ArticleClinical rheumatology2025
Clinical features and risk factors of flare in anti-synthetase syndrome.
Article in Clinical rheumatology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed.
- Flares and definition of worsening in inflammatory myopathies: a narrative review of the literature.Rheumatology (Oxford, England) · 2026Review
- Clinical characteristics and risk factors associated with objective flares in patients with dermatomyositis.Seminars in arthritis and rheumatism · 2026Article
- Combination of tofacitinib and tacrolimus therapy in refractory interstitial lung disease associated with anti-synthetase syndrome: a case series of six patients.Frontiers in immunology · 2026Article
- Dose-Escalated Upadacitinib for Refractory Myositis in Anti-Jo-1-Positive Antisynthetase Syndrome: A Case Report.Case reports in rheumatology · 2026Article
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Authors and funding
17 authors.
Funding
Abstract
objectivesTo clarify clinical features and risk factors of flares in anti-synthetase syndrome (ASS).
methodsWe retrospectively identified consecutive patients with ASS by reviewing medical records. Clinical, laboratory, and imaging data were obtained. Patients were considered to have a flare when treatment was intensified for myositis and/or ILD with an increased dose of glucocorticoid (GC) and/or addition of a new immunosuppressant (IS).
resultsA total of 51 subjects were included. All 51 patients had ILD, 38 patients had myositis, and 39 patients had skin rash. Anti-aminoacyl-tRNA synthetase (ARS) antibody subtypes were anti-Jo-1 in 23 (45%), anti-EJ in 13 (25%), anti-PL-7 in 8 (16%), and anti-PL-12 in 7 (14%). As initial therapy, half of the patients were treated with GCs alone, while the rest were treated with a combination of GCs and ISs during observation. Flares occurred in 30 (58%) mostly when the prednisolone dose was tapered to 11 mg/day. Patients with anti-EJ or anti-Jo-1 frequently flared. The use of calcineurin inhibitors (CNIs) was significantly associated with less flares of myositis but not with those of ILD. In multivariate analyses, anti-ARS subtype and Gottron's sign were identified as independent risk factors for overall flare.
conclusionThe majority of ASS patients experienced a flare, which frequently occurred when the GC dose was tapered to 11 mg of prednisolone. The presence of anti-EJ/Jo-1 antibody and Gottron's sign were identified as independent risk factors for flare. In addition, the use of CNIs tended to associate with less flares of myositis. Key Points • The majority of patients with anti-synthetase syndrome experience a flare when glucocorticoids are tapered close to 10 mg of prednisolone. • The presence of anti-EJ/Jo-1 antibody and Gottron's sign are risk factors for flares in patients with anti-synthetase syndrome. • The use of calcineurin inhibitors may prevent flares of myositis in patients with anti-synthetase syndrome.
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