Abstract

OBJECTIVES: To compare clinical characteristics, including the frequency of cutaneous, extramuscular manifestations, and malignancy, between adults with anti-synthetase syndrome (ASyS) and dermatomyositis (DM).

METHODS: Using data regarding adults from the MYONET registry, a cohort of DM patients with anti-Mi2/-TIF1ɣ/-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 1,054 patients were included (DM, n = 405; ASyS, n = 649). In 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.

Original languageEnglish
Number of pages8
JournalRheumatology (Oxford, England)
Early online date12 Sept 2023
DOIs
Publication statusE-pub ahead of print - 12 Sept 2023

Bibliographical note

Funding statement This study was supported in part by: Association Francaise Contre Les Myopathies (AFM), The European Union Sixth Framework Programme (project AutoCure; LSH-018661), European Science Foundation (ESF) in the framework of the Research Networking Programme European Myositis Network (EUMYONET), The Swedish Research Council (K2014-52X-14045-14-3) and the regional agreement on medical training and clinical research (ALF) between Stockholm County Council and Karolinska Institutet; Myositis UK; Medical Research Council (MRC) UK grant MR/N003322/1; Arthritis Research UK (18474). The Czech cohort was supported by Project for Conceptual Development of Research Organization 00023728 from Ministry of Health in the Czech Republic.

Data Availability Statement

Data will be shared upon reasonable requests to the corresponding author.

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