clinical manifestations differentiate these patients from classical DM. Skin involvement in ASyS does not necessitate increased malignancy surveillance. These findings will inform future ASyS classification criteria and patient management.