Citations 1
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.