Dataset Information

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

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.

SUBMITTER: Hum RM

PROVIDER: S-EPMC11292049 | biostudies-literature | 2024 Aug

REPOSITORIES: biostudies-literature

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Publications

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

Hum Ryan Malcolm RM Lilleker James B JB Lamb Janine A JA Oldroyd Alexander G S AGS Wang Guochun G Wedderburn Lucy R LR Wedderburn Lucy R LR Diederichsen Louise P LP Schmidt Jens J Danieli Maria Giovanna MG Oakley Paula P Griger Zoltan Z Nguyen Thi Phuong Thuy T Kodishala Chanakya C Vazquez-Del Mercado Monica M Andersson Helena H De Paepe Boel B De Bleecker Jan L JL Maurer Britta B McCann Liza L Pipitone Nicolo N McHugh Neil N New Robert Paul RP Ollier William E WE Krogh Niels Steen NS Vencovsky Jiri J Lundberg Ingrid E IE Chinoy Hector H

Rheumatology (Oxford, England) 20240801 8

<h4>Objectives</h4>To compare clinical characteristics, including the frequency of cutaneous, extramuscular manifestations and malignancy, between adults with anti-synthetase syndrome (ASyS) and DM.<h4>Methods</h4>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 dermat ...[more]

PMID: 37698987

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Project description:ObjectivesMyocarditis is an overlooked manifestation of anti-synthetase syndrome (ASS). Our study describes the clinical and instrumental features of ASS myocarditis and evaluates the performance of cardiac MRI (CMRI) with mapping techniques in assisting diagnosis of ASS myocarditis.MethodsData from patients with ASS were retrospectively analysed. CMRI data for patients diagnosed with myocarditis, including late gadolinium enhancement (LGE), T2 ratio, T1 mapping, extracellular volume (ECV) and T2 mapping, were reviewed. Myocarditis was defined by the presence of symptoms and/or signs suggestive for heart involvement, including increased high-sensitive troponin T (hs-TnT) and/or N-terminal pro-brain natriuretic peptide (NT-proBNP), and at least an instrumental abnormality. The clinical features of patients with ASS with and without myocarditis were compared. A P-value of <0.05 was considered statistically significant.ResultsAmong a cohort of 43 patients with ASS [median age 58 (48.0-66.0) years; females 74.4%; anti-Jo1 53.5%], 13 (30%) were diagnosed with myocarditis. In 54% of those 13 patients, myocarditis was diagnosed at clinical onset. All patients with ASS with myocarditis had at least one CMRI abnormality: increased ECV in all cases, presence of LGE in 91%, and increased T1 and T2 mapping in 91%. The 2009 Lake Louise criteria (LLC) were satisfied by 6 patients, and the 2018 LLC by 10 patients. With the updated LLC, the sensitivity for myocarditis improved from 54.6% to 91.0%. Patients with ASS with myocarditis were more frequently males (53% vs 13%; P = 0.009) with fever (69% vs 17%; P = 0.001), and had higher hs-TnT [88.0 (23.55-311.5) vs 9.80 (5.0-23.0) ng/l; P < 0.001], NT-proBNP [525.5 (243.5-1575.25) vs 59.0 (32.0-165.5; P = 0.013) pg/ml; P = 0.013] and CRP [7.0 (1.7-15.75) vs 1.85 (0.5-2.86) mg/l; P = 0.011] compared with those without myocarditis.ConclusionIn ASS, myocarditis is frequent, even at clinical onset. Patients with ASS with myocarditis frequently presented with fever and increased CRP, suggesting the existence of an inflammatory phenotype. The use of novel CMRI mapping techniques may increase diagnostic sensitivity for myocarditis in ASS.

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Dataset Information

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

Objectives

Methods

Results

Conclusion

Publications

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

Similar Datasets

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