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Comparative Study
. 2024 Aug 1;63(8):2093-2100.
doi: 10.1093/rheumatology/kead481.

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

Ryan Malcolm Hum  1   2 , James B Lilleker  1   3 , Janine A Lamb  4 , Alexander G S Oldroyd  1   2 , Guochun Wang  5 , Lucy R Wedderburn  6 , Louise P Diederichsen  7 , Jens Schmidt  8   9   10 , Maria Giovanna Danieli  11 , Paula Oakley  12 , Zoltan Griger  13 , Thuy Nguyen Thi Phuong  14 , Chanakya Kodishala  15   16 , Monica Vazquez-Del Mercado  17 , Helena Andersson  18 , Boel De Paepe  19 , Jan L De Bleecker  19 , Britta Maurer  20 , Liza McCann  21 , Nicolo Pipitone  22 , Neil McHugh  23   24 , Robert Paul New  25 , William E Ollier  26 , Niels Steen Krogh  27 , Jiri Vencovsky  28 , Ingrid E Lundberg  29   30 , Hector Chinoy  1   31 ; MYONET Registry
Collaborators, Affiliations
Comparative Study

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

Ryan Malcolm Hum et al. Rheumatology (Oxford). .

Erratum in

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.

Keywords: Anti-synthetase syndrome; Cutaneous; Dermatomyositis; Epidemiology; Extramuscular; MYONET; Malignancy; Rashes; Skin.

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Figures

Figure 1.
Figure 1.
Flowchart illustrating the patients from the MYONET registry that were included and excluded from the study. DM-specific Ab refers to Mi2, TIF1γ, SAE, MDA5 and NXP2. ASyS-specific Ab refers to Jo1, PL12, PL7, EJ, OJ, Zo and KS. ASyS: anti-synthetase syndrome
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References

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