Comparison of clinical features between patients with anti-synthetase syndrome and dermatomyositis: results from the MYONET registry

被引:13
作者
Hum, Ryan Malcolm [1 ,2 ]
Lilleker, James B. [1 ,3 ]
Lamb, Janine A. [4 ]
Oldroyd, Alexander G. S. [1 ,2 ]
Wang, Guochun [5 ]
Wedderburn, Lucy R. [6 ]
Diederichsen, Louise P. [7 ]
Schmidt, Jens [8 ]
Danieli, Maria Giovanna [9 ]
Oakley, Paula [10 ]
Griger, Zoltan [11 ]
Phuong, Thuy Nguyen Thi [12 ]
Kodishala, Chanakya [13 ,14 ]
Vazquez-Del Mercado, Monica [15 ]
Andersson, Helena [16 ]
De Paepe, Boel [17 ]
De Bleecker, Jan L. [17 ]
Maurer, Britta [18 ]
McCann, Liza [19 ]
Pipitone, Nicolo [20 ]
McHugh, Neil [21 ,22 ]
New, Robert Paul [23 ]
Ollier, William E. [24 ]
Krogh, Niels Steen [25 ]
Vencovsky, Jiri [26 ,27 ]
Lundberg, Ingrid E. [28 ,29 ]
Chinoy, Hector [1 ,30 ]
机构
[1] Univ Manchester, Fac Biol Med & Hlth, Ctr Musculoskeletal Res, Div Musculoskeletal & Dermatol Sci, Manchester, Lancs, England
[2] Univ Manchester, Natl Inst Hlth Res, Manchester Biomed Res Ctr, Manchester, Lancs, England
[3] Northern Care Alliance NHS Fdn Trust, Salford Royal Hosp, Manchester Ctr Clin Neurosci, Manchester, Lancs, England
[4] Univ Manchester, Fac Biol Med & Hlth, Div Populat Hlth Hlth Serv Res & Primary Care, Epidemiol & Publ Hlth Grp, Manchester, Lancs, England
[5] China Japan Friendship Hosp, Dept Rheumatol, Beijing, Peoples R China
[6] Great Ormond St Hosp Children NHS Fdn Trust, Infect Immun & Inflammat, London, England
[7] Copenhagen Univ Hosp, Ctr Rheumatol & Spine Dis, Copenhagen, Denmark
[8] Univ Med Ctr Gottingen, Dept Neurol, Gottingen, Germany
[9] Univ Politecn Marche, Dipartimento Sci Clin & Mol, Clin Med, Ancona, Italy
[10] Myositis UK, Southampton, Hants, England
[11] Univ Debrecen, Dept Immunol, Debrecen, Hajdu Bihar, Hungary
[12] Hanoi Med Univ, Internal Med Dept, Hanoi, Vietnam
[13] St Johns Natl Acad Hlth Sci, Clin Immunol & Rheumatol, Bangalore, Karnataka, India
[14] Mayo Clin, Dept Rheumatol, Rochester, MN USA
[15] Univ Guadalajara, Hosp Civil Dr Juan I Menchaca, Serv Reumatol, Div Med Interna, Guadalajara, Jalisco, Mexico
[16] Oslo Univ Hosp, Dept Rheumatol, Oslo, Norway
[17] Univ Ziekenhuis Gent, Dept Neurol, Ghent, Belgium
[18] Univ Hosp Bern, Dept Rheumatol & Immunol, Inselspital, Bern, Switzerland
[19] Alder Hey Childrens NHS Fdn Trust, Dept Rheumatol, Liverpool, Merseyside, England
[20] Arcispedale Santa Maria Nuova Reggio Emilia, Dept Rheumatol, Reggio Emilia, Emilia Romagna, Italy
[21] Royal Natl Hosp Rheumat Dis, Dept Rheumatol, Bath, Avon, England
[22] Univ Bath, Dept Pharm & Pharmacol, Bath, Avon, England
[23] Univ Liverpool, MRC ARUK Inst Ageing & Chron Dis, Liverpool, Merseyside, England
[24] Manchester Metropolitan Univ, Fac Sci & Engn, Manchester, Lancs, England
[25] Zitelab Aps, Copenhagen, Denmark
[26] Charles Univ Prague, Inst Rheumatol, Prague, Czech Republic
[27] Charles Univ Prague, Dept Rheumatol, Prague, Czech Republic
[28] Karolinska Inst, Dept Med, Div Rheumatol, Stockholm, Sweden
[29] Karolinska Univ Hosp, Dept Gastroenterol Dermatol & Rheumatol, Stockholm, Sweden
[30] Northern Care Alliance NHS Fdn Trust, Salford Royal Hosp, Dept Rheumatol, Salford, Lancs, England
基金
瑞典研究理事会; 英国医学研究理事会;
关键词
Anti-synthetase syndrome; Dermatomyositis; Cutaneous; Rashes; Skin; Malignancy; Epidemiology; MYONET; Extramuscular; IDIOPATHIC INFLAMMATORY MYOPATHIES; INTERSTITIAL LUNG-DISEASE; CLASSIFICATION;
D O I
10.1093/rheumatology/kead481
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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/-TIF1 gamma/-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.
引用
收藏
页码:2093 / 2100
页数:8
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